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Validation and Re-Evaluation of a Discriminant Model Predicting Anatomic Suitability for Biventricular Repair in Neonates With Aortic Stenosis
Abstract
The purpose of this study was to validate and re-evaluate our previously reported scoring systems for predicting optimal management in neonates with aortic stenosis (AS).
In 1991, we reported a multivariate discriminant equation and an ordinal scoring system for predicting which neonates with AS are suitable for biventricular repair and which are better served by single ventricle management.
Retrospective analysis was performed to: 1) validate our scoring systems in 89 additional neonates with AS and normal mitral valve area, 2) assess the effects of 5% measurement variation on predictive scores, 3) evaluate our cohort with the Congenital Heart Surgeons' Society scoring system, and 4) repeat the discriminant analysis on the basis of all 126 patients.
The original scores each predicted outcome accurately in 68 patients (77%). Minor (5%) measurement variation changed the outcome predicted by the discriminant equation in 8 patients (9%) and by the threshold system in 13 patients (15%). The most accurate model for predicting survival with a biventricular circulation among the full cohort is: 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. With a cutoff of -0.65, outcome was predicted accurately in 90% of patients.
Both of our original scoring systems are less accurate at predicting outcome than in our original analysis. Revised discriminant analysis yielded a model similar to our original equation that was 90% accurate at predicting survival with a biventricular circulation among neonates with AS and a mitral valve area z-score >-2.
... The decision whether to perform biventricular repair (BVR) or univentricular palliation (UVP) in neonates and infants affected by multiple left heart obstructive lesions and borderline left ventricle (BLV) is always challenging [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16], and knowledge regarding the long-term prognosis of these patients remains scarce. Various scores utilizing echocardiography data have been proposed as a means of predicting the efficacy of BVR in BLV [2][3][4][5]9,14]; however, these are solely based on short-term results [3,4,14], with a mean follow-up period of 5-6 years. ...
... The decision whether to perform biventricular repair (BVR) or univentricular palliation (UVP) in neonates and infants affected by multiple left heart obstructive lesions and borderline left ventricle (BLV) is always challenging [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16], and knowledge regarding the long-term prognosis of these patients remains scarce. Various scores utilizing echocardiography data have been proposed as a means of predicting the efficacy of BVR in BLV [2][3][4][5]9,14]; however, these are solely based on short-term results [3,4,14], with a mean follow-up period of 5-6 years. Furthermore, these scoring systems are not without limitations, which include heterogeneity in the echocardiographic parameters and outcome measures evaluated, as well as the retrospective design during the score development [2][3][4][5]9,14]. ...
... Various scores utilizing echocardiography data have been proposed as a means of predicting the efficacy of BVR in BLV [2][3][4][5]9,14]; however, these are solely based on short-term results [3,4,14], with a mean follow-up period of 5-6 years. Furthermore, these scoring systems are not without limitations, which include heterogeneity in the echocardiographic parameters and outcome measures evaluated, as well as the retrospective design during the score development [2][3][4][5]9,14]. Additionally, significant variations in inclusion criteria were observed among the studies from differing authors [2][3][4][5]9,14]. ...
Background: The decision to perform biventricular repair (BVR) in neonates and infants presenting with either single or multiple left ventricle outflow obstructions (LVOTOs) and a borderline left ventricle (BLV) is subject to extensive discussion, and limited information is known regarding the long-term outcomes. As a result, the objective of this study is to critically assess and summarize the available data regarding the prognosis of neonates and infants with LVOTO and BLV who underwent BVR. Methods: In February 2023, we conducted a review study with three different medical search engines (the National Library of Medicine, Science Direct, and Cochrane Library) for Medical Subject Headings and free text terms including “congenital heart disease”, “outcome”, and “borderline left ventricle”. The search was refined by adding keywords for “Shone’s complex”, “complex LVOT obstruction”, “hypoplastic left heart syndrome/complex”, and “critical aortic stenosis”. Results: Out of a total of 51 studies, 15 studies were included in the final analysis. The authors utilized heterogeneous definitions to characterize BLV, resulting in considerable variation in inclusion criteria among studies. Three distinct categories of studies were identified, encompassing those specifically designed to evaluate BLV, those focused on Shone’s complex, and finally those on aortic stenosis. Despite the challenges associated with comparing data originating from slightly different cardiac defects and from different eras, our results indicate a favorable survival rate and clinical outcome following BVR. However, the incidence of reintervention remains high, and concerns persist regarding residual pulmonary hypertension, which has been inadequately investigated. Conclusions: The available data concerning neonates and infants with LVOTO and BLV who undergo BVR are inadequate and fragmented. Consequently, large-scale studies are necessary to fully ascertain the long-term outcome of these complex defects.
... From the apical 4-chamber view were obtained mitral and tricuspid annulus in protodiastole, systolic and diastolic LV volumes and LAR. In particular, LAR was calculated as the ratio of LV long axis from the mitral plane to the endocardial border, divided by the heart long axis from the tricuspid plane to the epicardial border of the right ventricle ( Figure 3) [15]. In the case of AVSD, annular planes and ventricular volumes were measured by tracing an imaginary line of the crux cordis from the edge of the ventricular septum [16]. ...
... Finally, we applied the original "Rhodes score" and the "Threshold score" reported by Rhodes et al. [17] and the revised "Discriminant score" described by Colan et al. [15] to all the patients at birth in order to test the potential predictive value of the existing scores in this population with multi-level left heart obstruction, even though the same scores were created for critical aortic stenosis. Requested parameters include weight and height, aortic annulus (mm), aortic root (mm), mitral annulus (mm) measured in apical four-chamber and parasternal long axis views, LV long axis and heart long axis (mm), and grade of endocardial fibroelastosis (0-3). ...
... Some of these criteria are the "Rhodes scores" [17], the apex-non-forming LV, the endomyocardial fibroelastosis, indexed LV end-diastolic volume <20 mL/m 2 , elevated end-diastolic pressure and depressed contractile function of the LV [23]. In particular, the "threshold score" of Rhodes is an ordinal scoring system based on four points: indexed aortic root diameter < 3.5 cm/m 2 ; indexed MV area < 4.75 cm 2 /m 2 ; LAR < 0.8; indexed LV mass < 35 g/m 2 ; a score of two or more means the ventricle is not suitable for biventricular repair [15,17]. ...
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.
... Several echocardiographic formulas are used to predict biventricular repair (BVR) risk prediction for varying etiologies of complex congenital heart disease (CHD) with borderline left ventricle (LV) [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19]. ...
... Several risk scores to predict the feasibility of BVR with either a left outflow tract (LVOT) obstruction or a diminutive left ventricle have been developed and validated [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18]. Examples include scores for assessment of critical aortic stenosis [1][2][3], critical left ventricle outflow (LVOT) obstruction occurring at any level, from the sub-aortic region to the innominate artery [4,5], hypoplastic left heart complex (HLHC) [6], aortic arch hypoplasia, and small left heart structures [7]. ...
... Several risk scores to predict the feasibility of BVR with either a left outflow tract (LVOT) obstruction or a diminutive left ventricle have been developed and validated [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18]. Examples include scores for assessment of critical aortic stenosis [1][2][3], critical left ventricle outflow (LVOT) obstruction occurring at any level, from the sub-aortic region to the innominate artery [4,5], hypoplastic left heart complex (HLHC) [6], aortic arch hypoplasia, and small left heart structures [7]. In addition, several indices and scores have been developed and validated for BVR unbalanced atrioventricular septal defects (uAVSD) with right ventricle (RV) dominance and a diminutive LV [10][11][12][13][14][15][16][17][18][19]. ...
