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Critical aortic stenosis in the neonate: a multi-institutional study of management, outcomes, and risk factors. Congenital Heart Surgeons Society
Abstract
We sought to determine factors that would predict whether a biventricular repair or Norwood procedure pathway would give the best survival in neonates with critical aortic stenosis.
Survival and risk factors were determined with parametric time-dependent event analysis for patients undergoing either the Norwood procedure or biventricular repair, and predicted optimal pathway and survival benefit were derived from multivariable linear regression.
From 1994 to 2000, 320 neonates with critical left ventricular outflow obstruction were entered into a prospective multi-institutional study. Patients who died without intervention (n = 19) and those with primary cardiac transplantation (n = 6) were excluded. An initial intended biventricular repair pathway was indicated in 116 patients, with survival of 70% at 5 years. An initial Norwood procedure was performed in 179 patients, with survival of 60% at 5 years. Using predictions from separate multivariable hazard models for survival with each of the 2 pathways, we determined predicted optimal pathway and survival benefit for each patient. Significant independent factors associated with greater survival benefit for the Norwood procedure versus biventricular repair included younger age at entry, lower z-score of the aortic valve and left ventricular length, higher grade of endocardial fibroelastosis, absence of important tricuspid regurgitation, and larger ascending aorta. Predicted survival benefit favored the Norwood procedure in 50% of patients who had biventricular repair, and it favored biventricular repair in 20% of patients who had the Norwood procedure.
Morphologic and functional factors can be used to predict optimal pathway and survival benefit in neonates with critical left ventricular outflow obstruction.
... At 2 months of age, control cardiac MRI 8 showed striking LV growth (LVEDVI 37ml/m2). Herein an interventional hybrid take-down was 9 performed with dilatation of both pulmonary arterial bands and closure of the ductal stent with 10 an Amplatzer vascular plug-II thus, achieving biventricular circulation. ...
... 8 The aim of the initial staged procedure is to secure an unobstructed systemic outflow and 9 ensure effective/limited pulmonary blood flow. This initial palliation has been performed during 10 the last four decades by the classical Norwood surgical procedure (4). Alternatively, the hybrid 11 procedure (placement of bilateral pulmonary artery (PA) bands and ductal stenting) emerged in 12 Germany in 2002 aiming to avoid major neonatal surgery requiring cardiopulmonary bypass (5). ...
... Considering the 7 underdevelopment of the LV, however with favorable mitral, myocardial LV and aortic valve 8 anatomy, the decision to treat the HLHC patient with a hybrid procedure was made to increase 9 antegrade LV flow and thus, promote LV recruitment. 10 On the 6th DOL, the patient successfully underwent an operative correction of the TAPVC with 11 a side-to-side anastomosis between the pulmonary venous confluence and the left atrium and The stents exclusively drained the ductus into the aorta, without straddling the aortic vessel. 17 The post-procedural course was favorable, and the patient was extubated on the 13th DOL. ...
Background
Hybrid approach in hypoplastic left heart syndrome (HLHS) implies placement of bilateral pulmonary artery bands and ductal stenting. This approach serves as a less invasive surgical-interventional alternative to the Norwood procedure with reported equivalent survival. Potential benefits include deferrable surgical risks and in some borderline left ventricle patients, when combined with a restrictive interatrial communication, it offers a chance for biventricular circulation(n.
Case summary
Term newborn with total anomalous pulmonary venous connection (TAPVC), hypoplastic left heart, small aortic arch and ASD who was multidisciplinary deemed eligible for a hybrid palliation performed on day 6 of life after TAPVC surgical correction. Subsecuently, percutaneous ductal stent implantation was performed (2 Sinus Superflex DS stents) on day 11 of life. Magnetic resonance imaging (MRI) showed a hypoplastic non-apex forming left ventricle (LV) (left ventricular end-diastolic volume index (LVEDVI) 15 ml/m2). On day 27th of life, the ASD was closed surgically with a fenestrated patch. At 2 months of age, control cardiac MRI showed striking LV growth (LVEDVI 37 ml/m2). Herein an interventional hybrid take-down was performed with dilatation of both pulmonary arterial bands and closure of the ductal stent with an Amplatzer vascular plug-II thus, achieving biventricular circulation.
Discussion
Hybrid approach has proven as a safe alternative to the Norwood procedure for HLHS. This case illustrates the importance of multidisciplinary care in high-risk 2 ventricular patients by means of LV rehabilitation. Further studies are needed to systematically define and understand the intricacies of potential candidates for staged biventricular repair initially managed with hybrid approach.
... 1,3,4,[7][8][9][10] Two-dimensional (2D) echocardiographic assessment has historically been the main determinant in decision-making. Echocardiographic parameters to support pursuit of a biventricular strategy have included left ventricular end-diastolic volume indexed (LVEDVi) $20 mL/m 2 , MV z-score >À2, AoV/ root z-score >À2, absence of EFE, and presence of an apex-forming LV. 4,5,11 Cardiac magnetic resonance imaging (CMR) is increasingly being utilized as it provides additional parameters such as ascending aortic (AAo) flow and superior 3-dimensional quantification of ventricular volume. 6,12 Our institution began a protocol in the year 2003 to perform CMR in infants with borderline hypoplastic left heart (b-HLH) in whom the management decision for SV or biventricular circulation was uncertain. ...
... 13 Measurements were normalized for body surface area as z-scores. 11 LV EFE was defined as brightness of the endocardial wall or papillary muscle compared to the right ventricle endocardium with or without a globular LV geometry and categorized as per Congenital Heart Surgeon Society definition. 4 Hemodynamic data were obtained from 2D echocardiography and cardiac catheterization. Right ventricular systolic pressure was measured by tricuspid regurgitation velocity jet plus right atrial pressure (assumed as 5 mmHg). ...
... Cox Proportional Hazards Model for Transplant-Free andAAo ¼ ascending aortic flow; CoA ¼ aortic coarctation. AoV interventions, which are both worrisome colesions but neither deleterious by our survival analyses.4,11 . Our therapies for b-HLH promote left heart growth which is constrained by LV interstitialfibrosis (overt or microscopic). ...
Background
Cardiac magnetic resonance imaging (CMR) may augment 2-dimensional (2D) echocardiography in decision-making for biventricular repair in borderline hypoplastic left hearts.
Objectives
This study evaluates: 1) the relationship between 2D echocardiography and CMR; 2) imaging variables affecting assignment to biventricular vs non-biventricular management; and 3) variables affecting transplant-free biventricular survival.
Methods
We reviewed clinical, echocardiographic, and CMR data in 67 infants, including CMR-determined ascending aortic (AAo) flow and comparable left ventricular end-diastolic volume indexed (LVEDVi) by 2D-echocardiography and CMR.
Results
Treatment assignment to biventricular repair was either direct (BV, n = 45) or with a bridging hybrid procedure (H1-BV, n = 12). Echocardiographic LVEDVi was <20 mL/m² in 83% of biventricular repair infants and underestimated CMR-LVEDVi by 16.8 mL/m². AAo flows had no/weak correlation with aortic and mitral valve z-scores or LVEDVi. AAo flows differed between BV, H1-BV, and single-ventricle groups (median): 2.1, 1.7, and 0.7 L/min/m², respectively. Important variables for treatment assignment were presence of endocardial fibroelastosis, AAo flow, and mitral valve z-score. Biventricular repair was achieved in 54. The median follow-up was 8.0 (0.1-16.4) years. Transplant-free biventricular survival was 96%, 82%, and 77% at 1, 5, and 10 years, respectively. Patients without aortic coarctation repair were at higher risk of death, transplantation, or single-ventricle conversion (HR: 54.3; 95% CI: 6.3-47.1; P < 0.001) during follow-up. AAo flow had a smaller nonlinear effect with hazard ratio increasing at lower flows.
Conclusions
Historical 2D echocardiographic criteria would have precluded many patients from successful biventricular repair. AAo flow, an integrative index of left heart performance, was important in assigning patients to a biventricular circulation and affected survival. Biventricular survival was strongly associated with the need for aortic coarctation repair.
... For many CAS cases, the neonatal decision regarding clinical management towards either a biventricular or functionally single ventricle pathway remains one of the major challenges for the cardiac clinical team [10,11,12]. Existing postnatal imaging criteria [13,14,15] do not reliably differentiate neonates who will not thrive with biventricular repair. ...
... Whilst EFE is a well-known marker of poor initial outcome despite successful initial procedure [14,15] our findings suggest that EFE is associated with severe LV diastolic dysfunction. The association between EFE and diastolic function was reported by Robinson et al, observing teenagers who developed LV diastolic dysfunction after balloon valvulopasty of aortic stenosis in early infancy [18]. ...
... Late gadolinium enhancement on MRI may assist in assessing the severity and distribution of EFE but may be technically difficult in early infancy [46]. Different scores have been proposed to predict the likelihood of successful biventricular repair in the short term [47][48][49]. However, some patients will develop pulmonary hypertension (PHT) due to LV diastolic dysfunction. ...
... Manifestations of LCOS include low pulse volume, prolonged capillary refill time, low BP, metabolic acidosis, high lactate and oligoanuria [48]. In LCOS, the SVO 2 is low (i.e. ...
The IPCCC is a standardized international system of paediatric and congenital cardiac nomenclature that should be used in all registries, databases and research studies in the domain of paediatric and congenital cardiac care, including those related to HLHS. This recommendation is level 1 class C [15, 16, 700]. Multi-institutional databases and registries allow benchmarking of data concerning death, patterns of practice, morbidity and postoperative length of stay [540]. As a level 1 class C recommendation, all paediatric and congenital cardiac teams should routinely assess their own data against national and international benchmarks using multi-institutional databases and registries [540, 697–699, 701]. © 2020 European Association for Cardio-Thoracic Surgery. All rights reserved.
... Fetal Diagn Ther DOI: 10.1159/000460247 3 natal management based on clinical and echocardiographic assessment [24] . For the purpose of this study, patients were classified as having BVC or UVC according to the situation at last revision. ...
... The median GA at diagnosis was 22.0 weeks (range, 20-30.3) and at intervention was 23.0 [20][21][22][23][24][25][26][27][28][29][30][31][32]. The majority of fetuses showed reversed flow at the aortic arch and/or monophasic MV inflow as well as mitral regurgitation, left-to-right interatrial shunt, and moderate or severe LV systolic dysfunction (11/28, 39.3%, and 14/28, 50.0%, respectively). ...
Objective:
Fetal aortic valvuloplasty (FAV) may avoid progression of critical aortic stenosis (CAS) to hypoplastic left ventricle, improving the options for biventricular circulation (BVC). We describe the results of FAV in 2 referral centers in Spain.
Methods:
We analyzed all FAVs performed in the period 2007-2015. The selection of candidates, the technique, and postnatal management were made following an agreed protocol. A descriptive analysis of survival, type of circulation after birth, and complications was made, considering all deaths in the first 48 h after FAV as FAV-related.
Results:
FAV was performed in 28 fetuses at a median gestational age (GA) of 23 weeks (range, 20-32). FAV was technically successful in 22 (78.6%), of whom 11 were born alive and with intention to treat. Eight (72.7%) resulted in BVC and 3 (27.3%) in univentricular circulation. The rate of FAV-related deaths was 32%. These patients underwent FAV earlier than live-born fetuses (median GA at FAV 22 weeks [range, 20.0-25.0] vs. 24.5 weeks [range, 21.0-32.0], respectively, p = 0.031).
Conclusions:
A significant proportion of fetuses with CAS who undergo technically successful FAV have BVC postnatally. However, FAV implies a high risk of fetal death, which highly depends on the GA at which this intervention is required.
... Admission, diagnostic, and surgical information was abstracted from institutional medical records, echocardiogram reports, and surgical reports as described previously. 11 The median duration of follow-up for all neonates from the date of the initial procedure was 2.2 years (range, 1 day to 8.3 years). For survivors, the median duration of follow-up was 3.9 years (range, 1 day to 8.3 years), and data were complete for 79% of the patients within 1 year of the last annual follow-up. ...
... Demographic, diagnostic, and procedural information before stage 1 palliation were included as baseline variables and handled as described previously. 11 Measurements of cardiac dimensions were standardized as z-scores on the basis of published normative data, if available, or otherwise indexed to height or body surface area (BSA). 12 Missing values were estimated using multiple imputation, 13 and final variable selection was guided by bootstrap resampling (Appendix E1). 14 Continuous variables were compared with the Kruskal-Wallis test, using Wilcoxon rank scores or Student's t test. ...
Background
For neonates with critical left ventricular outflow tract obstruction (LVOTO), hybrid procedures are an alternative to the Norwood stage 1 procedure. Despite perceived advantages, however, outcomes are not well defined. Therefore, we compared outcomes after stage 1 hybrid and Norwood procedures.
Methods
In a critical LVOTO inception cohort (2005-2014; 20 institutions), a total of 564 neonates underwent stage 1 palliation with the Norwood operation with a modified Blalock-Taussig shunt (NW-BT; n = 232; 41%), Norwood operation with a right ventricle–to–pulmonary artery conduit (NW-RVPA; n = 222; 39%), or a hybrid procedure (n = 110; 20%). Post–stage 1 outcomes were analyzed via competing-risks and parametric hazard analyses and compared among all 564 patients and between patients who underwent propensity-matched hybrid and those who underwent NW-BT/NW-RVPA.
Results
By 6 years after the stage 1 operation, 50% ± 3%, 7% ± 2%, and 4% ± 1% of patients transitioned to Fontan, transplantation, and biventricular repair, respectively, whereas 7% ± 2% were alive without transition and 32% ± 2% died. Risk factors for death without transition included procedure type, smaller ascending aorta, aortic valve atresia, and lower birth weight. Risk-adjusted 4-year survival was better after NW-RVPA than after NW-BT or hybrid (76% vs 60% vs 61%; P < .001). Furthermore, for neonates with lower birth weight (<∼2 kg), an interaction between birth weight and hybrid resulted in a trend toward better survival after hybrid compared with NW-BT or NW-RVPA. For propensity-matched neonates between hybrid and NW-BT (88 pairs), 4-year survival was similar (62% vs 57%; P = .58). For propensity-matched neonates between hybrid and NW-RVPA (81 pairs), 4-year survival was better after NW-RVPA (59% vs 75%; P = .008).
Conclusions
For neonates with critical LVOTO undergoing single-ventricle palliation, NW-RVPA was associated with the best overall survival. Hybrid strategies are not a lower-risk alternative to Norwood operations overall; however, the impact of lower birth weight on survival may be mitigated after hybrid procedures compared with Norwood operations.
... The Congenital Heart Surgeons Society also sought to determine patients with aortic stenosis and small left ventricles who would do well with a biventricular repair. 28 This study was unique in that it also considered risk factors for single ventricle palliation. A multivariable analysis of factors associated with outcomes was used to predict the magnitude and the direction of the survival benefit for the optimal pathwaybiventricular repair or single ventricle palliation (Fig 2). ...
