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Predictors of survival in neonates with critical aortic stenosis
Abstract
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.
... Criteria for selecting biventricular repair versus univentricular palliation are evolving. [1][2][3][4] In neonates with critical aortic stenosis selected for biventricular repair, valvotomy has been the mainstay of therapy. Both surgical and transcatheter balloon valvotomy, however, are associated with important mortality, residual or recurrent valve dysfunction, and need for reintervention. ...
... 17 Numerous studies have assessed risk factors for death after valvotomy in neonates and small infants with critical aortic stenosis. Morphological features reported to increase mortality include lower-indexed aortic root/annular dimension 2,3 ; lower ascending aortic or arch dimensions 3 ; lower-indexed mitral valve area or mitral valve dimension 2,19 ; lower left ventricular end-diastolic dimensions, cross-sectional area, length, or volume [2][3][4]10,20 ; increased presence of endocardial fibroelastosis 12,20 ; and lower body surface area. 2 Physiological variables have also been reported to increase mortality, including elevated pulmonary artery pressures or left ventricular end-diastolic pressures, 10,12 retrograde direction of ascending aortic blood flow, 3,21 and lower ejection fraction. 12,20 The cause of death in neonates with critical aortic stenosis after balloon or surgical valvotomy is often low output syndrome due to an inadequate left heart. ...
... 17 Numerous studies have assessed risk factors for death after valvotomy in neonates and small infants with critical aortic stenosis. Morphological features reported to increase mortality include lower-indexed aortic root/annular dimension 2,3 ; lower ascending aortic or arch dimensions 3 ; lower-indexed mitral valve area or mitral valve dimension 2,19 ; lower left ventricular end-diastolic dimensions, cross-sectional area, length, or volume [2][3][4]10,20 ; increased presence of endocardial fibroelastosis 12,20 ; and lower body surface area. 2 Physiological variables have also been reported to increase mortality, including elevated pulmonary artery pressures or left ventricular end-diastolic pressures, 10,12 retrograde direction of ascending aortic blood flow, 3,21 and lower ejection fraction. 12,20 The cause of death in neonates with critical aortic stenosis after balloon or surgical valvotomy is often low output syndrome due to an inadequate left heart. ...
Background For neonates with critical aortic valve stenosis who are selected for biventricular repair, valvotomy can be achieved surgically (SAV) or by transcatheter balloon dilation (BAV).
Methods and Results Data regarding 110 neonates with critical aortic valve stenosis were evaluated in a study by the Congenital Heart Surgeons Society from 1994 to 1999. Reduced left ventricular function was present in 46% of neonates. The initial procedure was SAV in 28 patients and BAV in 82 patients. Mean percent reduction in systolic gradient was significantly greater with BAV (65±17%) than SAV (41±32%; P <0.001). Higher residual median gradients were present in the SAV versus BAV group (36 mm Hg [range, 10 to 85 mm Hg] versus 20 mm Hg [0 to 85 mm Hg], P <0.001). Important aortic regurgitation was more often present after BAV (18%) than SAV (3%; P =0.07). Time-related survival after valvotomy was 82% at 1 month and 72% at 5 years, with no significant difference for SAV versus BAV, even after adjustment for differences in patient and disease characteristics. Independent risk factors for mortality were mechanical ventilation before valvotomy, smaller aortic valve annulus ( z score), smaller aortic diameter at the sinotubular junction ( z score), and a smaller subaortic region. A second procedure was performed in 46 survivors. Estimates for freedom from reintervention were 91% at 1 month and 48% at 5 years after the initial valvotomy and did not differ significantly between groups.
Conclusions SAV and BAV for neonatal critical aortic stenosis have similar outcomes. There is a greater likelihood of important aortic regurgitation with BAV and of residual stenosis with SAV.
... Hypoplastic left ventricle (LV) with outflow obstruction and duct-dependent systemic circulation creates a dilemma in decision-making regarding univentricular versus biventricular repair. [1,2] Prediction of postnatal ability of LV to sustain a systemic cardiac output without a ductus is based on the severity of left ventricular hypoplasia and multiplicity of levels of obstruction. [2] The echocardiographic indices proposed to predict the outcome after surgical repair have been observed to improve progressively on follow-up when the neonate is medically managed for weeks, thereby allowing biventricular repair in some infants who were initially deemed unsuitable for sustaining a two-ventricle circulation. ...
... [1,2] Prediction of postnatal ability of LV to sustain a systemic cardiac output without a ductus is based on the severity of left ventricular hypoplasia and multiplicity of levels of obstruction. [2] The echocardiographic indices proposed to predict the outcome after surgical repair have been observed to improve progressively on follow-up when the neonate is medically managed for weeks, thereby allowing biventricular repair in some infants who were initially deemed unsuitable for sustaining a two-ventricle circulation. [3] This questions the validity of these echocardiographic predictors. ...
... [1] Decision-making is simpler when the hypoplastic LV is at either extremes. [2] Severe forms require Norwood palliation or cardiac transplantation; mildest forms get complete biventricular repair. Echocardiographic scores based on left heart dimensions and body surface area, proposed to define mortality risk after biventricular repair are questioned as they progressively improve with growth of the neonate. ...
Duct dependent aortic arch obstruction with borderline left ventricular hypoplasia presents a diagnostic dilemma. If the left ventricle (LV) is adequate to sustain systemic cardiac output without a patent duct, arch obstruction is relieved surgically and duct is divided. Inadequate LVs do not tolerate duct division, and these patients need more complex Norwood type surgeries. However, catheter-based interventions for arch obstruction can retain ductal patency. The progressive changes in anatomy and physiology of a neonate who presented with hypoplastic left heart structures and patent duct with advancing age and modified by serial catheter-based interventions are presented to highlight the use of interventions in this therapeutic dilemma.