The aim of this review is to highlight the strengths and limitations of major echocardiographic biventricular repair (BVR) prediction models for borderline left ventricle (LV) in complex congenital heart disease (CHD). A systematic search in the National Library of Medicine for Medical Subject Headings and free text terms including echocardiography, CHD, and scores, was performed. The search was refined by adding keywords for critical aortic stenosis (AS), borderline LV, complex left ventricular outflow tract (LVOT) obstruction, hypoplastic left heart syndrome/complex (HLHS/HLHC), and unbalanced atrio-ventricular septal defects (uAVSD). Fifteen studies were selected for the final analysis. We outlined what echocardiographic scores for different types of complex CHD with diminutive LV are available. Scores for CHD with LVOT obstruction including critical AS, HLHS/HLHC, and aortic arch hypoplasia have been validated and implemented by several studies. Scores for uAVSD with right ventricle (RV) dominance have also been established and implemented, the first being the atrioventricular valve index (AVVI). In addition to AVII, both LV/RV inflow angle and LV inflow index have all been validated for the prediction of BVR. We conclude with a discussion of limitations in the development and validation of each of these scores, including retrospective design during score development, heterogeneity in echocardiographic parameters evaluated, variability in the definition of outcomes, differences in adopted surgical and Interventional strategies, and institutional differences. Furthermore, scores developed in the past two decades may have little clinical relevance now. In summary, we provide a review of echocardiographic scores for BVR in complex CHD with a diminutive LV that may serve as a guide for use in modern clinical practice.
... Over the years, different nomograms have been published. The Z-score calculator from the Marfan Foundation 7 is based on the publication by Colan et al, 8 but their website also refers to three other publications on aortic root Z-score calculations. [9][10][11] The Marfan guideline even refers to a fifth nomogram. ...
... Body surface area was calculated in two ways. First, it was calculated according to Du Bois and Du Bois, 15 which was used by Pettersen et al 9 and Gautier et al. 10 Second, body surface area was calculated according to Haycock,16 which was used by Lopez et al 14 and Colan et al. 8 ...
... The aortic root diameters were measured as described in the echocardiographic nomogram publications. [8][9][10]14 In brief, the aortic annulus was measured from hinge point to hinge point during systole in all nomogram publications. However, the sinus of Valsalva and the sinotubular junction were measured using two different methods; inner edge to inner edge in maximum systole (according to Pettersen et al, 9 Colan et al, 8 and Lopez et al 14 (Fig. 2a)) and leading edge to leading edge in diastole (according to Gautier et al 10 (Fig. 2b)). ...
Background
Aortic root dilation is a major complication of Marfan syndrome and is one of the most important criteria in establishing the diagnosis. Currently, different echocardiographic nomograms are used to calculate aortic root Z-scores. The aim of the present study was to assess the potential differences in aortic root measurements when aortic root Z-scores were obtained in a cohort of paediatric Marfan patients using several published nomograms.
Methods
In a cohort of 100 children with Marfan syndrome, Z-scores for aortic root dimensions were calculated according to the nomograms of Pettersen et al, Gautier et al, Colan et al, and Lopez et al. Bland–Altman plots were used to estimate mean differences in Z-scores and to establish limits of agreement.
Results
The mean Z-score of the sinus of Valsalva for Lopez et al was significantly higher compared to Gautier et al (p < 0.01) and Pettersen et al (p = 0.03). The nomogram of Lopez et al resulted in substantially higher Z-scores in patients with a large sinus of Valsalva diameter. Thirty-five percentage of the studied patients would have a Z-score ≥ 2 using Lopez et al compared to 20% for Pettersen et al, 21% for Gautier et al, and 33% for Colan et al.
Conclusion
The currently available nomograms for calculating Z-scores of aortic dilation in children with Marfan syndrome lead to clinically relevant differences in Z-scores, especially in children with a relative large aortic root diameter. This could have impact on both the diagnosis and treatment of patients with Marfan syndrome.
... Considering the growth-related changes, the aortic diameter was standardized as the z fraction, that was, the ratio of the aortic diameter to the square root of the body surface area (BSA). The aortic diameter z fraction was determined according to the description of Colan et al. [20] and confirmed by the method described by Dallaire et al. [21] The BSA of all patients was calculated by the DuBois formula [22] . Aortic dilatation was defined as the diameter of the main artery z score > 2.0 [20,21] . ...
... The aortic diameter z fraction was determined according to the description of Colan et al. [20] and confirmed by the method described by Dallaire et al. [21] The BSA of all patients was calculated by the DuBois formula [22] . Aortic dilatation was defined as the diameter of the main artery z score > 2.0 [20,21] . Age, sex, degree of coarctation, CoA complexity, hypertension, and coarctation segment length were set as the main related factors of arterial internal diameter. ...
Objective
To evaluate the occurrence of aortic dilatation and its associated predictors with coarctation of the aorta (CoA) in infants using multi-slice computed tomography (MSCT).
Methods
The clinical data of 47 infantile patients with CoA diagnosed by MSCT and 28 infantile patients with simple ventricular septal defect were analyzed retrospectively. Aortic diameters were measured at six different levels, and aortic sizes were compared by z score. The coarctation site-diaphragm ratio was used to describe the degree of narrowing. Relevant clinical data were collated and analyzed.
Results
The dilation rate and z score of the ascending aorta in the severe CoA group were significantly higher than those in the mild CoA group (11 [52.38%] vs. 21 [80.77%], P=0.038 and 2.00 ± 0.48 vs. 2.36 ± 0.43, P=0.010). Pearson’s correlation analysis found that the z score of the ascending aorta was negatively correlated with the coarctation site-diaphragm ratio value (r=-0.410, P=0.004). A logistic retrospective analysis found that an increased degree of coarctation was an independent predictor of aortic dilatation (adjusted odds ratio 0.002; 95% confidence interval 0.00-0.819; P=0.043). The z score of the ascending aorta in the severe CoA group was significantly higher than that in the ventricular septal defect group (P<0.05).
Conclusion
Most infants with CoA can also have significant dilatation of the ascending aorta, and the degree of this dilatation is related to the degree of coarctation. Assessment of aortic diameter and related malformations by MSCT can predict the risk of aortic dilatation in infants with CoA.
... 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]. ...
... Endocardial fibroelastosis was added to the new formula. With the new equation with a cutoff of − 0.65, outcome was predicted accurately in 90% of patients [15]. ...
... Echocardiography has several limitations while assessing the left ventricle. Lack of uniform z-scores and an impact of even minor variations on the outcomes of scores make them quiet arbitrary rather than exact [15,19]. Indeed, there is a variation in echocardiographic measurements and intraoperative findings [20]. ...
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.
... 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. ...
... Various LV scores calculated retrospectively would favour the same decision. 3 The fact that there were persisting signs of pulmonary hypertension and Damus-Kaye-Stansel procedure was performed later than usual, at the age of 5 weeks, presented an undisputable risk. Despite this, Fontan circulation was completed uneventfully. ...
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.
... Discriminant score (2006) [8]: ...
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.
... Olgun et al. demonstrated that PLT activation was increased in patients with cyanotic congenital heart disease [18]. According to our analysis, preoperative Hb was negatively and SaO2 was positively correlated with PLT, highlighting the negative correlation between z scores of aortic dimension and PLT [19,20]. ...
Background
Aortic root dilation is an increasingly recognized feature in repaired tetralogy of Fallot (TOF) patients. However, the dilation at the aortic root and ascending aorta in unrepaired TOF is rarely studied. This study aims to confirm whether aortic dilation is a common feature in unrepaired TOF and investigate the factors attributed to aortic dilation.
Methodology
Patients with an echocardiographic diagnosis of TOF undergoing computed tomography angiography were retrospectively studied. Diameters and z scores of aortic annular (Ao1), aortic sinotubular junction (Ao2), ascending aorta (Ao3), and distal transverse aortic arch (Ao4) were measured. Preoperative body surface area (BSA), hemoglobin (Hb), hematocrit (HCT), arterial oxygen saturation (SaO2), and platelet (PLT) count were recorded.