... Early postnatal studies have demonstrated that neonates with coarctation of the aorta and a hypoplastic, but morphologically normal, left ventricle can have successful secondary growth of the left-sided Survival benefit = Intercept + b1 (age at entry) + b2 (z-score of aortic valve at the sinuses) + b3 (grade of EFE) + b4 (ascending aorta diameter) + b 5 (presence of moderate or severe tricuspid regurgitation) + b6 (z-score of the left ventricular length) Figure 2. Congenital Heart Surgeons Society's multiple linear regression equation for survival benefit after biventricular repair. 28 10.98 (body surface area) + 0.56 (aortic annulus z-score) + 5.89 (left ventricular to heart long-axis ratio) -0.79 (grade 2 or 3 endocardial fibroelastosis) -6.78. structures after repair. ...
Borderline left ventricle refers to a spectrum of left ventricular underdevelopment, typically associated with other cardiac anomalies. The left ventricle may be mildly hypoplastic, as is sometimes seen accompanying aortic coarctation, or it can be severely hypoplastic, as is seen in hypoplastic left heart syndrome. For patients with a borderline left ventricle that is at either extreme, the treatment decision is relatively straightforward. Those with the most severe form of left ventricle hypoplasia will require single ventricle palliation or cardiac transplantation, whereas those with the mildest form may not need any intervention. It is the management strategy of children that fall within the grey zone of the spectrum, which continues to be controversial and remains variable within and among different institutions. Cardiac diseases with associated left ventricle hypoplasia include critical aortic stenosis, mitral stenosis, coarctation of the aorta, arch hypoplasia, cor triatriatum, unbalanced common atrioventricular canal, Shone's complex, total anomalous pulmonary venous return, and complex conotruncal abnormalities. In this review, we will discuss the assessment and management of infants with borderline left ventricle with critical aortic stenosis or arch obstruction and associated mitral anomalies.
... Of course, the above pathophysiological mechanisms should be understood as a continuum, [13,14] and some patients may be born with a markedly dilated LV and depressed contractility, known as critical AS ( Figure 1B). Such patients suffer from severe circulatory failure and pulmonary congestion, which is often life threatening and requires emergency intervention, either by catheters or surgery, as discussed below. ...
... In such a case, the Fontan procedure becomes a goal of treatment. Multicenter studies have elucidated that the outcome of biventricular repair with a small LV is much worse than that of the Fontan procedure [13,14], although the survival rate of Fontan completion for patients with a small LV or severely reduced LV function is only approximately 50-70%, even in the recent reports. [33][34][35] Based on the pathophysiology of evolving HLHS as previously discussed and the poor survival rate of HLHS patients, fetal intervention has been attempted, aimed at relieving AS at earlier stages before the LV cavity is reduced. ...
... Anatomic predictors of mortality after BVR have been reported for homogeneous groups of left-sided lesions, most notably for critical aortic stenosis [2,8,9]. However, these scoring systems often fail to predict survival with other forms of LHO, including arch hypoplasia and coarctation of the aorta [10,11]. ...
... Surgical strategy is challenging in patients with critical LHO associated with MV abnormalities. Echocardiography-derived survival estimates for patients with LHO are largely limited to patients with critical aortic stenosis [2,8,9]. These scores cannot be used to predict survival for other LHO lesions, such as arch obstruction or coarctation of the aorta, because the variables that are important for critical aortic stenosis are not necessarily the same in other forms of LHO [10,11]. ...
Mitral valve (MV) abnormalities may adversely impact outcomes after biventricular repair of critical left-side heart obstruction.
Neonates with left-side heart obstruction and MV abnormality (anatomically abnormal or hypoplastic but otherwise normal [Z-score -1.5 to -4]) who underwent biventricular repair from December 2005 to December 2012 were included. Institutional selection criteria determined suitability for biventricular repair. Primary outcomes (death, univentricular conversion, pulmonary hypertension) and secondary outcomes (reinterventions) were compared between hypoplastic and anatomically abnormal MV groups. Freedom from reintervention was determined by Kaplan-Meier analysis.
There were 55 patients with hypoplastic MV (n = 35) or anatomically abnormal MV (n = 20). Initial interventions included arch repair (n = 45, with ventricular septal defect closure in 7), balloon aortic valvuloplasty (n = 8), and Ross-Konno operation (n = 2). None had initial MV intervention. Follow-up was available on 46 patients (84%) at a median of 25 months. Primary outcome occurred in 6 patients (13%): 2 deaths, 1 univentricular conversion, and 3 pulmonary hypertension. Twenty-six patients (57%) underwent 36 follow-up interventions, often for arch obstruction (n = 26). All follow-up MV interventions (n = 4) occurred in patients with anatomically abnormal MV. Median reintervention-free time was 7.3 months. Freedom from reintervention at 6, 9, and 12 months was 65%, 38%, and 27%, respectively. Neither MV size nor anatomy was associated with primary outcome.
Using our selection criteria, most neonates with left-side heart obstruction and MV abnormalities who underwent biventricular repair had reasonable survival with complex reintervention needs in early follow-up. The MV reintervention occurred in patients with abnormal anatomy, not hypoplasia.
Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.
... Critical aortic stenosis (cAS) is defined as severe congenital valvular AS with two balanced ventricles and a ductdependent systemic circulation [10]. Interventional treatment must be undertaken during the neonatal period to reduce left ventricular (LV) pressure afterload with expectation of recovery of LV function; however, the optimal option and subsequent treatment remain controversial [2,8,11,17]. ...
... Especially in patients with severely depressed LV function, mainly associated with endocardial fibroelastosis (EFE), it is difficult to determine during the neonatal and early infantile period which track they should be put on, a single ventricle or biventricular. Even if single ventricular palliation (Norwood-type arch reconstruction) was selected, the presence of relatively sized, but dysfunctional LV must be a risk factor for the establishment of the Norwood circulation [4,7,10]. ...
The optimal management strategy for neonates with congenital aortic stenosis, two balanced ventricles, and duct-dependent systemic circulation (critical aortic stenosis) is still controversial. Thirteen patients with critical aortic stenosis underwent balloon aortic valvotomy (BAV) between 1996 and 2013, at the median age of 1 day old (range 0-28). Since 2010, bilateral pulmonary artery banding with ductal stenting following BAV was conducted for patients with reduced left ventricular (LV) function as a hybrid stage I palliation for the bridge to decision for further treatment. A follow-up was completed on all patients and the median follow-up period was 3.3 years (max 16.0). The overall survival rate at 15 years was 67.1 %. Six of the seven patients with maintained LV function could go on to the definitive Ross or Konno-aortic valve replacement at the median duration of 311 days after initial BAV, without any mortality. Three of four patients with reduced LV function died before 2010 with conventional treatment. With use of a hybrid stage I palliation, one of two patients ultimately underwent Fontan completion at 38 months of age and the other successfully underwent the definitive Ross-Konno operation at 9 months of age after recovery of the LV function. Although a statistically significant improvement has not been observed yet, the application of hybrid stage I palliation following BAV would be a favorable alternative for patients with reduced LV function to avoid a high-risk neonatal Ross or Norwood-type operation, and also to determine further treatment carefully.
... Although several studies have identified echocardiographic parameters to predict successful biventricular conversion in both BHLHS [12,[23][24][25] and RDAVC [26][27][28][29], decision-making remains challenging. In a multicenter, retrospective evaluation of 362 neonates with critical left ventricular outflow obstruction, Hickey et al. found that inappropriate pursuit of biventricular repair in patients with borderline left heart structures was common and resulted in poor long-term survival compared to that predicted with single ventricle palliation [6]. ...
... However, this error is at least partially mitigated in our study by the use of the ratio of atrioventricular valve flow as both valves move in the same direction. Several studies have identified the presence of extensive EFE as a risk factor for unsuccessful biventricular repair [24,31,32]. In our study, the high rate of EFE resection confounded the interpretation of EFE data. ...
Background
We sought to identify cardiovascular magnetic resonance (CMR) parameters associated with successful univentricular to biventricular conversion in patients with small left hearts.
Methods
Patients with small left heart structures and a univentricular circulation who underwent CMR prior to biventricular conversion were retrospectively identified and divided into 2 anatomic groups: 1) borderline hypoplastic left heart structures (BHLHS), and 2) right-dominant atrioventricular canal (RDAVC). The primary outcome variable was transplant-free survival with a biventricular circulation.
Results
In the BHLHS group (n = 22), 16 patients (73%) survived with a biventricular circulation over a median follow-up of 40 months (4–84). Survival was associated with a larger CMR left ventricular (LV) end-diastolic volume (EDV) (p = 0.001), higher LV-to-right ventricle (RV) stroke volume ratio (p < 0.001), and higher mitral-to-tricuspid inflow ratio (p = 0.04). For predicting biventricular survival, the addition of CMR threshold values to echocardiographic LV EDV improved sensitivity from 75% to 93% while maintaining specificity at 100%. In the RDAVC group (n = 10), 9 patients (90%) survived with a biventricular circulation over a median follow-up of 29 months (3–51). The minimum CMR values were a LV EDV of 22 ml/m2 and a LV-to-RV stroke volume ratio of 0.19.
Conclusions
In BHLHS patients, a larger LV EDV, LV-to-RV stroke volume ratio, and mitral-to-tricuspid inflow ratio were associated with successful biventricular conversion. The addition of CMR parameters to echocardiographic measurements improved the sensitivity for predicting successful conversion. In RDAVC patients, the high success rate precluded discriminant analysis, but a range of CMR parameters permitting biventricular conversion were identified.
... We can only speculate that, based on the echocardiographic data only, this diagnosis and its functional impact cannot be made correctly. 18,19 The recent treatment era carried a significantly better neonatal survival than the earliest treatment period. Survival free from any reintervention did not differ among the eras. ...
Background
The goal of this study was to evaluate long‐term results of percutaneous balloon valvuloplasty (BVPL) used exclusively for initial management of congenital aortic stenosis in children.
Methods and Results
A total of 409 consecutive pediatric patients (134 newborns, 275 older patients) who underwent BVPL as initial treatment of aortic stenosis in a single nationwide pediatric center were subjected to a retrospective follow‐up study. The resulting follow‐up time reached a median of 18.5 (interquartile range, 12.2–25.1) years. Successful BVPL was defined by residual Doppler gradient <70/40 (systolic/mean) mm Hg. The primary end point was death; secondary end points included any valve reintervention, balloon revalvuloplasty, any aortic valve surgery, and aortic valve replacement, respectively. BVPL effectively reduced the peak and mean gradient both immediately and at the latest follow‐up ( P <0.001). There was significant procedure‐related progression of aortic insufficiency ( P <0.001). Higher aortic annulus z score was predictive for severe aortic regurgitation ( P <0.05) and lower z score for insufficient gradient reduction ( P <0.05). The actuarial probability of survival/survival free from any valve reintervention was 89.9%/59.9%, 85.9%/35.2%, and 82.0%/26.7% at 10, 20, and 30 years after first BVPL, respectively. Left ventricular dysfunction or arterial duct dependency as the indication for BVPL was predictive of both worse survival and survival free from any reintervention ( P <0.001). Lower aortic annulus z score and lower balloon‐to‐annulus ratio were predictive of a need for revalvuloplasty ( P <0.001).
Conclusions
Percutaneous BVPL provides good initial palliation. In patients with hypoplastic annuli and left ventricular or mitral valve comorbidity, the results are less favorable.
... Varying degrees of hypoplasia are seen in diseases such as HLHS, critical aortic stenosis, double outlet RV and malaligned atrioventricular canals to name a few. There has been a number of studies which attempt to aid in management of these patients, however, many questions remain [542][543][544]. ...
Cardiovascular magnetic resonance (CMR) has been utilized in the management and care of pediatric patients for nearly 40 years. It has evolved to become an invaluable tool in the assessment of the littlest of hearts for diagnosis, pre-interventional management and follow-up care. Although mentioned in a number of consensus and guidelines documents, an up-to-date, large, stand-alone guidance work for the use of CMR in pediatric congenital 36 and acquired 35 heart disease endorsed by numerous Societies involved in the care of these children is lacking. This guidelines document outlines the use of CMR in this patient population for a significant number of heart lesions in this age group and although admittedly, is not an exhaustive treatment, it does deal with an expansive list of many common clinical issues encountered in daily practice.
... In Case 2, the fetus had significant EFE affecting the left ventricular endocardium and MV apparatus (grade 3 according to McElhinney et al. [16]). However, even after FOV, the left ventricular function did not improve until the postnatal period; where inotropic support initiated and ventricle function start to improve, the pressure gradient across the MV start to show up .. The combination of aortic stenosis, mitral stenosis and significant EFE in a neonate, even with an adequate LV size, is a risk factor for a poor outcome [16][17][18]. ...
Background:
Fetal aortic stenosis may progress to hypoplastic left heart syndrome (HLHS), which carries a poor prognosis. We report two infants with fetal aortic stenosis successfully treated with fetal aortic valvuloplasty (FAV) using balloon dilatation.
Case presentation:
Of five fetuses with aortic stenosis fulfilling the FAV criteria of severe aortic stenosis with a left ventricular length Z-score of ≥ - 2, retrograde flow in the transverse aortic arch, left-to-right flow across the foramen ovale, monophasic mitral inflow, and significant left ventricular dysfunction, we obtained permission for FAV in two fetuses. FAV was performed successfully under echocardiographic guidance using balloon dilatation. Both fetuses survived to birth. During FAV, mild pericardial effusion developed when introducing the stylet needle in the second fetus, and this resolved within 48 h. No intraprocedural complications occurred in the first patient, and no maternal complications occurred. The first infant underwent the Ross procedure after birth and is currently 7 years old and doing well. The second patient underwent aortic and mitral valve repair with endocardial fibroelastosis resection approximately 2 weeks after birth, which temporarily addressed the mitral valve stenosis; high doses of inotropes were subsequently required. The infant died of sepsis at 2 months of age.
Conclusion:
FAV using balloon dilatation to treat fetal aortic stenosis was successful in our two patients, with subsequent neonatal biventricular repair resulting in long-term survival in one patient and death secondary to sepsis in the second patient.
... This predictive equation is well known as Rhodes score. In 2001, the Congenital Heart Surgeons Society (CHSS) [14] proposed a regression equation to predict 5-year survival benefit of univentricular repair vs. biventricular repair as follows: Survival benefit = Intercept + b1 (age at entry) + b2 (z-score of aortic valve at the sinuses) + b3 (grade of EFE) + b4 (ascending aorta diameter) + b5 (presence of moderate or severe tricuspid regurgitation) + b6 (z-score of the LV length). The CHSS regression equation incorporated EFE and tricuspid regurgitation into the scoring system. ...