... 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. ...
... 6 The patient was presented at a multi-disciplinary team meeting. 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. ...
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.
... Adequacy of LV in severe AS in infants determines the success of PBAV. Four anatomical variables that were found to have the most significant independent relation to survival with AS as described by Rhodes et al. in 1991 are: (1) a left ventricular long-axis to heart long-axis ratio of 0.8 or less; (2) an indexed aortic root diameter of 3.5 cm/m 2 or less; (3) an indexed mitral valve area of 4.75 cm 2 /m 2 or less and 4) an LV mass index of 35 g/m 2 or less. [13] ...
... Four anatomical variables that were found to have the most significant independent relation to survival with AS as described by Rhodes et al. in 1991 are: (1) a left ventricular long-axis to heart long-axis ratio of 0.8 or less; (2) an indexed aortic root diameter of 3.5 cm/m 2 or less; (3) an indexed mitral valve area of 4.75 cm 2 /m 2 or less and 4) an LV mass index of 35 g/m 2 or less. [13] ...
Aortic valve obstruction accounts for majority of left ventricular outflow tract obstruction. When present in neonatal age group, presentation is more severe and is mostly due to unicomissural aortic valve while at later age, bicuspid aortic valve is the most common cause for aortic stenosis. Echocardiography plays an importance role in the diagnosis and to assess the anatomy for suitability for balloon valvuloplasty. Its role also extends to assess the left ventricle size and function, to diagnose associated lesions and to suggest the suitable plan of management. Echocardiography also plays an important role immediately after balloon dilatation and during follow up to assess adequacy of dilatation and resultant complications. Balloon dilation of bicuspid aortic valves is associated with better outcome and lesser need for reinvention as compared to unicuspid valves.
... Therefore, predictive scores have been developed to ease decision-making in this subset in attempt to find the most appropriate type of circulation. These scores, however, bear limitations and are less reliable particularly for patients close to discriminant cut-off values, thus rendering parental counselling difficult [4,5]. Furthermore, published series frequently included a broad variety of bLV associated with further intracardiac anomalies leading to substantial bias in the predictive value of examined parameters [6][7][8]. ...
... However, Weber included cases with AVSD and DORV, which might lead to selection bias [7]. Rhodes et al. developed a scoring system based on retrospective postnatal echocardiographic factors predicting in-hospital mortality in patients with critical AS [4]. However, natural history differs from bLV without AS, and the outcome of bLV with AS is usually worse due to myocardial and endocardial abnormalities leading to EFE, impaired diastolic properties, pulmonary hypertension, and elevated left atrial pressure [15,16]. ...
Objectives Prenatal prediction of postnatal univentricular versus biventricular circulation in patients with borderline left ventricle (bLV) remains challenging. This study investigated prenatal fetal echocardiographic parameters and postnatal outcome of patients with a prenatally diagnosed bLV.
Methods We report a retrospective study of bLV patients at four prenatal centers with a follow-up of one year. BLV was defined as z-scores of the left ventricle (LV) between –2 and –4. Single-ventricle palliation (SVP), biventricular repair (BVR), and no surgical or catheter-based intervention served as the dependent outcome. Prenatal ultrasound parameters were used as independent variables. Cut-off values from receiver operating characteristic curves (ROC) were determined for significant discrimination between outcomes.
Results A total of 54 patients were diagnosed with bLV from 2010 to 2018. All were live births. Out of the entire cohort, 8 (15 %) received SVP, 34 (63 %) BVR, and 12 (22 %) no intervention. There was no significant difference with regard to genetic or extracardiac anomalies. There were significantly more patients with endocardial fibroelastosis (EFE) in the SVP group compared to the BVR group (80 % vs. 10 %), (p < 0.001). Apex-forming LV (100 % vs. 70 %) and lack of retrograde arch flow (20 % vs. 80 %) were associated with no intervention (p < 0.001). With respect to BVR vs. SVP, the LV sphericity index provided the highest specificity (91.7 %) using a cutoff value of ≤ 0.5.
Conclusion The majority of bLV patients maintained biventricular circulation. EFE, retrograde arch flow, and LV sphericity can be helpful parameters for counseling parents and further prospective studies can be developed.
... Borderline left side mandates are more detailed analysis of z-scores and cavity volumes to guide further management. Of particular value are the mitral and aortic valve z-scores, LV volumes, and ratio of LV length and total cardiac length [13,14]. Cardiac catheterization is reserved for cases with a potential for therapeutic intervention(s) in the form of balloon aortic valvotomy for cases suitable for biventricular physiology or septostomy for single ventricle pathway. ...
... Using discriminant analysis, a best predictive equation was arrived at using the same variables. A score of < − 0.35 was predictive of death after BVR with an accuracy of 88% [14]. Fifteen years later, the score was revisited, validated, and redesigned because the predictability was found to be only 77%. ...
The left ventricular outflow tract (LVOT) comprises of the subvalvular area, the aortic valve, and the supravalvular region. Obstructive lesion of LVOT is a spectrum with varying levels and degree of obstruction with or without associated hypoplasia of the left ventricle. Decision-making in small and borderline LVOT can be challenging. Imaging modalities such as echocardiography and magnetic resonance imaging and scores based on imaging aid in the decision making in truly borderline cases. Newer treatment strategies like staged left ventricular rehabilitation and hybrid procedure have come to the fore in the past decade or so. Although these do not address small LVOT per se, they delay the decision-making to a more appropriate age. The goal of management in these cases is to achieve a biventricular repair whenever feasible. Several surgical techniques could be employed to achieve this goal. However, it is important to be cognizant of the fact that an overzealous approach to achieve a biventricular repair might be counterproductive. A univentricular palliation could be a safer alternative; especially considering the possibility of a future transplant candidacy.