Results
A total of 101 TOF patients aged 6.8 ± 9.5 years were included in this study, whose mean BSA (m²), Hb (g/L), HCT, SaO2 (%), and PLT (10⁹/L) were 0.7 ± 0.4, 162.1 ± 3.8, 0.5 ± 0.1, 85.1 ± 9.3, and 238.1 ± 101.1, respectively. The mean z score of Ao1, Ao2, Ao3, and Ao4 were 10.3 ± 3.5, 4.7 ± 2.9, 4.0 ± 2.7, and 4.1 ± 2.4, respectively. Age and BSA were positively correlated with the z scores of Ao1 and Ao2. Preoperative Hb and HCT were positively correlated with the z scores of Ao1, Ao2, Ao3, and Ao4. Preoperative SaO2 and PLT were negatively correlated with the z scores of Ao1, Ao2, Ao3, and Ao4.
Conclusions
Aortic dilation is common in unrepaired TOF patients. The dilation of different levels of the aorta was correlated with age, BSA, preoperative Hb, HCT, SaO2, and PLT.
... The maximum diameters of each aortic structure, as determined from the chest CT, were re-calculated as z-scores using the reference values from Colan's study for the aortic root and Gautier's study for the ascending aorta [8,9]. In our study, "significant dilatation of the aortic structures" was defined as a z-score of 2 or greater, corresponding to the 95% confidence interval, for each respective diameter [10]. ...
Objectives
Dilatation of the aortic root structures or ascending aorta is often observed in patients with Fallot-type anomalies. We aimed to determine the dilation rate of the aortic structures and investigate strategies for managing this phenomenon.
Methods
In this retrospective study, we enrolled 66 out of 801 patients who underwent corrective surgery for Fallot-type anomalies (tetralogy of Fallot [TOF] and Fallot-type double outlet right ventricle [DORV]) between 2004 and 2020. These 66 patients had follow-up cardiac computed tomography (CT) angiography images taken at least 5 years after the initial CT study. We analyzed the diameters and aortic cross-sectional area/height ratio (AH) of the aortic annulus, sinus of Valsalva, sinotubular junction, and ascending aorta between the initial and follow-up CT scans. “Dilatation” was defined as a z-score over 2 for each aortic structure.
Results
The median age at the initial and follow-up CT scans was 5.9 years (interquartile range [IQR]: 0.4 ~ 12.4) and 15.9 years (IQR 9.3 ~ 23.4), respectively. The median CT interval (from initial to latest CT) was 9.5 years (IQR 6.6 ~ 12.0). The sinus of Valsalva exhibited the most significant dilation (32.8 mm at follow-up CT) over the study period. The AH ratio increased significantly in all four aortic structures. The patient’s age was significantly associated with higher AH in the follow-up CT. Aortic dilatation was present in 74.2% of patients at the initial CT and increased to 86.4% at the follow-up CT.
Conclusion
In Fallot-type anomalies, the AH ratio of aortic root structures significantly increased over an average period of approximately 9.5 years. The number of patients diagnosed with aortic dilatation also increased. Based on our observations in this study, these patients’ group should be considered for more frequent follow-up examinations, as significant dilatation could occur in their mid-20 s.
... Unfortunately, there is no consensus regarding the criteria of the appropriateness of biventricular repair for borderline HLHS instead of what is available for critical aortic stenosis [34,35]. The long-standing dispute about the appropriate way to address a borderline LV is still a matter of debate. ...
Hypoplastic left heart syndrome is a spectrum of complex congenital cardiac defects. Although in borderline cases, biventricular repair is a viable option, in the majority of cases, univentricular palliation is the treatment of choice. Hybrid palliation can be a valid alternative to classic Norwood operation in the neonatal period, especially in selected cases such as high-risk patients or borderline left ventricles. Echocardiography is the main diagnostic modality in this pediatric population, from the fetal diagnosis to the subsequent surgical steps of palliative treatment. Hybrid palliation is performed after birth and is characterized by surgical banding of the pulmonary arteries along with transcatheter stenting of the ductus arteriosus. There are some peculiar aspects of cardiac imaging that characterize this type of palliation, and that should be considered in the different phases before and after the procedure. We aimed to review the current literature about the role of echocardiography in the management of patients with hypoplastic left heart undergoing hybrid palliation.
... 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 has been a number of studies which attempt to aid in management of these patients, however, many questions remain. [542][543][544] There are other lesions which occur in pediatric and CHD where the assessment of biventricular performance is important. Patients with anomalous coronary artery origins and courses, patients with surgically manipulated coronary arteries (see section on Coronary arteries), and patients with myocarditis (see Myocarditis section) are all examples where ventricular performance are useful. ...
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.
... 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.
... 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]. ...
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.
... The aorta was measured on the sinus from the leading edge to leading edge. Aortic root diameter z scores adjusted for body surface area were determined as described by Colan et al. [23] and confirmed using the method described by Dallaire et al. [24]. The body surface area was calculated using the Haycock formula [25]. ...
Background:
Cardiovascular abnormalities have been observed in patients with mucopolysaccharidosis (MPS) of any type, with the most documented abnormalities being valvular regurgitation and stenosis and cardiac hypertrophy. Only a few studies have focused on aortic root dilatation and the long-term effects of enzyme replacement therapy (ERT) in these patients.
Methods:
We reviewed echocardiograms of 125 Taiwanese MPS patients (age range, 0.1 to 19.1 years; 11 with MPS I, 49 with MPS II, 25 with MPS III, 29 with MPS IVA, and 11 with MPS VI). The aortic root diameter was measured at the sinus of Valsalva.
Results:
Aortic root dilatation (z score >2) was observed in 47% of the MPS patients, including 66% of MPS IV, 51% of MPS II, 45% of MPS VI, 28% of MPS III, and 27% of MPS I patients. The mean aortic root diameter z score was 2.14 (n = 125). The patients with MPS IV had the most severe aortic root dilatation with a mean aortic root diameter z score of 3.03, followed by MPS II (2.12), MPS VI (2.06), MPS III (1.68), and MPS I (1.03). The aortic root diameter z score was positively correlated with increasing age (n = 125, p < 0.01). For the patients with MPS II, III, and IV, aortic root diameter z score was also positively correlated with increasing age (p < 0.01). For 16 patients who had received ERT and had follow-up echocardiographic data (range 2.0-16.2 years), the mean aortic root diameter z score change was -0.46 compared to baseline (baseline 2.49 versus follow-up 2.03, p = 0.490).
Conclusions:
Aortic root dilatation was common in the patients with all types of MPS, with the most severe aortic root dilatation observed in those with MPS IV. The severity of aortic root dilatation worsened with increasing age, reinforcing the concept of the progressive nature of this disease. ERT for MPS appears to stabilize the progression of aortic root dilatation.
... 35,36 Conversely, in children and in individuals ≤ 25 years, aortic root dimensions have been validated using the inner-wall-to-inner-wall technique during systole. 37 The use of these two different techniques may significantly influence the result of the echocardiographic examination and a careful selection of the correct method is mandatory in order to properly measure aortic size (see Figure 2). Notably, F I G U R E 1 Echocardiographic examination of aortic dimension in a 14-year-old patient with bicuspid aortic valve (BAV). ...
Bicuspid aortic valve (BAV) is the most common congenital heart defect in adults. Although a BAV may remain without clinical consequences for a lifetime, it can dete- riorate in aortic valve stenosis and regurgitation and aortic dilatation. Unfortunately, the impact of regular training on patients with BAV and its natural course is not fully understood, although preliminary evidence suggests that the progression of valvular disease occurs primarily in an independent manner from sports practice. The current review aims to report how to perform a comprehensive echocardiographic examination in athletes with BAV and analyze the current literature on the influence of sports practice and how it impacts the aortic valve in athletes with BAV. The article also summarizes the current recommendations on sports eligibility and disqualification for competitive athletes with BAV.