Objective:
In recent years, attempting the biventricular pathway or biventricular conversions in patients with borderline ventricle has become a hot topic. However, inappropriate pursuit of biventricular repair in borderline candidates will lead to adverse clinical outcomes. Therefore, it is important to accurately assess the degree of ventricular development before operation and whether it can tolerate biventricular repair. This review evaluated ventricular development using echocardiography for a better prediction of biventricular repair in borderline ventricle.
Data sources:
Articles from January 1, 1990 to April 1, 2019 on biventricular repair in borderline ventricle were accessed from PubMed, using keywords including "borderline ventricle," "congenital heart disease," "CHD," "echocardiography," and "biventricular repair."
Study selection:
Original articles and critical reviews relevant to the review's theme were selected.
Results:
Borderline left ventricle (LV): (1) Critical aortic stenosis: the Rhodes score, Congenital Heart Surgeons Society regression equation and another new scoring system was proposed to predict the feasibility of biventricular repair. (2) Aortic arch hypoplasia: the LV size and the diameter of aortic and mitral valve (MV) annulus should be taken into considerations for biventricular repair. (3) Right-dominant unbalanced atrioventricular septal defect (AVSD): atrioventricular valve index (AVVI), left ventricular inflow index (LVII), and right ventricle (RV)/LV inflow angle were the echocardiographic indices for biventricular repair. Borderline RV: (1) pulmonary atresia/intact ventricular septum (PA/IVS): the diameter z-score of tricuspid valve (TV) annulus, ratio of TV to MV diameter, RV inlet length z-score, RV area z-score, RV development index, and RV-TV index, etc. Less objective but more practical description is to classify the RV as tripartite, bipartite, and unipartite. The presence or absence of RV sinusoids, RV dependent coronary circulation, and the degree of tricuspid regurgitation should also be noted. (2) Left-dominant unbalanced AVSD: AVVI, LV, and RV volumes, whether apex forming ventricles were the echocardiographic indices for biventricular repair.
Conclusions:
Although the evaluation of echocardiography cannot guarantee the success of biventricular repair surgery, echocardiography can still provide relatively valuable basis for surgical decision making.
... To determine the therapeutic option for single ventricle versus two-ventricle repair, the Rhode's equation can be used. The Congenital Heart Surgeons Society, after a large multicenter prospective study, produced a multiple linear regression equation that predicts the magnitude and the direction of the optimum pathway for a 5-year survival benefit of Norwood (staged single-ventricle repair) compared with biventricular repair [52]: ...
Aortic valve stenosis in children is a congenital heart defect that causes fixed form of hemodynamically significant left ventricular outflow tract obstruction with progressive course. Neonates and young infants who have aortic valve stenosis, usually develop congestive heart failure. Children and adolescents who have aortic valve stenosis, are mostly asymptomatic, although they may carry a small but significant risk of sudden death. Transcatheter or surgical intervention is indicated for symptomatic patients or those with moderate to severe left ventricular outflow tract obstruction. Many may need reintervention.
... Other variables, such as a complex mix of anatomic substrates, cardiac and non-cardiac comorbidities, institutional factors, and a broad array of surgical techniques, should be comprehensively considered in determining the feasibility of BVR (3,16). Therefore, various scoring systems, including multiple variables such as endocardial fibroelastosis and tricuspid regurgitation, have been developed to improve the predictability of successful BVR exclusively for patients with critical aortic stenosis (4,(22)(23)(24). ...
Objective
To determine the utility of computed tomography (CT) ventricular volumes and morphometric parameters for deciding the treatment strategy in children with a hypoplastic left ventricle (LV).
Materials and Methods
Ninety-four consecutive children were included in this study and divided into small LV single ventricle repair (SVR) (n = 28), small LV biventricular repair (BVR) (n = 6), disease-matched control (n = 19), and control (n = 41) groups. The CT-based indexed LV volumes, LV-to-right-ventricular (LV/RV) volume ratio, left-to-right atrioventricular valve (AVV) area ratio, left-to-right AVV diameter ratio, and LV/RV long dimension ratio were compared between groups. Proportions of preferred SVR in the small LV SVR group suggested by the parameters were evaluated.
Results
Indexed LV end-systolic (ES) and end-diastolic (ED) volumes in the small LV SVR group (6.3 ± 4.0 mL/m² and 14.4 ± 10.2 mL/m², respectively) were significantly smaller than those in the disease-matched control group (16.0 ± 4.7 mL/m² and 37.7 ± 12.0 mL/m², respectively; p < 0.001) and the control group (16.0 ± 5.5 mL/m² and 46.3 ± 10.8 mL/m², respectively; p < 0.001). These volumes were 8.3 ± 2.4 mL/m² and 21.4 ± 5.3 mL/m², respectively, in the small LV BVR group. ES and ED indexed LV volumes of < 7 mL/m² and < 17 mL/m², LV/RV volume ratios of < 0.22 and < 0.25, AVV area ratios of < 0.33 and < 0.24, and AVV diameter ratios of < 0.52 and < 0.46, respectively, enabled the differentiation of a subset of patients in the small LV SVR group from those in the two control groups. One patient in the small LV biventricular group died after BVR, indicating that this patient might not have been a good candidate based on the suggested cut-off values.
Conclusion
CT-based ventricular volumes and morphometric parameters can suggest cut-off values for SVR in children with a hypoplastic LV.
... Z-scores are superior to dichotomous ''normal values'' because they allow clinicians to appreciate the ''magnitude of abnormality.'' Z-score estimates are now part of the decision making in clinical and surgical management in pediatric cardiology [24,25]. ...
Objective
To establish normal reference ranges and Z-scores for aortic diameters in preterm infants according to the body surface area and assess their correlation with body weight, body surface area, and gestational age.
Patients and methods
In a prospective study, 268 preterm infants who fulfilled the criteria for inclusion were examined. Echocardiograms were performed to measure the ascending aorta, transverse aorta, and aortic isthmus diameters on 0 days to 6 days of life and at weekly intervals until the babies reached 36 weeks. Body surface area was divided into 13 groups from 0.07 m² to 0.19 m².
Results
The mean gestational age was 29.8 [± 2.38 standard deviation (SD)] weeks, ranging from 24 weeks to 35 weeks. The mean body weight was 1479 (± 413 SD) g, ranging from 588 g to 3380 g, and the mean body surface area was 0.13 m², ranging from 0.07 m² to 0.19 m². All the aortic diameters correlated well with both body weight and body surface area. Reference ranges with the mean ± SD, range, and Z-scores were calculated for aortic diameters according to the body surface area. A significant gradual increase was observed in ascending aorta, transverse aorta, and aortic isthmus diameters with increasing body surface area. Overall, a progressive and significant increase in ascending aorta, transverse aorta, and aortic isthmus diameters was observed during the first 9 weeks of life.
Conclusion
The ascending aorta, transverse aorta, and aortic isthmus diameters exhibited a significant correlation with the body surface area and body weight. This study provides reference data with Z-scores that can be used as a normal reference tool for the ascending aorta, transverse aorta, and aortic isthmus diameters for preterm infants based on the body surface area.
... Risk factors for death after the Norwood and after S2P were identified. Candidate covariables are listed in Supplemental Tables 3 to 5. Variables were extracted from patient medical records and processed as previously described [8,9]. Multiple imputation was used for variables with 50% or greater completeness; those with less were excluded [10]. ...
Background:
The effect of the timing of stage-2-palliation (S2P) on survival through single ventricle palliation remains unknown. This study investigated the optimal timing of S2P that minimizes pre-S2P attrition and maximizes post-S2P survival.
Methods:
The Congenital Heart Surgeons' Society's critical left ventricular outflow tract obstruction cohort was used. Survival analysis was performed using multiphase parametric hazard analysis. Separate risk factors for death after the Norwood and after S2P were identified. Based on the multivariable models, infants were stratified as low, intermediate, or high risk. Cumulative 2-year, post-Norwood survival was predicted. Optimal timing was determined using conditional survival analysis and plotted as 2-year, post-Norwood survival versus age at S2P.
Results:
A Norwood operation was performed in 534 neonates from 21 institutions. The S2P was performed in 71%, at a median age of 5.1 months (IQR: 4.3 to 6.0), and 22% died after Norwood. By 5 years after S2P, 10% of infants had died. For low- and intermediate-risk infants, performing S2P after age 3 months was associated with 89% ± 3% and 82% ± 3% 2-year survival, respectively. Undergoing an interval cardiac reoperation or moderate-severe right ventricular dysfunction before S2P were high-risk features. Among high-risk infants, 2-year survival was 63% ± 5%, and even lower when S2P was performed before age 6 months.
Conclusions:
Performing S2P after age 3 months may optimize survival of low- and intermediate-risk infants. High-risk infants are unlikely to complete three-stage palliation, and early S2P may increase their risk of mortality. We infer that early referral for cardiac transplantation may increase their chance of survival.
... Z-score estimates are now part of the decision making in clinical and surgical management in pediatric cardiology. [23,24]. ...
To establish normal reference ranges and Z-scores for pulmonary artery diameters in preterm infants and their correlation with body surface area, body weight, and chronological age. In a prospective study, 268 preterm infants, who fulfilled the inclusion criteria were examined. Echocardiograms were performed to measure the main pulmonary artery, right pulmonary artery, and left pulmonary artery diameters on day(s) 0 to −6 of life and at weekly intervals until they reached 36 weeks of age. Body surface area was divided into thirteen groups from 0.07 to 0.19 m². The mean gestational age was 29.8 (±2.38 SD) weeks, ranging between 24 and 35, the mean body weight was 1479 (±413 SD) grams, ranging between 588 and 3380, and the mean body surface area was 0.13 m², ranging between 0.07 and 0.19 m². All the pulmonary artery diameters correlated well with both body weight and body surface area. Reference ranges, with mean ± SD, range, and Z-scores for aortic diameters according to body surface area were calculated. A significant gradual increase was observed in main and branch pulmonary artery diameters with increasing body surface area. Overall, a progressive and significant increase for main and branch pulmonary artery diameters was observed during the first nine weeks of life. The main and branch pulmonary artery diameters were found to have significant correlation with body surface area. The study also provides reference data with Z-scores, which can be used as a normal reference tool for measuring the main pulmonary artery, right, and left pulmonary artery diameters of preterm infants against body surface area.
... However, the predictive value of the Rhodes score cannot be applied to other forms of left-sided obstructive lesions, specifically those with aortic arch obstruction [16][17][18][19]. Similarly, the Congenital Heart Surgeons Society analyzed data from a large, multicenter cohort to develop a model that predicted survival benefit for BVC in patients with critical aortic stenosis and other forms of left ventricular outflow tract obstruction [1,20]. As critical left ventricular outflow tract obstruction is a necessary component of both of these formulas, they may not be applicable to infants presenting with primary aortic arch obstruction. ...
In infants with aortic arch hypoplasia and small left-sided cardiac structures, successful biventricular repair is dependent on the adequacy of the left-sided structures. Defining accurate thresholds of echocardiographic indices predictive of successful biventricular repair is paramount to achieving optimal outcomes. We sought to identify pre-operative echocardiographic indices of left heart size that predict intervention-free survival in infants with small left heart structures undergoing primary aortic arch repair to establish biventricular circulation (BVC). Infants ≤2 months undergoing aortic arch repair from 1999 to 2010 with aortic and/or mitral valve hypoplasia, (Z-score ≤−2) were included. Pre-operative and follow-up echocardiograms were reviewed. Primary outcome was successful biventricular circulation (BVC), defined as freedom from death, transplant, or single ventricular conversion at 1 year. Need for catheter based or surgical re-intervention (RI), valve annular growth, and significant late aortic or mitral valve obstruction were additional outcomes. Fifty one of 73 subjects (79%) had successful BVC and were free of RI at 1 year. Seven subjects failed BVC; four of those died. The overall 1 year survival for the cohort was 95%. Fifteen subjects underwent a RI but maintained BVC. In univariate analysis, larger transverse aorta (p = 0.006) and aortic valve (p = 0.02) predicted successful BVC without RI. In CART analysis, the combination of mitral valve (MV) to tricuspid valve (TV) ratio ≤0.66 with an aortic valve (AV) annulus Z-score ≤−3 had the greatest power to predict BVC failure (sensitivity 71%, specificity 94%). In those with successful BVC, the combination of both AV and MV Z-score ≤−2.5 increased the odds of RI (OR 3.8; CI 1.3–11.4). Follow-up of non-RI subjects revealed improvement in AV and MV Z-score (median AV annulus changed over time from −2.34 to 0.04 (p < 0.001) and MV changed from −2.88 to −1.41 (p < 0.001), but residual mitral valve stenosis and aortic arch obstruction were present in one-third of subjects. In this cohort of infants requiring initial aortic arch repair with concomitant small left heart structures, successful BVC can be predicted from combined echocardiographic indices. In this complex population, 1 year survival is high, but the need for RI and the presence of residual lesions are common.
... Finally, critical aortic stenosis (AS) of the neonate is an entity apart from other forms of paediatric valvulopathies, as for those patients the prognosis will mostly be dependent on parameters such as the size of the aortic annulus and of the mitral valve, and the presence of endocardial fibroelastosis, all surrogate markers of an optimal in utero LV development [4,5]. ...
OBJECTIVES
To analyse our institutional results in the setting of paediatric aortic valve (AV) repair. Primary end-points were overall survival, freedom from AV reoperation and freedom from AV replacement.
METHODS
A retrospective analysis of all patients under 18 years of age operated on from 1977 to 2015 in a single tertiary care level institution. Patients were included if they benefited from any type of AV repair procedure, including commissurotomy, leaflet shaving or plication, or leaflet augmentation. All data were gathered from patients’ medical records, operative reports and referring paediatric cardiologists. The median follow-up was 50 months (IQR [13–140]).
RESULTS
Sixty-six patients were included. Indications for surgery were aortic stenosis, aortic regurgitation and mixed disease in 13 (19%), 36 (55%) and 17 (26%) patients, respectively. According to El Khoury's functional classification, among the 55 patients with some degree of regurgitation there were 5 type Ib regurgitation, 23 type II and 27 type III. During AV repair, additional procedures were performed in 36 patients, VSD closure, subaortic membrane resection and mitral valve repair being the most frequent (18, 8 and 7 patients). RACHS score was predominantly 2 (98.5% of patients). The in-hospital mortality rate was 1.5% (1/66). Major morbidity included 10 pericardial effusions (1 pericardocentesis), 1 low cardiac output syndrome and 1 stroke. There were three late deaths (at 104, 140 and 179 months after repair). All were cardiac related. Overall 5- and 10-year survival rates were 100 and 95.7%. The rates of freedom from AV reoperation and AV replacement at 5 and 10 years were 90.6, 72.1 and 92.5, 82.7%, respectively. During follow-up, there was no occurrence of valve-related complication (endocarditis, thromboembolism and bleeding).