... 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. ...
... In literature, different risk scores were published to predict anatomic suitability for biventricular repair in neonates with aortic stenosis evaluating left ventricular dimensions, diameter of aortic valve and left ventricular outflow tract as well as aortic arch dimensions. We used Rhodes score [6], CHSS-1 [7], CHSS-2 [8] and Discriminant score [9] to compare anatomic complexity of left heart structures in our patient population as described in detail in literature. ...
Background
Hybrid procedure with ductal stenting and bilateral pulmonary banding offers a temporary approach in high-risk neonates with complex congenital heart defects aiming biventricular repair. This procedure may also have negative impact concerning post-banding pulmonary stenosis resulting in right ventricular pressure load.
Methods
Between 2010 and 2021 we identified 5 patients with interrupted aortic arch and complex congenital heart defect who underwent hybrid procedure and staged biventricular repair (“hybrid-group”). Other 7 cases with interrupted aortic arch were corrected in the neonatal phase without hybrid procedure (“nonhybrid-group”). Detailed intra- and extracardiac features and surgical procedures were documented as well as pulmonary interventions during follow up. Pulmonary vessel size was assessed by diameter of left and right pulmonary artery in absolute and indexed values. RV pressure was evaluated invasively via catheterization.
Results
Survival in cases with hybrid procedure and staged biventricular repair was 91% for a follow-up time of 40.7 months (95% CI 26–55 months) and 100% in the non-hybrid-group. Postoperative results concerning left ventricular function showed normal LV dimensions and systolic function without relevant stenosis on distal aortic arch. Hybrid procedure was associated with impaired local pulmonary arterial diameter after debanding resulting in increased right ventricular pressure and need for interventions (number intervention per patient: hybrid group 1.7 ± 0.95, non-hybrid group 0.17 ± 0.41; P 0.003).
Conclusions
Hybrid procedure in high-risk cases with interrupted aortic arch and staged biventricular repair shows good postoperative results with low perioperative mortality and normal left ventricular function. Due to potential risk of relevant pulmonary stenosis and right ventricular pressure load, follow up examinations must not only focus on left but also on the right heart.
Graphical Abstract
... There are scoring systems available such as Rhodes score, CHSS, and discriminant score, which allow the clinician to decide the adequacy of left heart size and plan management (univentricular vs. biventricular). As early as 1991, Rhodes et al. [9] put forward a biventricular repair predictive equation in critical aortic stenosis: score = 14.0 (BSA) + 0.943 (ROOTi) + 4.78 (LAR) + 0.157 (MVAi) -12.03. Among which, BSA = body surface area, ROOTi = aortic root dimension indexed to BSA, LAR = ratio of the long-axis dimension of LV to long-axis dimension of heart, and MVAi = MV area indexed to BSA, with a discriminating score of <−0.35 predictive of death after a biventricular repair. ...
The left ventricular outflow is divided into subvalvar area, the aortic valve and supra valvar region. There can occur a number of anomalies in the outflow tract, most commonly of which are obstructive lesions, which can exist in isolation or as a part of association with other defects such as ventricular septal defects or interrupted arch. The following chapter highlights on echocardiography imaging of the left ventricular outflow tract and its anomalies.
... 1,2 In critical aortic stenosis, especially in the setting of initial LV dysfunction, the decision-making can be challenging despite established scoring systems. [3][4][5] We present a case that illustrates how long-term development of haemodynamic parameters can be difficult to estimate. ...
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.
... 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. ...
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.
... В няколко проучвания е демонстрирана важността на адекватния входящ кръвоток в ЛК при случаите с хипоплазия на ЛК [25,26,27,28,29]. Често в тях входящият кръвоток в ЛК е измерен като z-скор, като стойност < -2 е приета като дефиниция за хипопластична клапа. ...
Unbalanced atrioventricular septal defect is seen by 10-15% of the patients with this congenital heart disease and poses serious challenges for the treating physicians regarding the choice of the most suitable operative intervention. The forms with moderate degree of unbalance between the two ventricles, especially when the size of the left ventricle is considered borderline, make the choice between two-ventricle correction and single-ventricle palliation quite difficult. The most important factor when judging the degree of unbalance is not the size of the ventricles but rather the redistribution of blood flow. The use of different echocardiographic measurements could contribute to the more precise determination of the indications for one of the two surgical strategies with resulting improvement of patient outcome.
... [2][3][4] There is a considerable risk of death in patients who fail 2V repair and require conversion to a 1V. 5,6 Predictors of successful 2V repair in this patient population are not well defined. It has been previously determined that factors described in the evaluation of critical aortic stenosis are not applicable to this patient population. ...
Objective:
Deciding on a surgical pathway for neonates with ≥2 left heart obstructive lesions is complex. Predictors of the successful biventricular (2V) repair in these patients are poorly defined. The goal of our study was to identify patients who underwent the 2V repair and assess anatomic and echocardiographic predictors of success.
Design:
Infants born between July 2015 and August 2017 with ≥2 left heart obstructive lesions with no prior interventions were identified (n = 19). Patients with aortic or mitral valve (MV) atresia and critical aortic stenosis were excluded. Initial echocardiograms were reviewed for aortic, MV, tricuspid valve annulus size, and left (LV) and right (RV) ventricle diastolic longitudinal dimensions. The valve morphology and presence of a ventricular septal defect (VSD) and coarctation were assessed. Clinical outcomes included successful 2V repair, complications, and repeat interventions or surgeries. Failed 2V repair was defined as a takedown to single ventricle (1V) physiology, cardiac transplantation, or death.