... Diameters were adjusted for body surface area to calculate Z-scores using nomograms from Boston Children's Hospital (zscore.chboston.org) [9]. Aortic valve stenosis or insufficiency and the presence of mitral valve prolapse were recorded if identified on any echocardiography reports reviewed. ...
Longitudinal changes in aortic diameters of young patients with thoracic aortic aneurysm (TAA) have not been completely described, particularly over long periods of follow-up. This retrospective study sought to characterize the rates of proximal aortic dilation in young patients, identify risk factors for TAA progression, and evaluate the predictive utility of early echocardiographic follow-up. Inclusion criteria were: (1) TAA or TAA-predisposing genetic diagnosis, (2) age < 25 years at first echocardiogram, and (3) minimum of 5 years of echocardiographic follow-up. Proximal aortic diameters were measured by echocardiography and Z-scores calculated to index for body surface area. TAA severity was classified as no TAA (Z-score < 2), mild (Z-score 2 to 4), or at least moderate (Z-score > 4). Among 141 included patients, mean age at first echocardiogram was 7.3 ± 3.5 years. Mean follow-up duration was 9.8 ± 3.5 years. Fifty five patients had a genetic syndrome, and 38 of the non-syndromic patients had bicuspid aortic valve (BAV). The rate of aortic dilation was significantly higher at the ascending aorta than other aortic segments. BAV and age > 10 years at first echocardiogram were associated with increased rate of ascending aorta dilation. At the ascending aorta, over 25% of patients had categorical increase in TAA severity between first and last echocardiograms, and such patients demonstrated higher rate of dilation within their first 2 years of follow-up. These longitudinal findings highlight progressive ascending aorta dilation in young patients, which may worsen around adolescence. This may help determine timing of follow-up and target ages for clinical trials.
This document has been developed to provide a guide for basic and advanced reporting in paediatric echocardiography. Furthermore, it aims to help clinicians in the interpretation of echocardiographic measurements and functional data for estimating the severity of disease in different paediatric age groups. The following topics will be reviewed and discussed in the present document: (i) the general principle in constructing a paediatric echocardiographic report, (ii) the basic elements to be included, and (iii) the potential and limitation of currently employed tools used for disease severity quantification during paediatric reporting. A guide for the interpretation of Z-scores will be provided. Use and interpretation of parameters employed for quantification of ventricular systolic function will be discussed. Difficulties in the adoption of adult parameters for the study of diastolic function and valve defects at different ages and pressure and loading conditions will be outlined, with pitfalls for the assessment listed. A guide for careful use of prediction scores for complex congenital heart disease will be provided. Examples of basic and advanced (disease-specific) formats for reporting in paediatric echocardiography will be provided. This document should serve as a comprehensive guide to (i) structure a comprehensive paediatric echocardiographic report; (ii) identify the basic morphological details, measures, and functional parameters to be included during echocardiographic reporting; and (iii) correctly interpret measurements and functional data for estimating disease severity.
Background: Most patients with significant left ventricular (LV) hypoplasia undergo single ventricle (SV) palliation, but biventricular (Bi-V) repair is viable in some patients with borderline LV hypoplasia. We sought to identify CMR (cardiovascular magnetic resonance) criteria predictive of successful primary Bi-V repair in neonates with borderline LV hypoplasia without significant stenosis of the mitral valve (MV) and aortic valve (AV), and to determine reasons for reintervention after successful Bi-V repair.
Methods: This retrospective study included all patients with borderline LV hypoplasia who underwent CMR from 2003-2024 for surgical decision-making. Patients with abnormal segmental connections, atrioventricular septal defects, unrestrictive ventricular septal defects, those with more than mild MV stenosis (mean Doppler flow gradient > 5 mmHg) and/or more than mild AV stenosis (peak Doppler flow gradient >20 mmHg) were excluded. Patients were divided into two groups based on initial intervention - primary Bi-V repair and hybrid/ other staging procedure. Outcomes were categorized as successful primary Bi-V repair, successful staged Bi-V repair and failure to achieve Bi-V repair (hybrid followed by SV palliation, transplant, death). Fisher exact test and Mann-Whitney U test was utilized to explore potential relationships. ROC curves were used to test diagnostic accuracy of parameters to predict successful primary Bi-V repair.
Results: Among 37 patients meeting the inclusion criteria, 23 (62%) patients underwent successful primary Bi-V repair, 8 (22%) underwent staged Bi-V repair, 6 (16%) failed to achieve Bi-V repair. Patients who underwent successful primary/ staged Bi-V repair had higher values for left ventricular diastolic volume index (LVEDVi 28 mL/m2 vs. 17.4.00 mL/m2; p <0.002), higher blood flow volume through the ascending aorta (QAo:1.99 L/min/m2 vs. 0.97 L/min/m2, p <0.012), and QAo / superior vena cava (QSVC) flow ratio (1.44 vs. 0.85, p =0.034) compared to those who had failure to achieve Bi-V repair. CMR LVEDVi cutoff of CMR 27 mL/m2, had 87% sensitivity and 79% specificity with an AUC of 87.6% and QAo threshold of 1.9 L/min/m2 had 65.2% sensitivity and 92.9% specificity (AUC: 86.0%) to predict successful primary Bi-V repair. Of 31 patients with primary or staged Bi-V repair, 7 (22%) underwent reinterventions for LVOT obstruction followed by mitral stenosis.
Conclusions: CMR plays a critical role in pre-operative evaluation, surveillance and decision-making in patients with borderline LV hypoplasia. In patients with borderline LV hypoplasia without MV/AV stenosis, successful primary Bi-V repair can be achieved when the CMR-derived LVEDVi is >27 mL/m2 and QAo is > 1.99 L/min/m2.
Marfan syndrome (MIM: # 154700; MFS) is an autosomal dominant disease representing the most common form of heritable connective tissue disorder. The condition presents variable multiorgan expression, typically involving a triad of cardiovascular, eye, and skeletal manifestations. Other multisystemic features are often underdiagnosed. Moreover, the disease is characterized by age related penetrance. Diagnosis and management of MFS in the adult population are well-described in literature. Few studies are focused on MFS in the pediatric population, making the clinical approach (cardiac and multiorgan) to these cases challenging both in terms of diagnosis and serial follow-up. In this review, we provide an overview of MFS manifestations in children, with extensive revision of major organ involvement (cardiovascular ocular and skeletal). We attempt to shed light on minor aspects of MFS that can have a significant progressive impact on the health of affected children. MFS is an example of a syndrome where an early personalized approach to address a dynamic, genetically determined condition can make a difference in outcome. Applying an early multidisciplinary clinical approach to MFS cases can prevent acute and chronic complications, offer tailored management, and improve the quality of life of patients.
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.
Congenital abnormalities of left ventricular inflow and outflow include abnormalities of the left atrium, mitral valve (supravalvar, valvar, and subvalvar) and abnormalities of the left ventricular outflow tract, the aortic valve, and supravalvar area. Cardiac magnetic resonance imaging (CMR) has become an important adjunctive tool in evaluating and following patients with this group of anomalies. This chapter reviews the role of CMR in the care of patients with congenital abnormalities of left ventricular inflow and outflow. In addition to describing the morphologic abnormalities and their clinical presentations, the indications and limitations of CMR in each condition are discussed, and a suggested CMR examination protocol is provided.
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.
Objectives
Dilatation of the aortic root structures or ascending aorta is often observed in patients with Fallot type anomalies. We tried to find how fast the aortic structures dilate and how to manage this phenomenon.