CONCLUSIONS
In our experience, AV repair in the paediatric population provides excellent results in terms of both overall survival and valve-related reoperation. It obviates the need for chronic anticoagulation and in most cases delays the time at which more complex surgery such as the Ross procedure should be undertaken.
... 453 Outcome for infants born with critical aortic valve stenosis has improved steadily, and 5-year survival rates are between 77% and 85% at 5 years. 454,455 The risk of sudden death in children after balloon aortic valvuloplasty is highest in infants with elevated PAP beyond 1 month of age. 453 PH can persist throughout childhood in some patients 4 to 12 years after treatment of congenital aortic valve stenosis in infancy. ...
Pulmonary hypertension is associated with diverse cardiac, pulmonary, and systemic diseases in neonates, infants, and older children and contributes to significant morbidity and mortality. However, current approaches to caring for pediatric patients with pulmonary hypertension have been limited by the lack of consensus guidelines from experts in the field. In a joint effort from the American Heart Association and American Thoracic Society, a panel of experienced clinicians and clinician-scientists was assembled to review the current literature and to make recommendations on the diagnosis, evaluation, and treatment of pediatric pulmonary hypertension. This publication presents the results of extensive literature reviews, discussions, and formal scoring of recommendations for the care of children with pulmonary hypertension.
... valvuloplasty. 8 The basis for decision of initial approach is ill defined 9 and is most often reflective of institutional bias. It may be influenced by the presence of associated cardiac anomalies and varying degrees of left-sided hypoplasia. ...
Initial palliative treatment of congenital aortic valve stenosis consists of either surgical commissurotomy or transcatheter balloon valvuloplasty. The basis for selection of primary approach usually is ill defined. It was hypothesized that aortic valve characteristics may be helpful in directing choice of intervention. This study assesses the impact of valve morphology on time to reintervention after catheter- or surgical-based therapy.
A retrospective review of preprocedural echocardiographic aortic outflow characteristics was compared with outcomes of primary surgical or balloon valve interventions. Characteristics studied included (1) annular dimension; (2) leaflet number; (3) leaflet excursion; and (4) degree of leaflet coaptation. Patients included those <20 years with a primary diagnosis of aortic stenosis (AS) and no other hemodynamically significant lesions at our institutions from 2000 to 2011. A total of 102 patients were included, 31 classified as having critical and 71 as having noncritical aortic stenosis. Of the patients, 79 were male, and 50 underwent primary catheter intervention. Echo parameters were compared with procedural outcome as defined by death or need for reintervention. Receiver operator curves were utilized to determine the point within each morphologic feature where the greatest difference occurred. This was utilized as the distinguishing point within each the morphologic group. Analysis was conducted separately for critical and noncritical aortic stenosis.
Kaplan-Meier analysis demonstrated no significant difference in time to reintervention or death whether initial palliation consisted of surgical commissurotomy or balloon valvuloplasty with respect to any of the morphologic characteristics studied.
Patients with AS do equally well with surgical commissurotomy or balloon valvuloplasty as initial palliation. This holds true for those with either critical or noncritical aortic stenosis. Valve morphology did not help in selection of initial palliative strategy. Current technologies should enable an improved selection of initial palliative approach through thoughtful, randomized trials.
© 2015 Wiley Periodicals, Inc.
... Following birth and shifting from fetal to neonatal circulation, the left ventricular muscle without endocardial fibroelastosis is able to stretch relative to the increase of cardiac output demanded for additional lower body perfusion [9]. Former studies evaluating left ventricular size in patients with aortic stenosis, have suggested that a minimal end-diastolic left ventricular volume of 20 mL/m 2 of body surface area enables a biventricular repair [15,16]. For patients without intrinsic valve stenosis much smaller ventricles, even in neonates with left ventricular end diastolic volume of 10 mL/m2 and non-apex forming left ventricle, significant growth of the left ventricular dimension has been proven [9][10][11]. ...
Leaving an inter-atrial communication (IAC) open for left atrial decompression is often recommended in neonates with aortic arch obstruction undergoing primary repair. In this study, outcomes in these patients were compared to those with intact atrial septum after repair.
Between 2000 and 2013, 53 consecutive neonates with severe aortic arch obstruction (hypoplasia: n = 45, interruption: n = 8) underwent primary repair from an anterior approach. Median age and weight were 8 days (range: 2-30) and 3.2 kg (range: 2.4-4.4), respectively. Cardiac morphology included a ventricular septal defect (VSD, large: n = 28, small: n = 7), malposition of great arteries (n = 10), and severe left ventricular outflow tract obstruction (LVOTO, n = 10). During corrective surgery IAC was closed (group-I, n = 37) or partially left-open (group-II, n = 16). Primary endpoints were hospital death, and re-intervention (surgery and/or balloon) due to aortic arch re-coarctation or recurrent LVOTO. Statistically significant variables by univariate analysis were incorporated in the corresponding multivariable regression model.
Regarding morphological discrepancies more patients in group-II presented with LVOTO (p = 0.05), or the combination of arch hypoplasia, intact ventricular septum and normal ventriculo-arterial connection (p = 0.017). Hospital mortality was 8.1% in group-I and 37.5% in group-II (p = 0.016). Re-intervention was performed in 13 patients (group-I: n = 6 vs. group-II: n = 7) due to aortic arch re-coarctation (n = 12) and/or recurrent LVOTO (n = 3), and resulted in a Kaplan-Meier freedom from re-intervention of 87 ± 6% and 79 ± 8% in group-I, and 64 ± 14% and 64 ± 14% in group-II after 1 and 5 years, respectively (p = 0.016). Multivariate analysis revealed LVOTO as an independent risk factor for hospital death (p = 0.042), whereas both LVOTO and left-open IAC (p = 0.001 and 0.01) were independent risk factors for re-intervention.
A left-open IAC increases risk of re-intervention at the left heart aorta complex. Sustained left-to-right shunting on atrial level seems to induce preload reduction of the often restrictive left ventricle leading to decreased aortic blood flow.
... It is usually dictated by the morphology of the aortic valve, the size and function of the left heart, the presence of associated defects and the overall condition and weight of the neonate. The key issue is to decide whether the left heart structures are adequate to sustain the systemic circulation, i.e. a biventricular repair, (22) and if so, what intervention will be the most beneficial for the individual neonate. (23,24) The pros and cons of each intervention have been the subject of many publications. ...
Management of severe aortic valve stenosis in the neonate AS may occur in isolation, or in association with other congenital cardiac defects. These include other left heart obstructive lesions in Shone association and the hypoplastic left heart syndrome (HLHS) as well as septal defects. It may also occur with extra-cardiac malformations of the renal, gastrointestinal and central nervous systems. (5) The LVOTO is mainly at the level of the annulus (70%) but may also occur at sub-valvar or supra-valvar levels. EMBRYOLOGY OF THE SEMI-LUNAR VALVES The semi-lunar valves develop from the fourth week of gestation. Neural crest cells from the fourth and sixth pharyngeal arches migrate into thetruncus arteriosus andconus cordis and transform into mesenchymal tissue that proliferates to form 2 truncoconal cushions. These 2 endocardial cushions or ridges appear opposite each other in the upper part of the truncus arteriosus, in the dextro-superior and sinistro-inferior positions, and fuse to form the truncal septum. Simultaneously, another 2 intercalated endo-cardial cushions form, each at 90º from the first 2 (Figure 1). Further cavitation of the ridges (Figure 2) form the 3 triangular-shaped leaflets in each outflow tract: the truncal septum differentiates to form the left and right aortic valve cusps and 2 of the leaflets of the pulmonary valve, and the additional 2 endocardial cushions also evolve, with the right cushion forming the posterior aortic valve cusp, and the left the anterior pulmonary valve leaflet. This occurs during the anti-clockwise rotation and caudal shifting of the cono-truncus. The formed semi-lunar valves are thus usually trileaflet with the pulmonary valve's cusps orientated left, right, and anterior and the aortic valve's cusps left, right and posterior. The endocardial ABSTRACT NEONATAL AORTIC VALVE STENOSIS INTRODUCTION
... Ou seja, o substrato anatômico nessas crianças de menor idade e baixo peso já é desfavorável por natureza. No estudo publicado por Lofland et al. 7 comparando os resultados da valvotomia cirúrgica e da valvoplastia aórtica, a ocorrência de insuficiência aórtica após o procedimento foi discretamente maior no grupo percutâneo, atingindo significância estatística limítrofe. ...
BACKGROUND: Balloon aortic valvuloplasty still remains a debatable procedure in the literature. In this paper, we report the immediate results of this procedure using the carotid access. METHOD: Longitudinal study of a cohort of patients with severe or critical congenital aortic valvular stenosis with up to six months of age treated with balloon aortic valvuloplasty from 1997 to 2008. RESULTS: Between March 1997 and March 2008, 25 procedures were performed in 24 patients (79% males) with mean age of 1.4 ± 1.0 months (0.1-4 months) and mean weight of 3.7 ± 1.2 kg (1.1-5.2 kg). The balloon-annulus ratio was 0.93 ± 0.1. There were 4 procedure-related deaths (17%), three of them due to left ventricular dysfunction and one due to the dissection of the left sinus of Valsalva during positioning of the guide wire and sheath prior valvuloplasty. The peak transvalvar systolic gradient at echocardiography decreased from 86 ± 25 mmHg to 36 ± 15 mmHg whereas the peak-to-peak gradient decreased from 61 ± 36 mmHg to 29 ± 17 mmHg after the procedure. There was significant improvement of ventricular function in patients with previous dysfunction. There was worsening or de-novo aortic failure in 10 patients, which did not require immediate surgical intervention. Ventricular tachycardia or ventricular fibrillation was observed in 4 patients. During follow-up, valve surgery was required in 2 of 7 patients. CONCLUSIONS: Although still associated to significant morbidity and mortality, balloon aortic valvuloplasty is effective in reducing transvalvar gradient and in preserving left ventricular function and may delay the need for surgical intervention.
Newborns with congenital heart disease often require interventions linked to high morbidity and mortality rates. In the last few decades, many transcatheter interventions have become the first-line treatments for some critical conditions in the neonatal period. A catheter-based approach provides several advantages in terms of procedural time, length of hospitalization, repeatability and neurodevelopmental issues (usually related to cardiopulmonary bypass). The main transcatheter procedures will be reviewed, as they are now valid alternatives to conventional surgical management.
Objective
To compare long-term survival, reinterventions and risk factors using strict definitions of neonatal critical and non-critical valvular aortic stenosis (VAS).
Design
A nationwide retrospective study using data from patient files, echocardiograms and the Swedish National Population Registry.
Setting and patients
All neonates in Sweden treated for isolated VAS 1994–2018. We applied the following criteria for critical aortic stenosis: valvular stenosis with duct-dependent systemic circulation or depressed left ventricular function (fractional shortening ≤27%). Indication for treatment of non-critical VAS was Doppler mean gradient >50 mm Hg.
Main outcome measures
Short-term and long-term survival, aortic valve reinterventions need of valve replacements, risk factors for reintervention and event-free survival.
Results
We identified 65 patients with critical VAS and 42 with non-critical VAS. The majority of the neonates were managed by surgical valvotomy. Median follow-up time was 13.5 years, with no patients lost to follow-up. There was no 30-day mortality. Long-term transplant-free survival was 91% in the critical stenosis group and 98% in the non-critical stenosis group (p=0.134). Event-free survival was 40% versus 67% (p=0.002) in the respective groups. Median time from the initial treatment to reintervention was 3.6 months versus 3.9 years, respectively (p=0.008).
Conclusions
Critical VAS patients had significantly higher need for reintervention during the first year of life, lower event-free survival and lower freedom from aortic valve replacement at age ≥18 years, compared with neonates with non-critical stenosis.
Cardiovascular magnetic resonance has been utilized in the management and care of pediatric patients for nearly 40 years. It has evolved to become an invaluable tool in the assessment of the littlest of hearts for diagnosis, pre-interventional management and follow-up care. Although mentioned in a number of consensus and guidelines documents, an up-to-date, large, stand-alone guidance work for the use of cardiovascular magnetic resonance in pediatric congenital 36 and acquired 35 heart disease endorsed by numerous Societies involved in the care of these children is lacking. This guidelines document outlines the use of cardiovascular magnetic resonance in this patient population for a significant number of heart lesions in this age group and although admittedly, is not an exhaustive treatment, it does deal with an expansive list of many common clinical issues encountered in daily practice.
Ongoing concerns with single-ventricle palliation morbidity and poor outcomes from primary biventricular strategies for neonates with borderline left heart structures have led some centers to attempt alternative strategies to obviate the need for ultimate Fontan palliation and limit the risk to the child during the vulnerable neonatal period. In certain patients who are traditionally palliated toward single-ventricle circulation, biventricular circulation is possible. This review aims to delineate the current knowledge regarding converting certain patients with borderline left heart structures from single-ventricle palliation toward biventricular circulation.
Hypoplastic left heart complex with “borderline left ventricle” and intact ventricular septum is a clinical conundrum for the congenital heart surgeon. The choice between neonatal biventricular repair and single ventricle palliation can be challenging, and the current tools to aid in the decision-making process fail to account for the morbidity associated with intermediate and late diastolic dysfunction. Staged ventricular recruitment, consisting of valvular repair techniques, resection of endocardial fibroelastosis, atrial septal restriction, and augmentation of pulmonary blood flow, has been shown to improve left ventricular size and function culminating in eventual biventricular circulation. Despite staged ventricular recruitment, some patients cannot undergo biventricular conversion. Strategies to address these complex patients, including the “reverse” double switch, are the next frontier in biventricular repair.
Severe aortic stenosis can be accompanied by various degrees of left ventricular underdevelopment. The assessment whether a borderline‐sized left ventricle can or cannot support the systemic circulation is crucial. The decision‐making still remains challenging. We present a case that illustrates that the development of haemodynamic parameters can be difficult to estimate, even in the long term. The patient went from biventricular to univentricular circulation and back and could finally be palliated by heart transplantation. Modern technology including long‐term mechanical cardiac support as a bridge to candidacy and drug therapy for pulmonary hypertension were vital to successfully combat a previously lethal disease.
When do we label a left ventricle as small? How is the decision made regarding suitability for a two-ventricle repair? Are dimensions the only criteria with which we decide, whether a ventricle will support the systemic circulation? Can we actually stimulate the growth of a borderline small left ventricle, so that it could support the systemic circulation in future? What role does mass and shape have to play in whether a borderline ventricle will support a biventricular repair? What role does the morphology and segmental anatomy play in this decision-making? This is a review article to address these issues.
Aims:
The aims of this study were to provide a detailed descriptive analysis of pre-intervention morphologic and functional echocardiographic parameters in a large, unselected, multicentre cohort of neonates diagnosed with critical left heart obstruction and to compare echocardiographic features between the different subtypes of left-sided lesions.