Results:
For 2V repair, 14/19 patients were selected and for 1V, 5/19 patients were selected. Initial surgical procedures of the 2V group were simple coarctation repair (5), complex coarctation/arch reconstruction +/- septal defect closure (6), hybrid stage 1 (2), and none (1). Three of the 2V patients required reintervention in the first 90 days. The LV to RV diastolic longitudinal ratio >0.75 and mitral/tricuspid ratio of <0.8 were observed in 13/14 of the 2V patients. The LV:RV ratio and the aortic valve z score were significantly larger in the 2V group compared to the 1V group. All patients in the 1V group had a nonapex forming LV. There was no mortality with follow-up to three years of age.
Conclusions:
This study showed excellent short-term and midterm surgical results in the 2V population. The LV:RV diastolic longitudinal ratio may be a useful tool in the risk stratification of a successful 2V repair even in cases with a small MV.
... [4,12] To predict the feasibility of biventricular repair in critical AS, several prediction models have been proposed. As early as 1991, Rhodes et al [13] put forward a biventricular repair predictive equation in critical AS: Score = 14.0 (BSA) + 0.943 (ROOTi) + 4.78 (LAR) + 0.157 (MVAi) -12.03. Among which, BSA = body surface area, ROOTi = aortic root dimension indexed to BSA, LAR = ratio of the longaxis dimension of LV to long-axis dimension of heart, and MVAi = MV area indexed to BSA, with a discriminating score of less than -0.35 predictive of death after a biventricular repair. ...
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.
... It should also be noted that the V E/V CO2 slope of patients with Fontan palliation was significantly higher than that of patients with biventricular palliation,[62] once again highlighting the importance of the subpulmonary ventricle in maintaining pulmonary artery pulsatility, normal pulmonary blood flow distribution, normal V/Q matching, and efficient gas exchange during exercise. The importance of these physiologic functions should also be taken into consideration It has been found that the exercise function of children and adolescents with biventricular circulations following interventions for severe aortic stenosis within the first 6 months of life (including some who had Congenital Heart Surgeon Society scores and modified Rhodes stratification scores [64] that predicted them to have a survival advantage with single-ventricle palliation) tends to be better than that of Fontan patients. [65] If this were not the case, the rationale for pursuing an aggressive approach toward fetal aortic stenosis and/or the recruitment of a congenitally hypoplastic left ventricle would be weakened. ...
The Fontan procedure was initially suggested as a treatment for patients with tricuspid atresia. Since its introduction the application of this innovative surgical approach has expanded to include patients with single ventricle physiology secondary to a variety of anatomic disorders including hypoplastic left heart syndrome, double inlet or other single left ventricle, pulmonary atresia with intact ventricular septum, unbalanced atrioventricular septal defects, complex heterotaxy syndromes, and others. There has also been extensive evolution of the surgical approach. Currently two types of total cavopulmonary anastomoses, the lateral tunnel and extracardiac conduit modifications, are the most commonly employed modifications, as they appear to reduce the energy loss within the Fontan circuit and reduce the long-term risk of arrhythmias. Older patients with historic anatomic Fontan modifications including right atrial to pulmonary artery or right atrial to right ventricular anastomoses, with or without interposed valves within the Fontan circuit, are still encountered. Fontan physiology can have a dramatic upon the cardiopulmonary response to exercise and exercise testing can provide important information regarding a patient’s clinical status.
... Because adequacy of left heart structures and function influences the outcome of intervention in infants with VAS, many echocardiographic morphometric parameters and hemodynamic variables of the left heart have been used to determine whether relieving the LVOT obstruction will achieve a two-ventricle repair or whether a staged single-ventricle repair (the Norwood procedure) should be considered [30,31]. ...
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.
... On the other hand, the choice of appropriate surgical approach in subjects with borderline LV can be challenging, in particular when it comes to identifying the patients suitable for BVR. A few predictive scores based on echocardiographic measurements have been suggested to help with decision-making [5][6][7][8]. Nevertheless, there is often no uniformity among them to recommend either a univentricular or a biventricular strategy. ...
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OBJECTIVES
We analysed the outcomes of patients undergoing biventricular repair (BVR) after an initial hybrid Norwood approach as a salvage procedure in extremely sick infants; or as the initial palliation in patients with uncertain feasibility of single-stage BVR due to severe left ventricular outflow tract obstruction; or as part of a left ventricle (LV) recruitment strategy in patients with borderline LVs.
METHODS
Between September 2010 and July 2018, 26 patients underwent BVR after initial hybrid palliation at a median age of 13 days. The rationale for the hybrid approach was to promote the growth of the LV in 10 patients and that of the left ventricular outflow tract and/or aortic valve in 12 patients and to be a salvage procedure in 4 patients. Significant growth of the LV was noted during the interstage period, which had a median length of 65 days (P = 0.008). Fourteen patients underwent aortic arch repair, ventricular septal defect closure and relief of subaortic stenosis; 5 patients underwent the Yasui procedure; 4 patients had the Ross–Konno procedure; 2 patients had an arterial switch operation; and 1 patient had truncus arteriosus repair.
RESULTS
Twenty-two patients (84.6%) are alive at a median follow-up period of 1.8 (range 0.04–6.2) years. There were 2 early and 2 late deaths. Nineteen catheter-based reinterventions and 15 reoperations were performed after BVR.
CONCLUSIONS
The hybrid Norwood procedure permits stabilization of critical infants. It allows for growth of left ventricular structures in some patients with borderline left hearts and in those with severe left ventricular outflow tract obstruction. More patients may eventually have BVR than was thought during the newborn period.