Methods
Among 801 patients who underwent corrective surgery of Fallot type anomalies, [tetralogy of Fallot (TOF) and Fallot type of double outlet right ventricle (DORV)] from 2004 to 2020, 66 patients who had follow-up cardiac computed tomography angiography (CT) images at least over 5-year interval after the initial CT study were enrolled in this retrospective study. We analyzed the diameters and aortic cross-sectional area/height ratio (AH) of aortic annulus, sinus of Valsalva, sinotubular junction and ascending aorta between initial and follow-up CTs. "Dilatation” was defined as a z-score over 2 in each aortic structure.
Results
The median age at initial and follow-up CTs were 5.9 years (Interquartile range (IQR): 0.4~12.4) and 15.9 years (IQR: 9.3~23.4), respectively. The median CT interval (initial ~ latest CT) was 9.5 years (IQR: 6.6~12.0). Sinus of Valsalva was the aortic structure that dilated the most rapidly (0.94 mm/year) and significantly (32.8 mm at follow-up CT) across the study period. The AH ratio increased significantly in the four aortic structures. Patient’s age was significantly associated with higher AH in follow-up CT. Aortic dilatation was found in 74.2% at initial CT and 86.4% at follow-up CT.
Conclusions
In Fallot type anomalies, the AH ratio of aortic root structures significantly increased over about 9.5 years. The number of the patients diagnosed within the range of aortic dilatation also increased. Shorter interval for regular follow-up should be considered for these young patients because it could reach a significant dilatation in their mid-20s according to our observation in this study.
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.
Hypoplastic left heart syndrome (HLHS) without intrinsic valvar stenosis or atresia is synonymous with the term hypoplastic left heart complex (HLHC) and is defined as a cardiac malformation at the milder end of the spectrum of HLHS with normally aligned great arteries without a common atrioventricular junction, characterized by underdevelopment of the left heart with significant hypoplasia of the left ventricle and hypoplasia of the aortic or mitral valve, or both valves, in the absence of intrinsic valvar stenosis or atresia, and with hypoplasia of the ascending aorta and aortic arch. This article describes the definitions, nomenclature, and classification of HLHC; the indications and contraindications for biventricular repair of HLHC; the surgical treatment of HLHC; and the associated outcomes.
Decision-making for biventricular repair (BVR) or univentricular palliation is challenging in neonates with hypoplastic left heart complex (HLHC). Hybrid strategy can be used successfully to achieve BVR in some of these patients. Between June 1998 and January 2022, 342 patients with a diagnosis of HLHS/variants, ductal-dependent lower body circulation with two ventricles, or HLHC with borderline left ventricle (LV) underwent initial bilateral pulmonary artery banding and ductal stenting in our institution. Among these 342 patients, 224 patients were defined as HLHS/variants and underwent univentricular palliation. 118 patients were determined to have borderline LV and hypoplastic left ventricular structures (HLHC, n = 48) or ductal-dependent lower body circulation with two ventricles (n = 70) considered suitable for BVR. 48 patients had multilevel obstructions including both aortic and mitral valve hypoplasia, aortic arch hypoplasia, and borderline LV. These were considered to have HLHC. These 48 HLHC patients are the subject of this report. Neonatal hybrid palliation at a median age of seven days was performed in 48 patients and 46 patients underwent BVR at a median age of 156 days. In 46 patients who underwent BVR, hospital mortality was not observed. The median follow-up was 66 months, with no late mortality. Heart transplant-free survival at 5, 10, and 15 years was 95.7%. 12 patients (26%) required reoperation and 9 (19.5%) required catheter reinterventions. Hybrid palliation may increase the chances for biventricular circulation in patients with borderline LV and small left-sided structures. Giessen hybrid approach as a left ventricular recruitment strategy achieves excellent early and long-term results.
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.
Objective
We reviewed our center's prenatal detection and surgical experience with high-risk, 2-ventricle patients, with complex congenital heart disease that underwent stage-1 hybrid palliation.
Methods
We retrospectively identified those born between March 2008 and March 2021 with 2-ventricle hearts, complex congenital cardiovascular malformations, and ductal-dependent systemic circulation that underwent stage-1 hybrid palliation consisting of surgical bilateral pulmonary artery banding and interventional catheterization placed ductus arteriosus stents.
Results
We identified 30 patients. Of the 30, 19 (63%) were male. For the 30, median gestational age was 35 weeks (29-39 weeks), and median birth weight was 2.2 kg (0.6-4.5 kg). Of the 30, 1 was transferred from an adjacent state, and 29 were born in Nevada. Of the 29 born in Nevada, overall statewide prenatal detection was 18 of 29 (62%); however, for 2008 to 2011 the prenatal detection rate was 3 of 10 (30%) and 15 of 19 (79%) for 2012 to 2021, P = .03. For the last 5 years, prenatal detection for Nevada-born patients was 8 of 8 (100%). Two full-term newborns, without a prenatal diagnosis, presented postnatally in extremis. For the 30 patients, there were 0 stage-1 hybrid palliation mortalities, 1 subsequent repair mortality, and 3 late nonsurgical deaths.
Conclusions
Stage-1 hybrid palliation may result in excellent surgical outcomes for high-risk, 2-ventricle patients. Additionally, high rates of population-wide prenatal detection are possible for high-risk congenital heart disease, allowing prenatal planning and possibly reducing postnatal extremis presentations.
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.
Sudden cardiac death (SCD) of an athlete is a rare but tragic event and sport
activity might play a trigger role in athletes with underlying structural or
electrical heart diseases. Preparticipation screenings (PPs) have been conceived for
the potential to prevent SCD in young athletes by early identification of cardiac
diseases. The European Society of Cardiology protocol for PPs includes history
collection, physical examination and baseline electrocardiogram, while further
examinations are reserved to individuals with abnormalities at first-line
evaluation. Nevertheless, transthoracic echocardiography has been hypothesized
to have a primary role in the PPs. This review aims to describe how to approach
an athlete-focused echocardiogram, highlighting what is crucial to focus on for the
different diseases (cardiomyopathies, valvulopathies, congenital heart disease,
myocarditis and pericarditis) and when is needed to pay attention to overlap diagnostic zone (“grey zone”) with the athlete's heart. Once properly tested,
focused echocardiography by sports medicine physicians may become standard
practice in larger screening practices, potentially available during first-line
evaluation.
Purpose:
To investigate the characteristics of foveal microvasculature in children with Marfan syndrome (MFS).
Methods:
Ninety eyes from 45 MFS patients and 76 eyes from 38 healthy individuals of age-matched, sex-matched, and axial length-matched were enrolled. Characteristics of the superficial capillary plexus including the vessel density, perfusion density, and foveal avascular zone were analyzed by optical coherence tomography angiography.
Results:
The vessel density and the circularity index of the foveal avascular zone were significantly decreased in the MFS group compared with the controls (P = 0.017 and P = 0.004 respectively). In MFS group, the central vessel density (P = 0.003) and perfusion density (P = 0.001) were negatively correlated with the best-corrected visual acuity. The foveal avascular zone area was correlated with the aortic diameters (P = 0.001) and the paratemporal perfusion density was correlated with the ejection fraction (P = 0.003). Moreover, the paratemporal perfusion density and the circularity index of foveal avascular zone were found to be correlated with the aortic Z-score (P < 0.001 and P = 0.003 respectively).
Conclusion:
Retinal microvascular decrease and its correlation with best-corrected visual acuity and cardiac functions were observed in the MFS group. The optical coherence tomography angiography may help to characterize the underlying pathophysiology features of MFS and enable early detection and prevention of vascular changes in MFS.