Methods and results:
Pre-intervention echocardiograms for 651 patients from 19 Congenital Heart Surgeons' Society (CHSS) institutions were reviewed in a core lab according to a standardized protocol including >150 morphologic and functional variables. The four most common subtypes of lesions were: aortic atresia (AA)/mitral atresia (MA) (29% of patients), AA/mitral stenosis (MS) (20%), aortic stenosis (AS)/MS (26%), and isolated AS (iAS) (18%). Only 17% of patients with AS/MS had an apex-forming left ventricle, compared with 0% of those with AA/MA and AA/MS (P < 0.0001). Aortic arch hypoplasia and coarctation were common across all four groups, while those with AA/MA and AA/MS had the smallest ascending aorta diameters. Flow in the ascending aorta was retrograde in 43% and 10% of the patients with AS/MS and iAS, respectively. The right ventricle was apex forming in 100% of patients with AA/MA and AA/MS, 96% with AS/MS and 70% with iAS (P < 0.0001). Moderate to severe tricuspid regurgitation was present in 13% of all patients.
Conclusion:
This large multi-institutional study generates insight into the distribution of the functional and morphologic spectrum in patients with critical left-sided heart disease and identifies differences in these functional and morphologic characteristics between the main anatomic subtypes of critical left heart obstruction.
The history of the first 41 years of the Congenital Heart Surgeons Society (CHSS) was recorded in 2015 which chronicled the metamorphosis of a small informal meeting into a mature organization with bylaws, officers, committees, funded research, the Kirklin-Ashburn Fellowship, and a vision to become the premier organization of congenital heart surgery in North America. Chief among these transformations was the implementation and development of the CHSS Data Center. Member participation, fellowship education, and significant outcomes research have been the hallmark of the CHSS. This incremental historical review highlights continued CHSS sentinel advances. Fifty-three CHSS Data Center manuscripts have been published. Citation scores (number of literature citations that each manuscript has accrued) have been collated and analyzed by cohort study. The average citation score for all manuscripts was 75.4 ± 76.3 (range: 1-333). The Kirklin/Ashburn Fellowship continues to thrive with academic achievements and generous contributions to the endowment. The World Journal of Pediatric and Congenital Heart Surgery has been adopted as the official organ of the CHSS. A Past President's Dinner has been inaugurated serving as a senior advisory committee to the Executive Council. Toronto Work Weekends continue. Congenital Heart Surgeons Society growth has accrued to 159 active members and 82 institutional members. Future considerations include the size, content, and duration of the annual meeting; the potential for increased membership; and political penetrance into national cardiothoracic governing organizations regarding committee appointments, executive council representation, and education initiatives. Congenital Heart Surgeons Society has achieved numerous advances during this incremental period.
Background:
Infants with critical left sided obstructive lesions usually present with left ventricle dysfunction and pulmonary arterial hypertension (PAH). Left ventricular dysfunction and pulmonary artery pressures usually normalize after relief of obstruction. In some, PAH persists despite adequate relief of obstruction.
Patients and methods:
We retrospectively reviewed records of infants (less than 3 months) who underwent intervention for left sided obstruction (n=50) over four years (May 2012-May 2016).
Result:
We report four infants who had persistent of PAH despite relief of left sided obstruction. The causes of PAH were found to be high left ventricular end diastolic pressure(n=1), severe hypoplasia of pulmonary veins (n=1), stenosis of the lower branch of the right pulmonary artery (n=1) and non-regression of pulmonary vascular resistance (n=1).
Conclusion:
That persistence of PAH after relief of obstruction should be investigated and other, less common causes should be sought.
Valvular aortic stenosis represents the most common congenital anomaly of the aortic valve. As such, this chapter focuses predominantly on this lesion’s diverse clinical presentations, pathophysiology, and current management strategies. Due to the marked differences between neonatal and childhood or adolescent valvular stenosis, anesthetic considerations are discussed in the context of each presentation. As a separate section within this chapter, supravalvular aortic stenosis is also reviewed. Though not indicated by its name, patients with supravalvular stenosis may also have stenosis at the valvular level (among other areas). Moreover, due to this lesion’s association with anesthetic morbidity and mortality, it warrants special attention. To assist in risk stratification and anesthetic planning, an overview of potential risk factors and pitfalls is presented.
A borderline left ventricle in a neonate is an ill-defined term; it suggests that if a biventricular (BV) circulation is anticipated, it is at an increased risk for morbidity and mortality. Hybrid procedures for hypoplastic left heart syndrome (HLHS) have allowed new treatment strategies to be developed. Right ventricle (RV) assist of left ventricle by means of the creation of a percutaneous or surgical shunt in combination with bilateral pulmonary artery banding allows time for catch-up growth of the left heart. In cases of failure of attempt at biventricular circulation, a surgical right ventricle assist of left ventricle may be considered as a bail-out procedure. In this chapter this management strategy is discussed.
The state of the pulmonary vascular bed impacts on the outcome of CHD. Both active vasoconstriction and pathological remodeling conspire to increase PVR. The combination of increased pulmonary blood flow and pulmonary artery pressure is a potent stimulator of pulmonary vascular disease. With the exception of a few high-risk malformations (e.g., d-TGA and VSD), permanent or progressive pathological changes seldom occur when malformations are corrected in the first 1-2 years of life. Patients with Eisenmenger syndrome were for a long time therapeutic orphans. However, the availability of orally administered therapies has resulted in the inclusion of patients with Eisenmenger syndrome in randomized controlled trials. New insights into pulmonary vascular biology, improved understanding of genetic predisposition, the development of therapies that engage novel pathways, and the improved delivery of medical and surgical care in underprivileged areas will, we hope, substantially reduce the medical burden caused by pulmonary vascular disease associated with congenital heart disease.
A series of technical refinements over the past 30 years, in combination with advances in perioperative management, have resulted in dramatic improvements in the survival of patients with univentricular heart. While the goal of single-ventricle palliation remains unchanged - normalization of the pressure and volume loads on the systemic ventricle, the strategies to achieve that goal have become more diverse. Optimal palliation relies on a thorough understanding of the changing physiology over the first years of life and the risks and consequences of each palliative strategy. This review describes how to optimize surgical decision-making in univentricular patients based on a current understanding of anatomy, physiology, and surgical palliation.
What is the best treatment for the child with valvar aortic stenosis - balloon or surgical valvotomy?
Operations for left ventricular outflow tract abnormalities are centred on hemodynamic conditions that relate to subvalvar stenosis, valvar stenosis/regurgitation, aortic annular hypoplasia, and supravalvar aortic stenosis. Operative interventions over the years have evolved because the intervening outcomes proved to be unsatisfactory. The resection for subvalvar aortic stenosis has progressed from a fibrous "membrane" resection to a more extensive fibromuscular resection. Operative solutions for valvar aortic stenosis and regurgitation have resulted in operative interventions that depend on simple commissurotomy, leaflet extensions, prosthetic mechanical valve replacement, biologic valve replacement, including the pulmonary autograft, and operations to treat aortic annular stenosis. Although there are enthusiastic proponents for all of these strategies, the fact remains that none have proven to be curative; patients can expect to undergo further procedures during their lifetimes. The short- and mid-term solutions to these left ventricular outflow tract abnormalities have improved based on operations that have been attended by increasing operative complexity. The purpose of this review is to chronicle the operative steps of the Ross operation, the Konno-Rastan operation, the modified Konno operation, the Ross-Konno operation, and the modified Ross-Konno operation.
Objectives
To better understand the natural history and spectrum of fetal aortic stenosis (AS), we aimed to 1) determine the prenatal diagnosis rate of neonates with critical AS and a biventricular (BV) outcome; and 2) describe the findings at fetal echocardiography in prenatally diagnosed patients.MethodsA multi-center, retrospective study was performed from 2000 to 2013. Neonates with critical AS who were discharged with a BV outcome were included. The prenatal diagnosis rate was compared to that reported for hypoplastic left heart syndrome (HLHS). Fetal echocardiographic findings in prenatally diagnosed patients were reviewed.ResultsOnly 10 of 117 neonates (8.5%) with critical AS and a BV outcome were diagnosed prenatally, a rate significantly lower than that for HLHS in the contemporary era (82%; p < 0.0001). Of the 10 patients diagnosed prenatally, all developed LV dysfunction by a median gestational age of 33 weeks (range, 28-35). When present, Doppler abnormalities such as retrograde flow in the aortic arch (n = 2), monophasic mitral inflow (n = 2), and left to right flow across the foramen ovale (n = 8) developed late in gestation (median 33 weeks).Conclusion
The prenatal diagnosis rate among neonates with critical AS and a BV outcome is very low, likely due to a relatively normal 4-chamber view in mid-gestation with development of significant obstruction in the 3rd trimester. This natural history contrasts with that of severe mid-gestation AS with evolving HLHS and suggests that the timing in gestation of significant AS has an important impact on subsequent left heart growth in utero.
Background
: The term “borderline left ventricle” describes a small left heart that may be inadequate to provide systemic cardiac output and implies the potential need for a single-ventricle palliation. The aim of this study was to identify foetal echocardiographic features that help discriminate which infants will undergo single-ventricle palliation versus biventricular repair to aid in prenatal counselling.
Methods
: The foetal database at our institution was searched to identify all foetuses with borderline left ventricle, as determined subjectively by a foetal cardiologist, from 2000 to 2011. The foetal images were retrospectively analysed for morphologic and physiologic features to determine which best predicted the postnatal surgical choice.
Results
: Of 39 foetuses identified with borderline left ventricle, 15 were planned for a univentricular approach, and 24 were planned for a biventricular approach. There were significant differences between the two outcome groups in the Z-scores of the mitral valve annulus, left ventricular end-diastolic dimension, aortic valve annulus, and ascending aorta diameter (p<0.05). With respect to discriminating univentricular outcomes, cut-offs of mitral valve Z-score ⩽−1.9 and tricuspid:mitral valve ratio ⩾1.5 were extremely sensitive (100%), whereas a right:left ventricular end-diastolic dimension ratio ⩾2.1 provided the highest specificity (95.8%).
Conclusion
: In foetuses with borderline left ventricle, a mitral valve Z-score ⩾−1.9 or a tricuspid:mitral valve ratio ⩽1.5 suggests a high probability of biventricular repair, whereas a right:left ventricular end-diastolic dimension ratio ⩾2.1 confers a likelihood of single-ventricle palliation.
Objectives
Multicentre registries facilitate studies of rarer conditions, but may introduce unidentified confounders. Fetal aortic valvuloplasty may prevent progression of aortic stenosis to hypoplastic left heart syndrome and allow biventricular rather than univentricular postnatal treatment. We investigate whether a blinded, simulated multidisciplinary team (MDT) approach aids interpretation of multicentre data to uncover institutional bias in postnatal decision-making following fetal cardiac intervention (FCI).Methods
The MDT, blinded to FCI, institutional location and postnatal treatment assigned a surgical pathway to 109 neonates diagnosed prenatally with aortic stenosis in 13 European countries, where 32 had undergone FCI. MDT decisions were the numerical consensus of silent voting with case review where a decision was split. Funnel plots showing concordance between MDT and local team's surgical choice (first pathway) and with outcome (final pathway) were created.Results105 had biventricular or univentricular decision with 4 undecided. Blinded MDT consensus for first pathway matched surgical centre decisions in 93/105 (89%) with no difference in agreement in those undergoing FCI (n = 32) and no (74) or unsuccessful (3) valvuloplasty (no-FCI) (Kappa 0.73, 95% CI 0.38-1.00 vs 0.74, 95% CI 0.51-0.96). However funnel plots comparing MDT individual decisions with local teams’ display more discordance (meaning biventricular – univentricular conversion) for final surgical pathway following FCI than no-FCI (36/74 vs 34/130), p = 0.002 and identified one outlying centre. The important difference was a deficiency of their integrated surgical programme.Conclusions
This method may improve interpretation of outcomes in multicentre studies by identifying and investigating outliers identified in funnel plots.
Adults with repaired coarctation of the aorta (CoA) are at risk for premature cardiovascular death or heart failure (HF). We sought to evaluate risk factors for death or HF in young adults with repaired CoA in childhood.
The medical records of a cohort of 159 adults diagnosed with CoA repaired at a mean age of 4.1±5.7years were retrospectively reviewed to identify predictors of a combined endpoint of all cause death or hospitalisation for HF by using Cox proportional hazard models.
Over a follow-up of 26±8years (median 27years) after repair, 5 patients died and 7 developed HF requiring hospitalisation. Pulmonary artery systolic pressure >40mmHg (PH) was the main predictor of death or admission for HF (HR 32; 95% CI 4.0-250; p=0.001). Neither systemic hypertension, recoarctation, aortic aneurysm, intracardiac lesions nor treatment with beta-blockers or ACEi/ARBs were statistically related to death or HF. Restrictive left ventricular physiology (RLVP) was the main predictor of PH by logistic regression analysis (OR 31; 95% CI 10-92; p<0.001). Patients with severe PH (pulmonary artery systolic pressure >60mmHg) showed RLVP (9/10), severe elevation of end-diastolic LV pressure at cardiac catheterization (8/8) and subendocardial late gadolinium enhancement on MRI (5/5). Pathological examination of an explanted heart demonstrated extensive left ventricular subendocardial fibrosis.
Pulmonary hypertension related to restrictive left ventricular physiology was the strongest predictor of death or HF in young adults with CoA repaired in childhood. Persistent subendocardial fibrosis might be the structural substrate in some patients.
La obstrucción congénita al vaciamiento del ventrículo izquierdo, consistente en el estrechamiento a uno o varios niveles de su porción de salida condicionando sobrecarga de presión, supone el 5-6% del total de las cardiopatías congénitas.
Las posibles manifestaciones clínicas (insuficiencia cardíaca, angina, síncope y/o endocarditis) son variables en frecuencia y momento temporal de presentación, si bien hoy en día la mayoría de pacientes se intervienen estando todavía asintomáticos. Las formas severas de estenosis aórtica valvular son las de debut clínico más precoz. La estenosis aórtica subvalvular es rara en neonatos y lactantes y más frecuente en niños pequeños. En muchas ocasiones se asocia a estenosis aórtica valvular. La estenosis aórtica supravalvular es la más infrecuente de las tres formas de estenosis aórtica congénita, la de presentación clínica más tardía y tiene una fuerte asociación con el síndrome de Beuren-Williams.
La ecocardiografía sigue siendo la prueba diagnóstica de elección en la evaluación pre y postoperatoria de la obstrucción congénita a la vía de salida izquierda.
Se revisan las distintas opciones técnicas quirúrgicas para reparar estas cardiopatías y sus resultados hospitalarios y en seguimiento tardío
Purpose:
The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) is the largest Registry in the world of patients who have undergone congenital and pediatric cardiac surgical operations. The Congenital Heart Surgeons' Society Database (CHSS-D) is an Academic Database designed for specialized detailed analyses of specific congenital cardiac malformations and related treatment strategies. The goal of this project was to create a link between the STS-CHSD and the CHSS-D in order to facilitate studies not possible using either individual database alone and to help identify patients who are potentially eligible for enrollment in CHSS studies.