... Although many scoring systems have been developed to assist with decision-making regarding biventricular conversion, the sensitivity and specificity of these tools are too low for prospective application. 6 It is clear that ventricular size matters, and left ventricular end-diastolic volume is frequently used to determine size adequacy. In patients with unbalanced atrioventricular (AV) canal defect or double outlet right ventricle, left ventricular end-diastolic volume of greater than 30 mL/m 2 may be adequate, whereas in patients with hypoplastic left heart syndrome variants, 45 mL/m 2 is a safer cutoff. ...
... 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.
... The decision-making process for this defect is complex, and the surgical decision must be made within the context of each patient's unique presentation. [16][17][18] Interrupted Aortic Arch With VSD. Interrupted aortic arch with VSD is usually associated with a type B interruption of the aorta (interruption between the left subclavian and left carotid arteries) with an associated conotruncal abnormality. ...
This article discusses congenital heart defects that are marginal candidates for biventricular repair and highlights the anatomic considerations upon which the surgical decision is based. Specifically, the article reviews the importance of capacitance and compliance of the ventricles and their associated atrioventricular valves. For each of the defects discussed, the imaging modalities used to diagnose a marginal ventricle are reviewed and the surgical decision-making process is outlined. The article also reviews immediate postoperative treatment of these patients and when to consider biventricular repair of a marginal lesion to be a failure.
... However, some patients, particularly those with risk factors for SVP, might be candidates for staged ventricular recruitment strategy in which procedures are performed to promote ventricular growth with the ultimate goal of biventricular (BIV) conversion. [1][2][3][4] In previous experience with staged left ventricle (LV) recruitment strategy in patients with borderline hypoplastic left heart and intact ventricular septum, fenestrated atrial septal defect (ASD) closure was the technique most significantly associated with LV growth. 1 However, because diastolic flow across a ventricular septal defect (VSD) limits the potential to volume load a hypoplastic ventricle, it was unclear whether a strategy of ASD restriction without VSD closure would promote ventricular growth in patients with an unrestrictive VSD. Although closure of the ASD as well as VSD ensures that all diastolic flow into the hypoplastic ventricle remains in that ventricle, such a strategy loads the unprepared ventricle with a full cardiac output and might lead to prohibitively elevated diastolic filling pressures and depressed systolic function. ...
Objectives:
Patients with borderline ventricles and ventricular septal defects (VSDs) who have previously undergone single ventricle palliation might be candidates for staged ventricular recruitment with the ultimate goal of biventricular conversion. This study aimed to determine the effect of atrial septal defect (ASD) restriction without VSD closure on ventricular growth in patients with borderline right or left ventricles and VSDs.
Methods:
Patients with borderline ventricles and VSD who underwent a staged ventricular recruitment procedure with strategies to increase blood flow through hypoplastic ventricle via ASD restriction without VSD closure after single ventricle palliation were retrospectively reviewed. Pre- and postrecruitment ventricular volumes were compared using Wilcoxon signed rank test.
Results:
A total of 21 patients underwent staged ventricular recruitment via ASD restriction without VSD closure at median age of 20.0 months (interquartile range [IQR], 8.0-52.5 months). At a median of 9.0 months (IQR, 8.0-11.8 months) after the recruitment procedure, there were increases in the median indexed ventricular diastolic volume (31.7 mL/m2 [IQR, 24.5-37.1] to 48.5 mL/m2 [IQR, 38.4-58.0; P < .01]), median indexed systolic volume (13.3 mL/m2 [IQR, 9.7-18.7] to 19.5 mL/m2 [IQR, 16.8-29.7]; P < .01), and the median indexed stroke volume (18.4 mL/m2 [IQR, 14.8-21.1] to 28.1 mL/m2 [IQR, 21.3-31.8]; P < .01). Biventricular conversion was ultimately performed in 14 (67%). Two patients died after biventricular conversion.
Conclusions:
Staged ventricular recruitment via ASD restriction without VSD closure is associated with growth of the hypoplastic ventricle. In patients who are deemed high-risk for single ventricle, this approach might facilitate eventual biventricular conversion. Further studies are needed to identify optimal candidates for this approach.
A heart with a borderline ventricle refers to a situation where there is uncertainty about whether the left or right underdeveloped ventricle can effectively support the systemic or pulmonary circulation with appropriate filling pressures and sufficient physiological reserve. Pediatric cardiologists often deal with congenital heart diseases (CHDs) associated with various degrees of hypoplasia of the left or right ventricles. To date, no specific guidelines exist, and surgical management may be extremely variable in different centers and sometimes even in the same center at different times. Thus, the choice between the single-ventricle or biventricular approach is always controversial. The aim of this review is to better define when “small is too small and large is large enough” in order to help clinicians make the decision that could potentially affect the patient’s entire life.
Background
Identification of risk factors for biventricular (BiV) repair in children with hypoplastic left ventricles (HLV) has been challenging. We sought to identify preoperative cardiovascular magnetic resonance (CMR) predictors of outcome in patients with HLVs who underwent BiV repair, with a focus on the mitral valve (MV).
Methods
Single-center retrospective analysis of preoperative CMRs on patients with HLV (≤50 mL/m²) and no endocardial fibroelastosis who underwent BiV repair from 2005–2022. CMR measurements included MV orifice area in diastole. The primary composite outcome included time to death, transplant, BiV takedown, heart failure admission, left atrial decompression, or unexpected reoperation; and the secondary outcome included more than or equal to moderate mitral stenosis and/or regurgitation.