Left ventricular outflow tract and aortic valve anomalies in a biventricular circulation are a heterogeneous group of lesions with resultant obstruction below, at the level of, and above the aortic valve. Valvar aortic stenosis accounts for the majority of left ventricular outflow obstructive lesions with predominance of bicuspid aortic valve disease. Majority of aortic stenosis progresses over time; clinically significant aortic stenosis will need intervention in the neonatal period. Current management strategies are evolving with improved understanding of this heterogeneous condition.
A newborn with supracardiac total anomalous pulmonary venous connection vein presented the small left ventricle with z score of −7.5, retrograde blood supply in the transverse arch, and the dutcus-dependent systemic circulation. The patient underwent the repair of the anomalous pulmonary vein and bilateral pulmonary arterial banding soon after the birth and then transcatheter pulmonary arterial debanding at the age of 10 months because of an appropriate growth of the left ventricle.
Aortic Z-score (Z-score) is utilized in clinical trials to monitor the effect of medications on aortic dilation rate in Marfan (MFS) patients. Z-scores are reported in relation to body surface area and therefore are a function of height and weight. However, an information void exists regarding natural, non-pharmacological changes in Z-scores as children age. We had concerns that Z-score decrease attributed to “therapeutic” effects of investigational drugs for Marfan disease connective tissue diseases might simply reflect normal changes (“filling out” of body contour) as children age. This investigation studies natural changes with age in Z-score in normal and untreated MFS children, teasing out normal effects that might erroneously be attributed to drug benefit. (1) We first compared body mass index (BMI) and Z-scores (Boston Children's Hospital calculator) in 361 children with “normal” single echo exams in four age ranges (0 to 1, 5 to 7, 10 to 12, 15 to 18 years). Regression analysis revealed that aging itself decreases ascending Z-score, but not root Z-score, and that increase in BMI with aging underlies the decreased Z-scores. (2) Next, we examined Z-score findings in both “normal” and Marfan children (all pharmacologically untreated) as determined on sequential echo exams over time. Of 27 children without aortic disease with sequential echos, 19 (70%) showed a natural decrease in root Z-score and 24 (89%) showed a natural decrease in ascending Z- score, over time. Of 25 untreated MFS children with sequential echos, 12 (40%) showed a natural decrease in root Z-score and 10 (33%) showed a natural decrease in ascending Z-score. Thus, Z-score is over time affected by natural factors even in the absence of any aneurysmal pathology or medical intervention. Specifically, Z-score decreases spontaneously as a natural phenomenon as children age and with fill out their BMI. Untreated Marfan patients often showed a spontaneous decrease in Z-score. In clinical drug trials in aneurysm disease, decreasing Z-score has been interpreted as a sign of beneficial drug effect. These data put such conclusions into doubt.
Background:
The terms "hypoplastic left heart complex" (HLHC) or borderline left heart have been used to describe fetuses with ventricular discrepancy involving small left heart structures but no overt valvar stenosis. Little evidence exists regarding predictors of successful biventricular (BV) repair in patients with HLHC.
Methods:
Fetal echocardiograms performed between 2004-2017 with HLHC were included. Studies at 18-26 weeks and 27-37 weeks were analyzed. The primary outcome was a successful BV circulation (Group 1). Group 2 included patients with single ventricle palliation, death or transplant. Univariate analysis was performed on studies at 18-26 weeks and 27-37 weeks. Multivariate logistic regression was performed on studies between 27-37 weeks.
Results:
Of 51 included cases, 44 achieved a successful BV circulation (Group 1) and 7 did not (Group 2). On univariate analysis, right to left foramen ovale (FO) flow and a larger mitral valve (MV) annulus z score were associated with successful BV circulation. On multivariate analysis right to left FO flow and a higher MV z score were found in Group 1. Bidirectional or left to right FO flow, LV length (LVL) z score of < -2.4 and a MV z score of < -4.5 correctly predicted 80% of Group 2 outcomes. Late follow-up was available for 41 patients. There were two late deaths in Group 2. Thirteen patients in Group 1 required re-intervention, 12 developed mitral stenosis and 5 developed isolated subaortic stenosis.
Conclusions:
A successful BV circulation is common in fetuses with HLHC. The size of the MV annulus, direction of FO flow and LVL z score are important predictors of a successful BV circulation. Long term sequelae in those with a BV circulation may include mitral and subaortic stenosis This article is protected by copyright. All rights reserved.
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.
Background
There are several published echo-derived scores to help predict successful biventricular versus univentricular palliation in neonates with critical aortic stenosis. This study aims to determine whether any published scoring system accurately predicted outcomes in these neonates.
Methods
Single centre, retrospective cohort study including neonates who underwent aortic valve intervention (surgical valvotomy or balloon valvuloplasty) with the intention of biventricular circulation. Primary outcome was survival with biventricular circulation at hospital discharge. Data from their initial neonatal echocardiogram were used to compute the following scores – Rhodes, CHSS 1, Discriminant, CHSS 2, and 2 V.
Results
Between 01/1999 and 12/2017, 68 neonates underwent aortic valve intervention at a median age of 4 days (range 1–29 days); 35 surgical valvotomy and 33 balloon valvuloplasty. Survival with biventricular circulation was maintained in 60/68 patients at hospital discharge. Of the remaining eight patients, three were converted to univentricular palliation, four died, and one underwent heart transplant prior to discharge. None of the binary score predictions of biventricular versus univentricular (using that score’s proposed cut-offs) were significantly associated with the observed outcome in this cohort. A high percentage of those predicted to need univentricular palliation had successful biventricular repair: 89.4% by Rhodes, 79.3% by CHSS 1, 85.2% by Discriminant, and 66.7% by CHSS 2 score. The 2 V best predicted outcome and agreed with the local approach in most cases.
Conclusion
This study highlights the limitations of and need for alternative scoring systems/cut-offs for consistently accurate echocardiographic prediction of early outcome in neonates with critical aortic stenosis.
Purpose of Review
The diagnostic capabilities, palliative procedures, and critical care therapies for neonates with single ventricle heart disease have rapidly evolved. We aimed to review current trends for intensivists and cardiologists caring for this population.
Recent Findings
Enhanced diagnostics and 3-dimensional modeling can be instrumental in planning care pathways for these neonates. Additionally, the ability to palliate many of these patients with catheter-based therapies rather than traditional surgical approaches has allowed for reduced morbidity. There are significant improvements in the understanding of how critical care therapies affect survival and neurodevelopmental outcomes in this population.
Summary
Despite advancements in the understanding of longer-term outcomes in this population, much remains to be discovered about how initial palliation, critical care strategies, surgical techniques, and extracorporeal support can be optimized for the most favorable neurodevelopmental outcomes, survival, and quality of life. This will be the major focus of future research through larger collaboratives.
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.
Aortic stenosis in the neonate has been associated in the past with a high operative mortality. As a result, in the current era of percutaneous balloon dilatation, the optimal mode of therapy remains controversial. An approach of stabilization with cardiopulmonary bypass, followed by relief of left ventricular outflow tract obstruction, was used at three institutions, and the results are presented. During the period 1983 to 1989, 40 neonates with isolated aortic stenosis and patent ductus arteriosus or coarctation of the aorta, or both, underwent operative therapy. Ages ranged from 1 to 30 days, median of 12 days, including 17 patients in the first week of life. There were 30 boys and 10 girls; weights ranged from 2.5 to 5.5 kg with a mean of 3.6 kg. Perioperative conditions included congestive heart failure in 38 and mitral regurgitation in 16; left ventricular-aortic gradients ranged from 15 to 130 mm Hg, with a mean of 67 mm Hg. There were 30 open valvotomies and 10 transventricular dilatations. The hospital survival rate was 87.5% (35/40) with no significant difference between the methods of valvotomy (9/10 in the transventricular dilatation group, 90%; 26/30 in the open valvotomy group, 87%). Although multiple methods of perfusion and valvotomy were used, the single unifying factor of cardiopulmonary bypass stabilization was present in all 40 patients. No significant difference in survival was noted between institutions, methods of cardiopulmonary bypass, cardiopulmonary bypass times, crossclamp times, or method of valvotomy. There have been five reoperations, with one late death in a patient requiring mitral valve replacement and an apical-aortic conduit. One sudden death occurred; autopsy revealed endocardial fibroelastosis. Results demonstrate that in the three institutions using the methods described, a high operative and late survival rate is possible. The results of this technique, against which percutanous dilatation should be compared, are standard in the current era.