Methods:
Centers were classified on the basis of participation in the STS-CHSD, the CHSS-D, or both. Five matrices, based on CHSS inclusionary criteria and STS-CHSD codes, were created to facilitate the automated identification of patients in the STS-CHSD who meet eligibility criteria for the five active CHSS studies. The matrices were evaluated with a manual adjudication process and were iteratively refined. The sensitivity and specificity of the original matrices and the refined matrices were assessed.
Results:
In January 2012, a total of 100 centers participated in the STS-CHSD and 74 centers participated in the CHSS. A total of 70 centers participate in both and 40 of these 70 agreed to participate in this linkage project. The manual adjudication process and the refinement of the matrices resulted in an increase in the sensitivity of the matrices from 93% to 100% and an increase in the specificity of the matrices from 94% to 98%.
Conclusion:
Matrices were created to facilitate the automated identification of patients potentially eligible for the five active CHSS studies using the STS-CHSD. These matrices have a sensitivity of 100% and a specificity of 98%. In addition to facilitating identification of patients potentially eligible for enrollment in CHSS studies, these matrices will allow (1) estimation of the denominator of patients potentially eligible for CHSS studies and (2) comparison of eligible and enrolled patients to potentially eligible and not enrolled patients to assess the generalizability of CHSS studies.
The most appropriate management of aortic stenosis in children remains controversial. Both balloon and surgical valvotomy
are firmly established as effective initial treatments with encouraging survival rates even in the troublesome neonatal group.
Improved early results are based rather on the better understanding of the limits of a biventricular repair than on the method
of treatment. Valvotomy of any kind is a palliative procedure and reintervention remains frequent. Direct surgical intervention,
where exact splitting of fused commissures and shaving off of obstructing nodules can produce a better valve with maximum
valve orifice without causing regurgitation, might offer superior longer-lasting results in comparison with blind ballooning.
Since the description by Lababidi in 1983 of balloon aortic valvuloplasty, it has been used extensively by several groups of workers for relief of valvar aortic stenosis of moderate to severe degree. The indications for the procedure are peak-to-peak gradients ≥ 50 mmHg with symptoms or ECG changes or a gradient of ≥ 70 mmHg irrespective of the symptoms and ECG changes. One or more balloon catheters are placed across the aortic valve, over an extra-stiff guidewire and balloon inflated, producing aortic valvuloplasty. The recommended balloon: annulus ratio is 0.8:1.0. Immediate reduction of peak-to-peak gradient along with a fall of left ventricular peak systolic and end-diastolic pressures occurs. Rarely, significant aortic insufficiency, particularly in the neonate, may develop. At intermediate-term follow-up, catheter-measured peak-to-peak gradients and Doppler-measured peak instantaneous gradients remain low for the group as a whole. However, nearly one-quarter of the patients may develop restenosis, defined as peak-to-peak gradient ≥ 50 mmHg. The restenosis is successfully treated with surgical or repeat balloon valvotomy. Predictors of restenosis were age ≤ 3 years and an immediate postvalvuloplasty aortic valve gradient ≥ 30 mmHg. Comparison with surgical results is difficult, but overall, the balloon therapy appears to carry less morbidity. Long-term follow-up data are scanty. The limited data suggest low Doppler peak instantaneous gradients, minimal additional restenosis beyond what was observed at intermediate-term follow-up, and progression of aortic insufficiency in nearly one-quarter of patients. Event-free rates are 76% and 60%, respectively, at 5 and 10 years following initial balloon valvuloplasty. Based on immediate and intermediate-term follow-up data, balloon aortic valvuloplasty appears to produce reasonably good results, avoiding/postponing the need for surgical intervention. The late follow-up data are of some concern in that significant aortic insufficiency with left ventricular dilatation may develop, and some require surgical intervention. Prospective studies on larger groups of children and careful comparison with long-term follow-up surgical data may be necessary to make a definitive recommendation that balloon aortic valvuloplasty is the therapeutic procedure of choice.
Objectives:
Increased afterload and multilevel LV obstruction is constant. We assumed that restoration of normal loading conditions by relief of LV obstructions promotes its growth, provided that part of the cardiac output was preoperatively supported by the LV, whatever the echocardiographic indexes.
Background:
Whether to perform uni- or biventricular repair in ducto dependent neonates with hypoplastic but morphologically normal LV (hypoplastic left heart syndrome classes II & III) remains unanswered. Echocardiographic criteria have been proposed for surgical decision.
Methods:
Twenty ducto dependent neonates presented with this anomaly. All had aortic coarctation associated to multilevel LV obstruction. Preoperative echocardiographic assessment showed: mean EDLW of 12.4 +/- 3.03 ml/m2 and mean Rhodes score of -1.73 +/-0.8. Surgery consisted in relief of LV outflow tract obstruction by coarctation repair in all associated to aortic commissurotomy in one and ASD closure in 2.
Results:
There were 3 early and 2 late deaths. Failure of biventricular repair and LV growth was obvious in patients with severe anatomic mitral stenosis. The other demonstrated growth of the left heart. At hospital discharge the EDLVV was 19.4+/-3.12 ml/m2 (p = 0.0001) and the Rhodes score was -0.38+/-1.01 (p = 0.0003). Actuarial survival and freedom from reoperation rates at 5 years were 72.5% and 46%, respectively.
Conclusions:
Biventricular repair can be proposed to ducto dependent neonates with hypoplastic but morphologically normal LV provided that all anatomical causes of LV obstruction can be relieved. Secondary growth of the left heart then occurs; however, the reoperation rate is high.
To assess the results of balloon dilatation of the aortic valve as first line treatment for infants presenting in the first two months of life with cardiac failure caused by severe aortic stenosis.
A prospective study of all such infants presenting to a single centre over a three year period.
Of 14 consecutive infants aged < 2 months presenting with heart failure due to severe stenosis of the aortic valve, 12 underwent balloon dilatation of the valve. Two infants with hypoplastic left heart syndrome were excluded. Six patients had other significant cardiac malformations, nine had endocardial fibroelastosis. Four infants were moribund at presentation.
Balloon dilatation was performed through the femoral artery under general anaesthetic, with heparin and antibiotic prophylaxis. Maximum inflated balloon size was selected as equal to or slightly less than the diameter of the aortic valve measured echocardiographically. In two infants balloon dilatation of coexistent severe coarctation was performed at the same time.
Dilatation was achieved in all cases. Early survival rate (up to one month) was 75%; 50% survive up to three years. The two deaths occurring < 24 hours after dilatation probably related to the infants' poor condition at presentation and the presence of significant left ventricular hypoplasia. The other early death was due to severe mitral regurgitation from papillary muscle dysfunction. All later deaths were related to associated malformations. The difference in left ventricular size (diameter and length) measured echocardiographically at presentation between survivors and those that died is significant (p < or = 0.002). 83% of deaths occurred in infants with associated malformations. Cusp perforation occurred in one infant, which may have contributed to this patient's death. Left ventricular perforation and cardiac tamponade were treated successfully by prompt pericardiocentesis in another.
Balloon dilatation of the aortic valve is an acceptable first line treatment for neonates with severe symptomatic stenosis of the aortic valve. Outcome depends principally on left ventricular size and on the presence, severity, and timing of treatment of associated malformations. Severe endocardial fibroelastosis was not a risk factor for early death but may have implications for long term survival. The survival rate for this high risk group might be improved by a stratified approach where patients with adequate left ventricular size have balloon dilatation whereas those with severe ventricular hypoplasia are managed like patients with the hypoplastic left heart syndrome.
With the current trend to performing surgical valvotomy for infantile aortic stenosis without cardiac catheterization, there is a need to develop echocardiographic criteria for adequacy of left ventricular size. The echocardiograms and catheterization data of all 25 infants <3 months of age undergoing aortic valvotomy for isolated aortic valve stenosis from September 1980 through July 1990 were reviewed. Significant differences (p < 0.05) between the survivors and nonsurvivors were noted for age at operation (30 ± 28 vs. 3 ± 1.5 days), mitral valve diameter (10.1 ± 1.7 vs. 7.7 ± 1.5 mm), left ventricular end-diastolic dimension (18.4 ± 0.4 vs. 11.4 ± 3 mm), left atrial dimensions (15.3 ± 3.8 vs. 10 ± 2.4 mm). left ventricular cross-sectional area on the parasternal long-axis echocardiogram (4 ± 1.9 vs. 2 ± 1.9 cm2) and angiographically determined left ventricular end-diastolic volume (43 ± 23 vs. 11 ± 5 ml/m2).There was no difference with respect to patient weight, body surface area, aortic root dimension or left ventricular ejection fraction. Left ventricular cross-sectional area <2 cm2 as measured on the parasternal long-axis echocardiogram was found in 5 of 7 nonsurvivors and 0 of 12 survivors, making this a risk factor for perioperative death (p < 0.05). Left ventricular end-diastolic dimension <13 mm was found in 5 of 6 nonsurvivors and 2 of 17 survivors, making this another risk factor for early mortality (p < 0.05). There was good correlation between angiographic left ventricular end-diastolic volume and both left ventricular cross-sectional area and end-diastolic dimension. It is concluded that a left ventricular cross-sectional area <2 cm2 in infants with critical aortic stenosis is useful as an adjunct in assessing the critical left ventricular size that is not adequate for survival after valvotomy.
Critical aortic stenosis (CAOS) is not compatible with life when the ductus arteriosus closes. We have treated 11 consecutive cases with isolated CAOS. Symptom presentation was in the early neonatal period and diagnosis was made noninvasively at a mean age of 4 days. All were operated on with transventricular dilation (TVD) at a mean age of 4.7 days. There was no early mortality. There were two late deaths due to fibroelastosis. Both had the smallest aortic anulus diameter (5 mm). Two other patients had aortic root replacement, one at the age of 6.5 weeks due to intractable heart failure, and the other at the age of 3 months due to increasing gradient. In these two cases elective surgery was made possible by a successful TVD in the early neonatal period. TVD in this material was not associated with any early mortality, which makes this procedure a good alternative in the treatment of CAOS.
The clinical presentation of infants with critical aortic stenosis, as well as the results of surgical treatment, differs from obstruction of the left ventricular outflow tract in older children. To investigate a possible anatomic basis for this situation, we performed a detailed morphometric study of 21 hearts from infants who had critical aortic stenosis and 11 normal hearts from infants less than 3 months of age. In each of the hearts with critical aortic stenosis, only one commissure extended to the sinutubular ridge. The other two commissures were represented by folds in the aortic wall that suspended the leaflet below the level of the sinutubular junction. The leaflet thus had a free edge shorter than the circumference of the sinus, in contrast with the normal valve, in which leaflets always were longer than the circumference of their supporting sinus. Analysis of the fibrous triangles on the ventricular aspect of abnormal valves showed a symmetric three-sinus arrangement. In all but one specimen, however, only the triangle related to the mitral valve was fully developed. Although incision of both rudimentary commissures to the aortic wall should achieve some relief of obstruction, these morphologic features strongly mitigate against surgical restoration of normal function or growth in aortic valves having the morphology observed in this series of hearts.
Critical aortic stenosis (AS) in the neonate represents a particularly severe form of AS where normal compensatory mechanisms are unable to maintain homeostasis, even at rest. Critical AS may be defined as aortic valve stenosis with congestive heart failure and signs of decreased systemic cardiac output.1 When patients present in infancy with aortic valve stenosis and congestive heart failure, medical management is frequently unsuccessful. Various surgical procedures using both open and closed heart techniques have been attempted to relieve critical AS in infancy.2–7 Percutaneous transluminal balloon valvuloplasty has been performed in patients with critical AS in infancy.7 This procedure is technically difficult and at times impossible. The use of balloon valvuloplasty in the operating room has been described for the treatment of AS.8,9 The technical aspects of intraoperative balloon valvuloplasty and the range of application are still being investigated. Reported complications include severe aortic regurgitation and disruption of an aortic valve cusp. This report describes the use of intraoperative balloon valvuloplasty in 4 neonates who presented with critical AS in the first week of life.
Failure of infants with critical aortic stenosis to survive after adequate valvotomy despite a left ventricular size that appears to be adequate indicates that additional preoperative anatomic features may contribute to mortality.
Discriminant analysis was used to determine which of several echocardiographically measured left heart structures were independent predictors of survival after valvotomy for neonatal critical aortic stenosis. It was possible to predict outcome after classic valvotomy (two-ventricle-type repair) with 95% accuracy based on mitral valve area, long-axis dimension of the left ventricle relative to the long-axis dimension of the heart, diameter of the aortic root, and body surface area. Left ventricular volume was not a major determinant in this study, in part because patients who had initial valvotomy had been preselected in favor of an adequately sized left ventricle. Patients with multiple small left ventricular structures were found to have significantly improved survival after initial Norwood operation. In contrast, balloon valvotomy with subsequent Norwood procedure was usually unsuccessful.
The adverse effects of small inflow, outflow, and/or cavity size of the left ventricle are cumulative. The accuracy of prediction of outcome based only on preoperative anatomy indicates that adequacy of valvotomy is not generally a limiting factor for survival in this group of patients. It is possible to identify subjects whose chance of survival is better after a Norwood procedure rather than valvotomy, even if left ventricular volume is not critically small.
Between 1986 and July 1990, balloon valvuloplasty was attempted in eight newborns (less than 28 days of age) with isolated critical aortic valve stenosis. Balloon valvuloplasty could not be successfully accomplished in any of the three infants presenting before 1989. Since March 1989, when improved catheter technology became available, all five neonates presenting with critical aortic stenosis were treated successfully by balloon valvuloplasty. A transumbilical approach was utilized in all four infants in whom umbilical artery access could be obtained. One newborn who was 25 days of age underwent transfemoral balloon valvuloplasty. Balloon valvuloplasty was immediately successful in all five newborns, as evidenced by a decrease in valve gradient and improvement in left ventricular function and cardiac output. Peak systolic gradient was reduced by 64% from 69 +/- 8 to 25 +/- 3 mm Hg (p = 0.005). Left ventricular systolic pressure decreased from 128 +/- 9 to 95 +/- 9 mm Hg (p = 0.02) and left ventricular end-diastolic pressure decreased from 20 +/- 2 to 11 +/- 1 mm Hg (p = 0.02). Moderate (2+) aortic regurgitation was documented in two infants after valvuloplasty. The time from first catheter insertion to valve dilation averaged 57 +/- 14 min (range 26 to 94) and the median length of the hospital stay was 4 days. With the use of recently available catheters, the transumbilical technique of balloon valvuloplasty can be performed quickly, safely and effectively in the newborn with critical aortic stenosis. It does not require general anesthesia, cardiopulmonary bypass or a left ventricular apical incision and it preserves the femoral arteries for future transcatheter intervention should significant aortic stenosis recur.