Results
Median follow-up was 0.7 (interquartile range 0.1, 2.2) years. Of 122 patients [59 atrioventricular canal (AVC) and 63 non-AVC] age 3 ± 2.8 years at the time of BiV repair, freedom from the primary outcome at 2 years was 53% for AVC and 69% for non-AVC (log rank p = 0.12), and freedom from the secondary outcome at 2 years was 49% for AVC and 79% for non-AVC (log rank p < 0.01). Independent predictors of primary outcome for AVC patients included MV orifice area z-score <−2 and transitional AVC; for non-AVC patients, predictors included MV orifice area z-score <−2, abnormal MV anatomy, and conal-septal ventricular septal defect. Independent predictors of secondary outcome for AVC patients included older age at surgery, transitional AVC, and transposition of the great arteries.
Conclusion
In children with HLV, low MV orifice area and pre-existing MV pathology are risk factors for adverse outcome after BiV repair.
This study aims to describe the creativity process of Imron Sadewo in song composing based on Kuntowijoyo’s biography theory, Wallas’s creativity theory and Rhodes’s creativity theory. This study uses a qualitative descriptive research method that is analyzed through three stages, namely data reduction, data display, and data conclusion. The validity test of this study is using triangulation technique and triangulation time. The results of this study indicate the creativity of Imron Sadewo in composing songs is always inspired by the background of his personal life, his relatives life and the surrounding environment. The creativity of Imron Sadewo is proven through four preparation stages; preparation, incubation, illumination and verification, also through the four creativity dimensions; person, proces, press, and product.
Treatment options for hypoplastic borderline left ventricle (LV) are critically dependent on the development of the LV itself and include different types of univentricular palliation or biventricular repair performed at birth. Since hybrid palliation allows deferring major surgery to 4–6 months, in borderline cases, the decision can be postponed until the LV has expressed its growth potential. We aimed to evaluate anatomic modifications of borderline LV after hybrid palliation. We retrospectively reviewed data from 45 consecutive patients with hypoplastic LV who underwent hybrid palliation at birth between 2011 and 2015. Sixteen patients (mean weight 3.15 Kg) exhibited borderline LV and were considered for potential LV growth. After 5 months, five patients underwent univentricular palliation (Group 1), eight biventricular repairs (Group 2) and three died before surgery. Echocardiograms of Groups 1 and 2 were reviewed, comparing LV structures at birth and after 5 months. Although, at birth, all LV measurements were far below the normal limits, after 5 months, LV mass in Group 2 was almost normal, while in Group 1, no growth was evident. However, aortic root diameter and long axis ratio were significantly higher in Group 2 already at birth. Hybrid palliation can be positively considered as a “bridge-to-decision” for borderline LV. Echocardiography plays a key role in monitoring the growth of borderline LV.
Over the last three decades tremendous progress has been made in the treatment of patients, particularly in newborns with congenital heart disease. This chapter address all hybrid procedures and focuses on hybrid strategies to treat patients with hypoplastic left heart syndrome (HLHS), hypoplastic left heart complex (HLHC), and other congenital anomalies of the left heart. The constellation of HLHC varies from a Shone complex with mitral, subaortic, aortic valve stenosis and coarctation to that of HLHS with an antegrade ascending aortic flow and duct‐dependent systemic circulation. The benefits of a simultaneous or consecutive surgical–interventional approach are described for multiple lesions and conditions. The chapter presents case reports based on experience in newborns with borderline left heart structures, some individualized hybrid strategies for children and young adults with “end‐stage” left heart failure.
Objective:
A subset of patients with borderline hypoplastic left heart may be candidates for single to biventricular conversion, but long-term morbidity and mortality persist. Prior studies have shown conflicting results regarding the association of preoperative diastolic dysfunction and outcome, and patient selection remains challenging.
Methods:
Patients with borderline hypoplastic left heart undergoing biventricular conversion from 2005 to 2017 were included. Cox regression identified preoperative factors associated with a composite outcome of time to mortality, heart transplant, takedown to single ventricle circulation, or hemodynamic failure (defined as left ventricular end-diastolic pressure >20 mm Hg, mean pulmonary artery pressure >35 mm Hg, or pulmonary vascular resistance >6 international Woods units).
Results:
Among 43 patients, 20 (46%) met the outcome, with a median time to outcome of 5.2 years. On univariate analysis, endocardial fibroelastosis, lower left ventricular end-diastolic volume/body surface area (when <50 mL/m2), lower left ventricular stroke volume/body surface area (when <32 mL/m2), and lower left:right ventricular stroke volume ratio (when <0.7) were associated with outcome; higher preoperative left ventricular end-diastolic pressure was not. Multivariable analysis demonstrated that endocardial fibroelastosis (hazard ratio, 5.1, 95% confidence interval, 1.5-22.7, P = .033) and left ventricular stroke volume/body surface area 28 mL/m2 or less (hazard ratio, 4.3, 95% confidence interval, 1.5-12.3, P = .006) were independently associated with a higher hazard of the outcome. Approximately all patients (86%) with endocardial fibroelastosis and left ventricular stroke volume/body surface area 28 mL/m2 or less met the outcome compared with 10% of those without endocardial fibroelastosis and with higher stroke volume/body surface area.
Conclusions:
History of endocardial fibroelastosis and smaller left ventricular stroke volume/body surface area are independent factors associated with adverse outcomes among patients with borderline hypoplastic left heart undergoing biventricular conversion. Normal preoperative left ventricular end-diastolic pressure is insufficient to reassure against diastolic dysfunction after biventricular conversion.