Background - Transcatheter balloon aortic valvuloplasty (BAVP) has become the first-line treatment for critical aortic stenosis (AS) in neonates. However, little is known about the growth and function of left heart structures or about patterns of reintervention on the left heart after neonatal BAVP. Methods and Results - Between 1985 and 2002, 113 patients underwent neonatal BAVP at ≤60 days of age. There were 16 early deaths (14%), with a significant decrease from 1985 to 1993 (22%) to 1994 to 2002 (4%), and 6 patients had successful early conversion to a univentricular circulation. In the short term, the mean relative gradient reduction was 54±26%, and significant aortic regurgitation (AR) developed in 15% of patients. The 91 early survivors with a biventricular circulation were followed up for 6.3±5.3 years, during which time there was a steady increase in the frequency of significant AR. Freedom from moderate or severe AR was 65% at 5 years. In almost all patients with a baseline aortic annulus z score less than -1, the annulus diameter increased to within the normal range within 1 to 2 years. Similarly, left ventricular (LV) end-diastolic dimension z scores, which ranged from -5 to 7.5 before BAVP, normalized within 1 to 2 years in nearly all patients with a predilation z score less than -1. Among early survivors with a biventricular circulation, reintervention-free survival on the LV outflow tract was 65% at 1 year and 48% at 5 years, with younger age, higher pre- and post-BAVP gradients, and a larger balloon-annulus diameter ratio associated with decreased reintervention-free survival (P<0.01). Seventeen surgical interventions were performed on the aortic valve in 15 patients, including replacement in 7. Survival free from aortic valve replacement was 84% at 5 years. Conclusions - BAVP for AS during the first 60 days of life results in short-term relief of AS in the majority of patients. Among early survivors, initially small left heart structures may be associated with worse subacute outcomes but typically normalize within 1 year. Reintervention for residual/recurrent AS or iatrogenic AR is relatively common, particularly during the first year after BAVP, but aortic valve replacement during early childhood is seldom necessary.
Objectives: 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. Methods: 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. Results: 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. Conclusions: Morphologic and functional factors can be used to predict optimal pathway and survival benefit in neonates with critical left ventricular outflow obstruction. (J Thorac Cardiovasc Surg 2001;121:10-27)
Congenital aortic stenosis presenting within the first 6 months of life is a highly lethal anomaly. Although aortic valvotomy has offered excellent palliation in many instances, the operative risk remains substantial. To better understand the factors associated with a poor operative result, the records of all patients less than 6 months of age undergoing aortic valvotomy at our institution from 1972 through 1986 were analyzed. Nineteen patients (58%) (Group I) survived operation; 14 (42%) (Group II) died. The following variables were analyzed in an attempt to define those with prognostic significance: mean pulmonary artery pressure (PAP), left ventricular (LV) peak systolic pressure, LV end-diastolic pressure, peak systolic aortic valve gradient, LV end-diastolic volume (LVEDV), LV ejection fraction, and age at operation. The only variables that were significantly different in the two groups were mean PA (Group I, 29 ± 3 mm Hg, and Group II, 54 ± 3 mm Hg; p < 0.001) and LVEDV (Group I, 50 ± 8 ml/m2, and Group II, 20 ± 4 ml/m2; p < 0.05). No patient with an LVEDV of 20 ml/m2 or less survived operation. We conclude that small LV dimension and elevation of PAP may be predictive of a poor surgical result in patients with severe aortic stenosis presenting in infancy.
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.
Estimates of body surface area were made based on measurement of 81 subjects, ranging from premature infants to adults. SA was calculated geometrically for each subject from 34 body measurements, and the values obtained compared with those based on previously published formulas and graphs. The most widely used formula, that of Du Bois and Du Bois, increasingly underestimated SA as values fell below 0.7 m2; the disparity was greatest in the newborn infant (7.96%). Closer agreement was obtained with the equations and nomograms of Body, Brody, Faber and Melcher, and Sendroy and Cecchini, although minor deviations were noted in some age ranges. The formula SA (m2) = weight (kg)0.5378 X height (cm)0.3964 X 0.024265, derived from the measured data by multiple regression analysis, gave a good fit for all values of SA from less than 0.2 m2 to greater than 2.0 m2 (r = 0.998). This formula was used to construct nomograms for estimation of SA in infants, children, and adults from height (length) and weight.
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.
The factors associated with survival in 40 neonates (age less than 28 days) with critical aortic stenosis undergoing either open (22 patients) or closed (18 patients) transventricular aortic valvotomy were reviewed. Significant adverse correlates with survival included evidence of poor perfusion preoperatively (low pH, greater than Grade 2/6 soft ejection systolic murmur) and marked congestive heart failure (hepatomegaly, cardiomegaly, elevated left atrial pressure). Congenital mitral stenosis (anulus less than 11 mm), a small aortic anulus (less than 6.5 mm), and failure to achieve an adequate aortic orifice (greater than 6 mm), at operation were identified as factors associated with increased mortality. Initial perioperative survival was better with closed aortic valvotomy. However, there was no significant difference in overall operative survival between closed (9/18, 50%) and open (8/22, 36%) aortic valvotomy (p = 0.26). The incidence of early reoperation (less than 1 year of age) was greater in perioperative survivors undergoing closed valvotomy (7/13, 54%) rather than open valvotomy (1/10, 10%) (p less than 0.05). In conclusion, long-term survival among patients with critical neonatal aortic valve stenosis remains disturbingly low (13/40, 32%) and has not significantly improved over the past 20 years.
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.
30 patients with severe aortic valve stenosis presented in severe congestive heart failure within the first 2 months of life. In 25 of them, left ventricular volume and contractility were assessed; five of them had a left ventricle of normal size, in 11 left ventricular size was diminished, and in nine patients it was enlarged. Eleven of the infants had extensive endocardial fibroelastosis (EFE) evidenced angiographically by myocardial sinusoids in ten of them and established at autopsy in six. The presence of EFE correlated with the size of the left ventricle; eight of 11 with a small left ventricle, two of five with a normal-sized left ventricle, and one of nine with an enlarged left ventricle displayed EFE. The severe depression of left ventricular function associated with EFE was documented by left ventricular volume determinations on exclusion of the myocardial sinusoids. Of 30 patients, 12 (including eight of 26 who underwent surgery) did not survive. Mortality, severity, and early onset of symptoms were associated mainly with small size of the left ventricle and with the severe left ventricular dysfunction associated with EFE.
The relation of left ventricular size, as estimated with echocardiography, to mortality was evaluated in three groups of infants with severe left ventricular outflow obstruction. Group I consisted of 17 patients with combined aortic and mitral stenosis or atresia associated with definite hypoplasia of the left ventricle. Group II consisted of eight patients with the primary diagnosis of severe aortic stenosis. Group III consisted of 12 patients with severe coarctation of the aorta. The left ventricular enddiastolic dimension measured with M mode echocardiography and the cross-sectional area of the left ventricular cavity as seen in the parasternal long axis view of the two dimensional echocardiogram were used as indexes of left ventricular volume. All patients with symptomatic outflow obstruction and a left ventricular end-diastolic dimension of less than 13 mm died in infancy. However, five patients with a hypoplastic left ventricle proved at angiography or at autopsy, or both, were found to have a ventricular end-diastolic dimension of 13 mm or greater. Two dimensional echocardiography showed that the left ventricle in these patients was foreshortened and spherical in shape. The cross-sectional area of the left ventricle of each patient in group I was less than 1.6 cm2. This was below the range of cross-sectional areas found in a group of normal infants (1.8 to 3.5 cm2 ± 2 standard deviations about the mean). Three patients in groups II and III had a slightly reduced left ventricular area (1.7 cm2) and none of these patients survived infancy. Measurement of the cross-sectional area of the left ventricle is a useful method of determining left ventricular size in infants suspected of having the hypoplastic left ventricle syndrome. Patients who have reduced left ventricular volume as assessed by this technique are at very great risk even if surgical relief of the outflow obstruction is attempted.