Balloon valvuloplasty in neonates with severe aortic valve stenosis is limited by difficulties in catheter manipulation around the arch and across the valve and by the risk of femoral artery complications. A right common carotid artery cutdown was utilized for balloon aortic valvuloplasty in five neonates 1 to 20 days of age, weighing 3.1 to 3.9 kg. Standard balloon valvuloplasty was performed through a 6F sheath inserted in the right carotid artery. The arteriotomy was repaired at the end of the procedure. Mean left ventricular systolic pressure was reduced from 142 to 97 mm Hg, with a decrease in mean peak systolic pressure gradient from 76 to 33 mm Hg. Only one patient developed mild aortic regurgitation. One patient with a hypoplastic left ventricle died, and one patient required open valvotomy. All four survivors have a normal carotid pulse and no neurologic sequelae. Two of these patients required repeat balloon dilation to treat residual aortic valve stenosis at 8 and 10 months of age, respectively. Balloon valvuloplasty using a carotid artery approach is feasible and was safe in five neonates with severe aortic valve stenosis.
Percutaneous balloon valvuloplasty was attempted in 10 newborn infants with critical aortic valve stenosis and severe congestive heart failure. Three had a very small left ventricle and aortic anulus. In one infant, the aortic valve could not be passed, and in another infant, a technical error resulted in severe valvular damage, aortic insufficiency and death. Among the eight patients who had effective dilation, the stenosis was relieved in seven as assessed by a significant decrease in transvalvular pressure gradient, improvement of left ventricular contraction and eventual inversion of the ductal shunting. The procedure failed in the only patient whose dilation was performed with an undersized balloon. Aortic insufficiency occurred in three infants and was severe (perforated cusp) in one, moderate in one whose valve was dilated with an excessively large balloon and mild and transient in one. None of the three infants with a very small left ventricle recovered (two died and one underwent cardiac transplantation). Among the seven infants with a left ventricle of acceptable size, three underwent subsequent aortic valvotomy; one of these died and two bad good results. The remaining four are doing well 16 +/- 5 months later (mean +/- SD) with mild to moderate residual aortic stenosis and normal left ventricular function. In conclusion, percutaneous balloon valvuloplasty is an acceptable alternative to surgery in neonates with critical aortic valve stenosis. Incidence of complications and good relief of the obstruction depend on a careful technique. Immediate results are similar to those of surgery. Late prognosis depends on the quality of the left heart structures.
This article has no abstract; the first 100 words appear below.
Hypoplastic left heart syndrome constitutes a spectrum of severe congenital cardiovascular anomalies dominated by underdevelopment or absence of the left ventricle, aortic valve, and mitral valve. The ascending and arch segments of aorta are usually of very small caliber, and a diaphragm-like segment of aortic coarctation typically occupies what should be the preductal aortic isthmus. The ductus arteriosus empties directly into a descending thoracic aorta of normal caliber. The surgical morphologic nature of this complex syndrome has been clearly described.¹²³⁴ These developmental malformations are a prelude to fatal postnatal pathophysiologic processes. Survival after birth depends entirely on adequate systemic blood . . .
We are indebted to a large circle of University colleagues and friends, in particular Dick Schaefer, Richard Weismeyer, and their staffs, and Cheryl Mathis, R.N., heart-transplant coordinator.
Source Information
From the Departments of Surgery, Pathology, Anesthesia, and Pediatrics, Loma Linda University School of Medicine, Loma Linda, Calif. Address reprint requests to Dr. Bailey at the Department of Surgery, Loma Linda University Medical Center, Loma Linda, CA 92354–2804.
Two infants, aged 36 days old (Case 1) and 18 days old (Case 2) with interrupted aortic arch types B and A, respectively, and with severe aortic stenosis, were successfully operated on by use of pulsatile cardiopulmonary bypass. The great arteries were normally related in Case 1 and were transposed in Case 2. Repair involved the following procedure: ligation of the patent ductus arteriosus, restoration of aortic continuity with an 8 mm polytetrafluoroethylene graft, placement of an internal patch to tunnel all left ventricular blood from the left ventricle through the ventricular septal defect into the pulmonary artery in Case 1 and patch closure of the ventricular septal defect in Case 2, transection of the main pulmonary artery, anastomosis between the proximal pulmonary artery and the ascending aorta, and interposition of a valved conduit between the right ventricle and the distal pulmonary artery. The operative field could be approached easily through a median sternotomy. Postoperative cardiac catheterization revealed satisfactory anatomical and hemodynamic results in both cases.
Aortic valvotomy for critical aortic stenosis in infancy has had a high mortality. To determine the factors that influence survival, we reviewed the cases of 24 infants who underwent aortic valvotomy in the first 6 months of life (mean 4 1/2 weeks) for aortic stenosis from 1978 to 1984. Cardiopulmonary bypass was used in all patients. Operative mortality was 21% (5/24), four of the five deaths occurring from low cardiac output. Analysis of preoperative factors affecting survival versus nonsurvival revealed that low ejection fraction (60% +/- 17% in survivors versus 36% +/- 2% in nonsurvivors), high left ventricular end-diastolic pressure (16 +/- 7 mm Hg in survivors versus 30 +/- 14 mm Hg in nonsurvivors), and presence of endocardial fibroelastosis (25% in survivors versus 100% in nonsurvivors) all were predictive of a poor outcome, although the small sample size indicated caution in interpreting results. Factors that did not appear to influence survival included peak systolic gradient (79 +/- 30 mm Hg in survivors versus 60 +/- 15 mm Hg in nonsurvivors) and left ventricular end-diastolic volume (37 +/- 17 cm3/m2 in survivors versus 36 +/- 7 cm3/m2 in nonsurvivors). Four patients with a left ventricular end-diastolic volume below 26 cm3/m2 survived. Postoperative gradients averaged 25 +/- 21 mm Hg at 3.4 +/- 2 years' follow-up in nine recatheterized patients. Ejection fraction of these patients increased from 45% +/- 10% to 70% +/- 11% and left ventricular end-diastolic volume increased from 37 +/- 17 to 58 +/- 5 cm3/m2. Two of 17 patients have required apical-aortic conduits; all other patients are asymptomatic. We conclude that infants with critical aortic stenosis benefit from valvotomy even with impaired left ventricular function and severely reduced left ventricular dimensions and many have nearly normal hemodynamics on late follow-up.
Aortic atresia is a form of hypoplastic left heart syndrome always complicated by associated severe hypoplasia of the ascending aorta and various degrees of mitral valve and left ventricular hypoplasia. At present it is a universally fatal lesion in early infancy. This is a report of a new palliative procedure for hypoplastic left heart syndrome that has resul;ed in early ongoing survival of two infants with aortic atresia. On the basis of experience with a third patient, an operation for future physiologic correction is proposed.
Aortic atresia is a form of congenital cardiac disease complicated by associated severe hypoplasia of the ascending aorta and various degrees of mitral valve and left ventricular hypoplasia. Occasionally, neonates with severe aortic stenosis have associated severe hypoplasia of the ascending aorta and left ventricle. These two defects constitute the most prevalent forms of so-called hypoplastic left heart syndrome. At present, this lesion is universally fatal with no established surgical management. This report describes our experience with the evolution and evaluation of staged surgical management of infants with hypoplastic left heart syndrome.
Percutaneous balloon aortic valvuloplasty (BAV) was performed in 23 consecutive patients with valvular aortic stenosis with no associated cardiac defects. The patients were 2 to 17 years old and were referred from 12 hospitals in 4 states. The balloon was positioned across the aortic valve and inflated to pressures of 80, 100, then 120 psi. Each inflation lasted 5 to 10 seconds. The arterial and venous catheters were connected together outside the groin to avoid excessive increase in left ventricular pressure during total aortic valve occlusion with the inflated balloon. Peak systolic aortic valve pressure gradient and cardiac output were measured before and 15 minutes after BAV. There was no significant change in cardiac output, but all patients had a lessened gradient. The gradient before BAV was 113 +/- 48 mm Hg, decreasing to 32 +/- 15 mm Hg after BAV (p less than 0.01). The left ventricular peak systolic pressure decreased from 221 +/- 54 to 149 +/- 21 mm Hg (p less than 0.01). No aortic regurgitation was noted in 13 patients and very mild aortic regurgitation was noted in 10 patients after BAV. The balloons were 10 to 20 mm in diameter, chosen at least 1 mm smaller than the diameter of the aortic valve anulus. Pressures of 100 to 120 psi were required to achieve full inflation of the balloons. Six patients had repeat cardiac catheterization studies 3 to 9 months after BAV. In none was there a significant change in peak systolic aortic valve pressure gradient or cardiac output compared with the study immediately after valvuloplasty.
Aortic atresia is a form of congenital cardiac disease always complicated by associated severe hypoplasia of the ascending aorta and various degrees of mitral valve and left ventricular hypoplasia. Rarely, there is an associated ventricular septal defect (VSD) and, consequently, a well-developed left ventricle. This complex malformation is universally lethal and survived after a reparative operation has not been previously reported. The following is an account of an infant with aortic atresia, VSD, and Type C aortic arch interruption who presented with a unique ductus-dependent physiology and is clinically well 11 months following a reparative operation.
The optimal treatment of critical aortic stenosis in the neonate and infant remains controversial. We compared transventricular dilation using normothermic cardiopulmonary bypass and percutaneous balloon aortic valvuloplasty with respect to early and late survival, relief of aortic stenosis, degree of aortic insufficiency, left ventricular function, and freedom from reintervention. Between July 1987 and July 1993, 30 neonates and infants underwent transventricular dilation or balloon aortic valvuloplasty for critical aortic stenosis. The patients in the transventricular dilation group (n = 21) ranged in age from 1 to 59 days (mean age 18.0 days +/- 19.1 days) and the balloon aortic valvuloplasty group (n = 9) from 1 to 31 days (mean age 10.0 days +/- 9.0 days). There were no significant differences in weight, body surface area, or aortic anulus diameter between the two groups (p = 1.0). Associated cardiovascular anomalies were more common in the transventricular dilation group (48%) than in the balloon aortic valvuloplasty group (11%). After intervention, the degree of residual aortic stenosis and insufficiency was equivalent in the two groups as assessed by postprocedural Doppler echocardiography. Ejection fraction improved within both groups (transventricular dilation 39% +/- 20.2% versus 47% +/- 22.0%; balloon aortic valvuloplasty 51% +/- 16.1% versus 62% +/- 8.4%), and there was no significant difference between groups. The left ventricular mass/volume ratio increased within both groups (p < 0.05) but with no significant difference between groups (transventricular dilation 1.4 +/- 0.5 gm/ml versus 1.8 +/- 0.6 gm/ml; balloon aortic valvuloplasty 1.1 +/- 0.6 gm/ml versus 1.7 +/- 0.4 gm/ml). Early mortality in the transventricular dilation group was 9.5% and in the balloon aortic valvuloplasty group, 11.1%. There was one late death in the transventricular dilation group. Four patients from the transventricular dilation group (19%) and two patients from the balloon aortic valvuloplasty group (22%) required reintervention for further relief of aortic stenosis. We conclude that both transventricular dilation and balloon aortic valvuloplasty provide adequate and equivalent relief of critical aortic stenosis. The treatment strategy adopted should depend on other factors, including associated cardiovascular anomalies, vascular access, preoperative condition, and the technical expertise available at each institution.
Among 183 neonates with interrupted aortic arch and ventricular septal defect entering a multiinstitutional study between 1987 and 1992, nine died before repair was accomplished. Among the remaining 174, survival at 1 month and 1, 3, and 4 years after repair was 73%, 65%, 63%, and 63%, respectively. The risk factors for death were low birth weight, younger age at repair, interrupted arch type B, outlet and trabecular ventricular septal defects, smaller size of the ventricular septal defect, and subaortic narrowing. Echocardiographically measured dimensions (expressed as Z-values) at all levels of the left heart-aorta complex were small. Two among thirty institutions were risk factors, and two others possibly were. Procedural risk factors for death after repair were (1) repair without concomitant procedures in patients with other important levels of obstruction in the left heart-aorta complex, (2) a Damus-Kaye-Stansel anastomosis, and (3) subaortic myotomy/myectomy in the face of subaortic narrowing. One-stage repair plus ascending aorta/arch augmentation had the highest predicted time-related survival in the 20% of patients with interrupted aortic arch and one or more coexisting levels of obstruction in the left heart-aorta complex, as did initial repair without or with aorta/arch augmentation in the 80% without these.
For patients with complex left ventricular outflow tract obstruction, including hypoplastic aortic anulus with or without severe diffuse subaortic stenosis, various aortoventriculoplasty procedures (e.g., Konno procedure and its modifications; extended aortic allograft root replacement) are important management options. In younger patients, however, reoperation for valve replacement is inevitably required, and anticoagulation issues pose additional problems. The pulmonary autograft provides a promising option for aortic valve replacement as part of the aortoventriculoplasty procedure in children. Long-term follow up shows that the pulmonary autograft functions well as the systemic arterial (neoaortic) valve and that valve growth occurs.
Between July 1993 and May 1995, 11 patients 4 days to 17 years old (median 12 months) underwent aortoventriculoplasty with pulmonary autograft (Ross-Konno procedure). The diagnoses were aortic stenosis with or without subaortic stenosis (n = 8), Shone complex (n = 2), and interrupted aortic arch with subaortic stenosis (n = 1). On average, 1.9 previous interventions had been performed per patient, including a previous Konno procedure in one patient. The aortic root was replaced with a pulmonary autograft valve. The left ventricular outflow tract was enlarged with a Dacron polyester fabric patch in two patients, with an allograft aortic patch in two patients and a right ventricular infundibular free wall muscular extension harvested in continuity with the autograft in seven patients.
Intraoperative transesophageal echocardiographic assessment revealed mild aortic insufficiency in one patient. One patient had a residual left ventricular outflow tract gradient of 15 mm Hg. Significant complications were cardiac tamponade from bleeding (n = 1) and complete heart block necessitating a permanent pacemaker (n = 1). Follow-up ranged from 2 weeks to 16 months. To date, there have been no late deaths or reoperations. Follow-up echocardiography revealed mild autograft insufficiency in one patient and a 16 mm Hg residual left ventricular outflow tract gradient in one patient.
Initial experience suggests that aortoventriculoplasty with the pulmonary autograft is an excellent alternative for young patients with complex left ventricular outflow tract obstruction. Because the pulmonary autograft has been shown to grow after implantation, reoperation on the left ventricular outflow tract is likely to be avoided.