This study reviews our early experience with the “reverse” double switch operation (R-DSO) for borderline left hearts. A retrospective review of children with borderline left hearts who underwent R-DSO between 2017 and 2021 was conducted. Patient characteristics and early hemodynamic and clinical outcomes were collected. R-DSO was performed in 8 patients with no operative or postoperative deaths; 5 underwent decompressing bidirectional Glenn. Left ventricular (LV) poor-compliance was the dominant pathophysiology. Four patients had undergone staged LV recruitment but were not candidates for anatomical biventricular circulation due to LV hypoplasia and/or diastolic dysfunction. 7/8 patients had risk factors for Fontan circulation including pulmonary vein stenosis, pulmonary hypertension, and pulmonary artery stenosis. Median age at R-DSO was 3.7 years (19mo-12yrs). All patients were in sinus rhythm at discharge. At median follow-up of 15 months (57d-4.1yrs) no mortalities, reoperations or heart transplants had occurred. All patients had normal morphologic LV systolic function. In one patient, pre-existing pulmonary HTN resolved after R-DSO. Reinterventions included transcatheter mitral valve replacement for residual mitral stenosis and neo-pulmonary balloon valvuloplasty. In 4 patients follow-up catheterization done at a median of 519 days (320d-4y) demonstrated median cardiac index of 3.2 L/min/m² (2.2-4); median sub-pulmonary LVEDP was 9 mmHg (7-15); median IVC/baffle pressure was 8 mm Hg (7-13). R-DSO is an alternative to anatomical biventricular repair or single ventricle palliation in patients with borderline left hearts and can result in low IVC pressures and favorable early results. This approach can also relieve pulmonary HTN and allow future transplant candidacy.
Optimal decision-making to determine the type and timing of surgical intervention for various congenital heart disease (CHD) requires adequate understanding and interpretation of anatomic and physiologic data obtained from various imaging modalities. Cardiac magnetic resonance (CMR) has revolutionized the way we evaluate the anatomy and physiology of CHD. In addition to 2- and 3-dimensional anatomic data and volumetry, phase-contrast CMR allows quantitative measurements of cardiac output, pulmonary blood flow, pulmonary-to-systemic flow ratio, the amount of intracardiac shunt, valve regurgitation, and aortopulmonary collateral flows. This review article describes the utilization of CMR-derived flow data in surgical decision-making in three distinct subgroups: (1) patients with borderline left ventricle (LV) with emphasis on the ascending aortic flow and other physiologic parameters, (2) single ventricle patients who undergo bidirectional cavopulmonary shunt with emphasis on the impact of superior vena cava blood flow on postoperative physiology, and (3) patients with pulmonary atresia and major aortopulmonary collateral arteries with emphasis on the impact of total pulmonary blood flow and systemic-to-pulmonary flow ratio on clinical outcomes.
For neonates with critical congenital heart disease requiring intervention, transcatheter approaches for many conditions have been established over the past decades. These interventions may serve to stabilize or palliate to surgical next steps or effectively primarily treat the condition. Many transcatheter interventions have evidence-based records of effectiveness and safety, which have led to widespread acceptance as first-line therapies. Other techniques continue to innovatively push the envelope and challenge the optimal strategies for high-risk neonates with right ventricular outflow tract obstruction or ductal-dependent pulmonary blood flow. In this review, the most commonly performed neonatal transcatheter interventions will be described to illustrate the current state of the field and highlight areas of future opportunity.
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 syndrome (HLHS) is the most common anatomic lesion in children born with single ventricle physiology and is characterized by the presence of a dominant right ventricle and a hypoplastic left ventricle along with small left-sided heart structures. Diagnostic subgroups of HLHS reflect the extent of inflow and outflow obstruction at the aortic and mitral valves, specifically stenosis or atresia. If left unpalliated, HLHS is a uniformly fatal lesion in infancy. Following introduction of the Norwood operation, early survival has steadily improved over the past four decades, mirroring advances in operative and peri-operative management as well as reflecting refinements in patient surveillance and interstage clinical care. Notably, survival following staged palliation has increased from 0% to a 5-year survival of 60-65% for children in some centres. Despite the prevalence of HLHS in childhood with relatively favourable surgical outcomes in contemporary series, this cohort is only now reaching early adult life and longer-term outcomes have yet to be elucidated. In this article we focus on contemporary clinical management strategies for patients with HLHS across the lifespan, from fetal to adult life. Nomenclature and diagnostic considerations are discussed and current literature pertaining to putative genetic etiologies is reviewed. The spectrum of fetal and pediatric interventional strategies, both percutaneous and surgical, are described. Clinical, patient-reported and neurodevelopmental outcomes of HLHS are delineated. Finally, note is made of current areas of clinical uncertainty and suggested directions for future research are highlighted.
This chapter presents a general discussion of the issues surrounding adjustment of cardiovascular structures for age and body size and the methods that were used in the derivation of the models in the Pediatric Heart Network project. The relation of cardiovascular dimensions to body size and age can be observed empirically and analyzed statistically, based on measurements of cardiac and vascular structures derived from imaging data such as echocardiography. Echocardiography remains the primary source of empirical normative pediatric allometric data. Allometric relations can often be predicted theoretically from basic physiologic principles. In addition to the implications of allometry for patient care, adjustment for the effects of changes in body size has important implications for clinical research. The most common approach to adjusting cardiovascular structures for body size has been to calculate their ratio relative to body surface area.
This chapter presents information on morphology, classification, pathophysiology, and imaging of subaortic stenosis, aortic valve stenosis, supravalvar aortic stenosis, bicuspid aortic valve, aortic valve cusp prolapsed, sinus of valsalva aneurysm, and aortico‐left ventricular tunnel. Obstruction of the left ventricular outflow tract may occur below the level of the aortic valve, at the level of the aortic valve, or above the aortic valve, typically at the level of the sinotubular junction. Subaortic stenosis may be familial, but in most cases is felt to be the result of the interaction of a number of factors. The development of subaortic stenosis is thought to be due to abnormal shear forces in the left ventricular outflow tract, leading to abnormal growth of fibrous tissue. Aortic valve stenosis in children is due to a congenitally abnormal aortic valve rather than degeneration of a normal valve as may occur in late adult life.