Although the Ross procedure has been applied successfully to the pediatric age group, it may have its ideal application in the selected infant with critical aortic stenosis and complex left ventricular outflow tract obstruction with adequate ventricular function and size. Two neonates presented with critical aortic stenosis and complex left ventricular outflow tract obstruction, and a combined Ross/Konno procedure was performed successfully. These cases are presented along with a discussion of this topic.
Smaller patient series have not explored the independent relations between patient selection and technical factors that may impact on acute results of percutaneous balloon aortic valvotomy. To determine such relations, results from the Valvuloplasty and Angioplasty of Congenital Anomalies Registry were examined from 630 balloon aortic valvotomies in 606 patients from 23 institutions. Median age was 6.8 years (range 1 day to 18 years). The procedure could not be performed or completed in 4.1%. The left ventricular to aortic gradient was reduced by a mean +/- SD of 60 +/- 23% (p < 0.0001). Procedure-related mortality was 1.9%. A suboptimal outcome was defined arbitrarily as failure to perform the procedure, a residual gradient > or = 60 mm Hg or LV: aortic pressure ratio > or = 1.6, or major morbidity or mortality, and was noted for 17% of procedures. Independent risk factors from multiple logistic regression analyses included age < 3 months, higher preprocedure gradient, earlier procedure date, the presence of unrepaired aortic coarctation, and the use of undersized balloons. Detailed analysis revealed the optimal balloon: valve diameter ratio to be 0.9 to 1.0, with undersized balloons resulting in significant residual obstruction and oversized balloons resulting in aortic regurgitation complications. Improvements in results over time were related to increasing experience within individual institutions. Thus, percutaneous balloon aortic valvotomy provides adequate relief of obstruction, with minimal complications in most patients.
We evaluated patient and procedural characteristics that influence the midterm success of balloon dilation of congenital aortic stenosis.
Balloon dilation is a new treatment for congenital aortic stenosis. Factors that influence midterm success are unknown.
We performed a retrospective review of 148 children >1 month old who underwent balloon dilation for aortic stenosis.
Balloon dilation was successful in 87% of patients, with a procedural mortality rate of 0.7%. The average immediate peak to peak gradient reduction was 56.4 +/- 19.9% (mean +/- SD). Prior valvotomy was the only factor that significantly reduced the immediate gradient reduction after dilation (47.1 +/- 21.8% vs. 57.8 +/- 19.6%, p < 0.01). Survival after dilation was 95% at 8 years. Seventy-five percent of patients were free of repeat intervention 4 years after dilation, whereas 50% remained free of repeat intervention at 8 years. Asymmetrically thick valve leaflets (risk ratio [RR] 0.17, p < 0.01) and prior aortic valvotomy (RR 0.35, p = 0.02) decreased the risk of repeat intervention. Aortic regurgitation grade > or = 3 (RR 4.27, p = 0.04) and residual gradient after dilation (RR 1.63 for 10 mm Hg, p < 0.01) increased the risk.
The 8-year survival rate after dilation was 95% with 50% of patients free of repeat intervention. Factors that increased the risk for repeat intervention included symmetrically thin or thick aortic valve leaflets, regurgitation grade > or = 3 after dilation and a high residual gradient after dilation. The incidence of repeat intervention after dilation was high owing to its palliative nature.
To compare characteristics of aortic regurgitation (AR), the results of 213 procedures (110 balloon aortic valvotomies [BAV] and 103 surgical aortic valvotomies [SAV]) for treatment of congenital aortic valve stenosis were reviewed. These procedures were performed in 187 patients from June 1981 to September 1993. Echocardiograms recorded immediately before, within 6 months afterward, and at latest follow-up were compared. Color Doppler was used to assess the degree of AR and was quantified as the ratio of the regurgitant jet width to valve annulus, the jet width ratio. Whereas BAV patients were older (median age 5.7 years vs 3 months; p = 0.0001), there was no significant difference in median follow-up interval (3.1 years [range 0.5 to 7.2] for BAV vs 3.6 years [range 0.6 to 10.4] for SAV; p = 0.44). The mean balloon-to-annulus ratio for BAV was 0.99 +/- 0.09. An open valvotomy was performed in 83% of surgical cases. Acute systolic gradient reduction and subsequent increase at late follow-up was similar for both groups. Acutely, the mean jet width ratio increased similarly (p = 0.84) for BAV (+9 +/- 15%; p = 0.0001) and SAV (+9 +/- 12%; p = 0.0003) and was not related to age at procedure. At late follow-up, mean jet width ratio further increased significantly in both groups, although there was no difference (p = 0.17) in amount of progression (BAV +10 +/- 12%; p = 0.0001, SAV +15 +/- 13%; p = 0.0002). Thus, BAV and SAV produce AR of similar severity with similar rates of progression.
Seven patients with critical aortic stenosis underwent aortic valve replacement with a pulmonary autograft (the Ross operation) between the ages of 5 weeks and 9 months. The operation was considered mandatory for survival because of continued severe heart failure or valve avulsion. Six of the patients had undergone unsuccessful previous palliations, such as commissurotomy, balloon dilation, and transventricular valvotomy, performed singly (n = 1) or in combination (n = 5). The other patient with a severely hypoplastic aortic valve ring underwent the Ross procedure as a primary operation. Two operative deaths occurred. In both cases severe endocardial fibroelastosis was detected at autopsy. One late death 1 year after the operation resulted from progressive hypertrophic cardiomyopathy and pulmonary hypertension. The rest of the patients are doing well, without medications. Apart from trivial regurgitation in two patients, the pulmonary autograft is performing well.
The purpose of this study was to report on 3- to 9-year follow-up data after balloon aortic valvuloplasty in children and to investigate causes of aortic insufficiency at late follow-up. Although the immediate and short-term results of balloon aortic valvuloplasty have been well documented, little information is available on long-term follow-up results. During a 7.3-year period ending December 1992, 26 young patients, aged 6 weeks to 20 years, underwent balloon aortic valvuloplasty with resultant reduction of peak-to-peak aortic valvar gradient from 71 +/- 20 (mean +/- SD) to 25 +/- 12 mm Hg (p < 0.001). None required immediate surgical intervention. At intermediate-term follow-up, 6 (23%) of 26 had restenosis develop and underwent surgical (4 patients) or repeat balloon valvuloplasty (2 patients). Clinical and echo-Doppler data 3 to 9 years (median 6 years) after balloon valvuloplasty revealed residual peak instantaneous Doppler gradients of 26 +/- 13 mm Hg (p < 0.001), without restenosis beyond what was observed at intermediate-term follow-up. Aortic insufficiency progressed in seven patients. However, none required intervention. Actuarial intervention-free rates at 1, 2, 5, and 9 years were 80%, 76%, 76%, and 76%, respectively. Logistic regression analysis suggested that the degree of Doppler-quantitated aortic insufficiency 1 day after valvuloplasty predicts persistent aortic insufficiency at late follow-up. These data indicate that immediately successful balloon aortic valvuloplasty in children yields a residual gradient of < or = 36 mm Hg at a median of 6 years of follow-up in most patients and an intervention-free rate at 9 years of 76%. Restenosis occurs but can be treated with a repeat intervention with good results. Aortic insufficiency remains stable and does not appear to require intervention, at least during the first decade after balloon dilatation.
We compare the clinical efficacy of two approaches for balloon aortic valvuloplasty (BAV) in infants with critical aortic valve stenosis. The approaches were through the carotid artery and the femoral artery. Eight catheterizations for BAV were performed in seven consecutive patients with critical aortic stenosis: four BAVs were approached through the femoral artery and four through the right common carotid artery. We inserted a 5F sheath into the right common carotid artery by a cutdown procedure; after BAV the sheath was removed and the carotid arteriotomy sutured with 7-0 monofilament. Two cases in which the femoral artery approach was used resulted in failure to perform BAV; two cases had complications. All four cases with the carotid artery approach were successful, with no complications; aortography performed 3 months after one balloon valvuloplasty revealed a smooth, unobstructed right carotid artery. Use of the carotid artery approach may reduce serious complications with BAV and offers quicker, easier maneuvering in infants and neonates with critical aortic valve stenosis.
Controversy persists with regard to the treatment of patients with aortic atresia. Staged reconstructive operations and primary transplantation have been advocated as treatment strategies, but in many instances no treatment is undertaken. A multi-institutional study was undertaken for the purpose of characterizing this challenging patient group, comparing the prevalence and outcomes of the various treatment strategies, and identifying potential predictors of success or failure with each.
A total of 323 neonates with aortic atresia were entered into a 21-institution prospective, nonrandomized study between January 1, 1994, and January 1, 1997. Three protocols were used, nonexclusively in many institutions: (1) staged reconstructive surgery with initial palliation by a Norwood procedure and eventual Fontan operation, (2) heart transplantation as initial definitive therapy, and (3) nonsurgical management. Analysis was based on initial protocol assignment: staged reconstructive surgery in 253 patients, heart transplantation in 49 patients, and nonsurgical management in 21 patients. For all patients initially entered into the 2 surgical treatment protocols, survival at 1, 3, 12, 24, and 36 months after entry was 67%, 59%, 52%, 51%, and 50%, respectively. A multivariable analysis found incremental risk factors for death at any time after entry to be lower birth weight (P=.04), associated noncardiac anomaly (P=.007), and entry into the nonsurgical protocol (P < .0001) or the staged reconstructive surgery protocol (P=.03). Four institutions had higher survival statistics; 2 used a heart transplantation protocol and 2 used a staged reconstructive surgery protocol. For the 113 patients treated at these 4 institutions, survival at 1, 3, 12, 24, and 36 months after entry was 77%, 70%, 64%, 62%, and 61%, respectively. Survival among the 4 institutions was similar (P=0.1).
Among patients with aortic atresia, other features of cardiac structure including aortic size, degree of left ventricular hypoplasia, and degree of mitral hypoplasia or atresia are not predictive of survival from 2 surgical protocols. The highest survival was achieved with either treatment strategy at institutions strongly committed to the use of one or the other surgical management protocol.
Multiple obstructions in the left heart-aorta complex have been associated with poor survival. No consensus exists as to whether these patients will have a favorable outcome with biventricular repair where most advocate a univentricular approach.
Since late 1988, all 11 neonates seen with hypoplastic left heart complex, which includes aortic arch obstruction, underwent biventricular repair. All patients had antegrade aortic flow and no intrinsic aortic or mitral stenosis. Elimination of the extracardiac afterload was achieved by extensive ascending aorta and aortic arch reconstruction with a pulmonary homograft patch. All intracardiac shunts were eliminated to fully preload the left heart. The median age at first operation was 7 days and the mean weight, 3.59+/-0.49 kg. The echocardiographic variables used to evaluate the left heart-aorta complex were reviewed, and the preoperative and postoperative measurements were compared.
There were two early deaths. Four patients had six reoperations for left ventricular outflow tract obstruction, 2 of whom have required prosthetic valve replacement (1, aortic and mitral; 1, aortic), and 2 patients had three reoperations for recurrent coarctation. There was one late death at 3 years from pulmonary hypertension. Mean follow-up was 44+/-35 months. The 8 current survivors are all in New York Heart Association class I or II. The actuarial survival rate at 8 years is 63%, and the freedom from reoperation at 3 years is 25%.
We have successfully achieved biventricular repair in most of the patients with hypoplastic left heart complex, a subset of patients with hypoplastic left heart syndrome. Some growth of the left ventricular structures was already observed at the time of hospital discharge. However, reoperation, particularly for left ventricular outflow tract obstruction, appears likely. Increasing experience will more accurately define predictive criteria for the feasibility of biventricular repair.
Although the influence of small left heart structures on outcome of a biventricular repair in neonatal critical aortic stenosis is well documented, little is known about its effect in neonates with aortic arch obstruction and coarctation. The purpose of this study was to evaluate the influence of small left heart structures on early and late results of repair and the ability to achieve a biventricular repair in neonates with coarctation and aortic arch obstruction.
Neonates included in this study had a left ventricular adequacy score (as proposed by Rhodes and associates for critical aortic stenosis) that would have predicted a need for a univentricular (Norwood) repair. All were ductus dependent but had antegrade ascending aortic flow and a small but nonstenotic aortic valve (<30 mm Hg gradient). Twenty neonates aged 10 +/- 9 days were identified for the study with weights averaging 3. 1 +/- 0.6 kg. Selected left heart measurements obtained by preoperative echocardiography included the following: aortic anulus 5.3 +/- 0.3 mm, mitral anulus 8.4 +/- 1.0 mm, transverse aortic arch 3.4 +/- 0.6 mm, and left ventricular volume 25 +/- 4 mL/m2. All patients underwent coarctation repair by resection and extended end-to-end anastomosis to enlarge the transverse arch as needed. Three patients underwent simultaneous pulmonary artery banding because of a hemodynamically significant ventricular septal defect. These 3 patients have subsequently had their defects successfully closed without mortality.
There were no early or late deaths at a follow-up of 38 +/- 16 months after the operation. Three patients (3/20, 15%) have had to undergo reintervention with balloon aortoplasty because of recurrent coarctation (gradient > 20 mm Hg) in 2 and resection of subaortic stenosis in 1. Late follow-up in the remaining patients reveals 1 with moderate subaortic stenosis (gradient = 43 mm Hg), 2 with mild aortic stenosis (gradient < 30 mm Hg), and 2 with mild to moderate mitral stenosis. At late follow-up, 16 patients (16/20, 80%) are completely free of symptoms and 4 (4/20, 20%) have mild residual symptoms.
Biventricular physiology can be successfully achieved in neonates with small left heart structures and aortic arch obstruction with minimal mortality and excellent late functional results. Standard echocardiographic measurements used to predict the need for a univentricular repair in critical aortic stenosis are not valid for the neonate with aortic arch obstruction.
Normalization of the dimensions of cardiac structures to the size of the body, using so-called Z scores, is becoming increasingly common in the management of infants and children with congenital heart disease. Current published nomograms for the ascertainment of Z scores for cardiac structures in childhood are based largely on normal data obtained in formalin-fixed hearts. Since decisions concerning management are frequently based on the findings of cross-sectional echocardiograms, the dimensions of 15 cardiac structures were measured using cross-sectional echocardiography in 125 normal infants and children. Regression equations were derived relating cardiac dimensions to the size of the body. The expression of size with the highest correlation to cardiac dimensions was body surface area. Nomograms were then developed from which the Z score of a cardiac structure could be estimated from a knowledge of the body surface area and the echocardiographically derived measurement.
Thirty-seven patients with congenital aortic stenosis have been studied. Only a few were without symptoms referable to the defect. Characteristically, the patients had a loud, harsh systolic murmur, accompanied by a thrill, to the right of the sternum. On x-ray examination the most frequent manifestation was dilatation of the ascending aorta. Electrocardiographic evidence of left ventricular hypertrophy was found in 60 per cent. Nineteen patients have been operated upon with 2 deaths. The procedure has been tolerated amazingly well. Although it is too early to judge the results, all but 2 of the surviving patients appear to have been helped.