For therapeutic decisions regarding uni- or biventricular surgical repair in congenital heart disease (CHD), left ventricular mass (LVM) is an important factor. The aim of this retrospective study was to determine the LVM of infants with CHD in thoracic computed tomography angiographies (CTAs) and to evaluate its usefulness as a prognostic parameter, with special attention paid to hypoplastic left heart (HLH) patients. Manual segmentation of the left ventricular endo- and epicardial volumes was performed in CTAs of 132 infants. LVMs were determined from these volumes and normalized to body surface area. LVMs of patients with different types of CHD were compared to each other using analyses of variances (ANOVA). An LVM cutoff for discrimination between uni- and biventricular repair was determined using receiver operating characteristics. Survival rates were calculated using Kaplan–Meier statistics. Patients with a clinical diagnosis of an HLH had significantly lower mean LVM (21.88 g/m2) compared to patients without applicable disease (50.22 g/m2; p < 0.0001) and compared to other CHDs, including persistent truncus arteriosus, left ventricular outflow tract obstruction, transposition of the great arteries, pulmonary artery stenosis or atresia, and double-outlet right ventricle (all, p < 0.05). The LVM cutoff for uni- vs. biventricular surgery was 33.9 g/m2 (sensitivity: 82.3%; specificity: 73.7%; PPV: 94.9%). In a subanalysis of HLH patients, a sensitivity of 50.0%, specificity of 100%, PPV of 100%, and NPV of 83.3% was determined. Patient survival was not significantly different between the surgical approaches or between patients with LVM above or below the cutoff. LVM can be measured in chest CTA of newborns with CHD and can be used as a prognostic factor.
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.
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.
Left-sided obstructive lesions of the heart may occur at various anatomic levels and with varying degrees of severity, ranging from a bicuspid aortic valve with minimal hemodynamic compromise to aortic atresia and hypoplastic left heart syndrome with profound hemodynamic derangements. These abnormalities in systemic blood flow may occur due to obstruction at multiple levels, such as seen in patients with Shone's complex, or from circumscribed obstruction at a single level, such as in hypertrophic cardiomyopathy, coarctation of the aorta, interrupted aortic arch, subvalvar, valvar, or supravalvar aortic stenosis. Depending on the specific type of left-sided obstruction, the child may be at risk for pulmonary hypertension and/or impaired coronary perfusion during anesthetic care. A clear understanding of the level and degree of the obstruction is critically important for optimal anesthetic management.
This study aimed to evaluate fetal echocardiographic parameters associated with neonatal intervention and single-ventricle palliation (SVP) in fetuses with suspected left-sided cardiac lesions. Initial fetal echocardiograms (1/2002–1/2017) were interpreted by the contemporary fetal cardiologist as coarctation of the aorta (COA), left heart hypoplasia (LHH), hypoplastic left heart syndrome (HLHS), mitral valve hypoplasia (MVH) ± stenosis, and aortic valve hypoplasia ± stenosis (AS). The cohort comprised 68 fetuses with suspected left-sided cardiac lesions (COA n = 15, LHH n = 9, HLHS n = 39, MVH n = 1, and AS n = 4). Smaller left ventricular (LV) length Z score, aortic valve Z score, ascending aorta Z score, and aorta/pulmonary artery ratio; left-to-right shunting at the foramen ovale; and retrograde flow in the aortic arch were associated with the need for neonatal intervention (p = 0.005–0.04). Smaller mitral valve (MV) Z score, LV length Z score, aortic valve Z score, ascending aorta Z score, aorta/pulmonary artery ratio, and LV ejection fraction, as well as higher tricuspid valve-to-MV (TV/MV) ratio, right ventricular-to-LV (RV/LV) length ratio, left-to-right shunting at the foramen ovale, abnormal pulmonary vein Doppler, absence of prograde aortic flow, and retrograde flow in the aortic arch were associated with SVP (p < 0.001–0.008). The strongest independent variable associated with SVP was RV/LV length ratio (stepwise logistical regression, p = 0.03); an RV/LV length ratio > 1.28 was associated with SVP with a sensitivity of 76% and specificity of 96% (AUC 0.90, p < 0.001). A fetal RV/LV length ratio of > 1.28 may be a useful threshold for identifying fetuses requiring SVP.
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.
Hypoplastic left heart syndrome describes a spectrum of cardiac anomalies characterized by hypoplasia of the left heart including hypoplasia, stenosis or atresia of mitral and aortic valve as well as hypoplasia of the ascending aorta and aortic arch. The diagnosis of HLHS is established by 2D echo in the precordial views showing hypoplasia of the left ventricle as well as hypoplasia, stenosis or atresia of the mitral and aortic valve. Hypoplasia of the ascending aorta and the aortic arch can be displayed from the high parasternal and suprasternal window. Colour Doppler is helpful to discriminate stenosis of the mitral and aortic valve from atresia. Retrograde perfusion of the aortic arch and of the ascending aorta can be displayed from the suprasternal window. Colour Doppler interrogation is important to confirm unrestrictive patency of the ductus arteriosus and to assess the size of the interatrial communication. The shunting pattern across the ductus arteriosus can be displayed by PW and CW Doppler, revealing possible gradients if the ductus arteriosus becomes restrictive. Restriction of the interatrial communication is confirmed by PW and CW Doppler interrogation of left to right shunting showing acceleration of flow and transition from phasic to continuous flow pattern.
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