We evaluated our immediate and midterm (mean 4.3 years) results of balloon dilation of critical valvular aortic stenosis in 33 neonates.
Balloon dilation has been used as an alternative to surgical treatment. Reports to date consist of small series (largest 16 babies) with short-term follow-up (longest 4.8 years).
From 1985 to 1991, 33 neonates had dilation at a mean age of 13 days and a mean weight of 3.4 kg. Nineteen of the neonates (58%) were intubated and received prostaglandins, and 94% had other cardiac abnormalities.
The dilation was completed retrograde in 31 of the neonates (umbilical artery in 11 and femoral artery in 20) and anterograde (femoral vein) in 2. The average immediate peak gradient and left ventricular end-diastolic pressure reductions were 54% and 20%, respectively. The overall mortality rate was 12% (three early deaths and one late). All 20 neonates dilated through a femoral artery initially had pulse loss with restoration in 35% after thrombolytic therapy. At 8.3 years, survival and freedom of reintervention probability rates were 88% and 64%, respectively. At mean 4.3 years of follow-up, 83% of the survivors were asymptomatic; Doppler study revealed a maximal instantaneous gradient < 50 mm Hg in 65% of neonates and significant aortic regurgitation in 14%.
This study confirms that dilation of aortic stenosis in neonates is effective; reintervention (mostly redilation) is frequent (40%); and midterm survival is encouraging (88%).
The purpose of this study was to identify echocardiographic hemodynamic and morphometric factors that would predict which infants with critical aortic stenosis could undergo relief of left ventricular outflow obstruction as opposed to the Norwood procedure.
Echocardiographic predictors of survival in infants with critical aortic stenosis after two-ventricle repair have been mainly limited to morphometric factors, which have limitations. Echocardiographic hemodynamic predictors of survival in these patients have not previously been studied.
Doppler color flow mapping and pulsed Doppler techniques were used to obtain hemodynamic measurements of flow in the ascending, transverse and descending aorta, the ductus arteriosus, and across the aortic and mitral valves in infants with critical aortic stenosis. Morphometric measurements of the left heart structures were obtained, and comparisons were made between survivors and nonsurvivors for the hemodynamic and morphometric factors.
Twenty-eight infants (mean age 1 +/- .6 days, mean weight 3.6 +/- .6 kg) with critical aortic stenosis were evaluated. Nineteen had a two-ventricle repair initially attempted, and nine had a Norwood operation. Among the patients with a two-ventricle repair, the hemodynamic factors associated with survival after two-ventricle repair included predominant or total antegrade flow in the ascending (p < 0.01) and transverse aorta (p < 0.05). Aortic valve gradient, mitral valve inflow and direction of flow in the ductus arteriosus and descending aorta were unrelated to outcome. The morphometric factors associated with survival after two-ventricle repair included the indexed aortic annulus (p < 0.0002), aortic root (p < 0.003), ascending aorta (p < 0.008) and left ventricular long-axis length (p < 0.01). Left ventricular volume, mass, ejection fraction and mitral valve area were not related to outcome after two-ventricle repair.
In infants with critical aortic stenosis, predominant or total antegrade flow in the ascending and transverse aorta was associated with survival after two-ventricle repair. Determination of a one- versus two-ventricle repair remains a complex issue in infants with critical aortic stenosis. In addition to established morphometric predictors, hemodynamic information on the direction of flow in the aorta may help to define candidates for the Norwood operation.
Patients with left ventricular hypoplasia and left-sided heart obstructive lesions other than critical aortic stenosis may be inappropriately subjected to single ventricular repair because their assessment is based on faulty qualitative evaluations or on quantitative methods developed for critical aortic stenosis. Patients with left ventricular hypoplasia and left-sided heart obstructions other than critical aortic stenosis successfully underwent biventricular repair despite "failing" to pass established criteria for critical aortic stenosis.
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.
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.
Hypoplastic left heart syndrome (HLHS) is frequently diagnosed prenatally, but this has not been shown to improve surgical outcome.
We reviewed patients with HLHS between July 1992 and March 1999 to determine the influence of prenatal diagnosis on preoperative clinical status, outcomes of stage 1 surgery, and parental decisions regarding care. Of 88 patients, 33 were diagnosed prenatally and 55 after birth. Of 33 prenatally diagnosed patients, 22 were live-born, and pregnancy was terminated in 11. Of 22 prenatally diagnosed patients who were live-born, 14 underwent surgery, and parents elected to forego treatment in 8. Of 55 patients diagnosed postnatally, 38 underwent surgery, and 17 did not because of parental decisions or clinical considerations. Prenatally diagnosed patients were less likely to undergo surgery than patients diagnosed after birth (P:=0.008). Among live-born infants, there was a similar rate of nonintervention. Among patients who underwent surgery, survival was 75% (39/52). All patients who had a prenatal diagnosis and underwent surgery survived, whereas only 25 of 38 postnatally diagnosed patients survived (P:=0.009). Patients diagnosed prenatally had a lower incidence of preoperative acidosis (P:=0.02), tricuspid regurgitation (P:=0.001), and ventricular dysfunction (P:=0.004). They were also less likely to need preoperative inotropic medications or bicarbonate (P:=0.005). Preoperative factors correlating with early mortality included postnatal diagnosis (P:=0.009), more severe acidosis (P:=0.03), need for bicarbonate or inotropes (P:=0.008 and 0.04), and ventricular dysfunction (P:=0.05).
Prenatal diagnosis of HLHS was associated with improved preoperative clinical status and with improved survival after first-stage palliation in comparison with patients diagnosed after birth.
Hypoplastic left heart syndrome encompasses a spectrum of structural cardiac malformations characterized by severe underdevelopment of the structures in the left heart-aorta complex. The severe end of the spectrum consists of aortic atresia and mitral atresia, whereas at the mild end patients have aortic valve and mitral valve hypoplasia without intrinsic valve stenosis, recently described as hypoplastic left heart complex. These patients may be candidates for biventricular repair. Since November 1988, 12 of 13 patients (92%) with hypoplastic left heart complex have undergone two-ventricle repair and one patient the Norwood operation. Median age at surgery was 7 days (5 to 45 days) and median weight was 3.59 kg (2.6 to 4.3 kg). Biventricular repair consisted of pulmonary homograft patch aortoplasty of the aortic arch and ascending aorta and closure of the interatrial and interventricular communications. The left heart was able to support the systemic circulation in 11 of 12 patients undergoing biventricular repair (92%). The early mortality was two patients (15.4%). The mean follow-up was 67 months (range, 11 months to 133 months). There was one late death at 39 months. Reoperations were necessary in four patients for left ventricular outflow tract obstruction and in two patients for recurrent coarctation. We have successfully achieved biventricular repair in most patients with hypoplastic left heart complex, a subset of patients with hypoplastic left heart syndrome. Reoperation, particularly for left ventricular outflow tract obstruction, appears likely. Finally, with increased experience, predictive criteria for the feasibility and optimal outcome of biventricular repair will be more accurately defined.
Background:
In general, neonates with severe left ventricular outflow tract obstruction, aortic valvar stenosis or atresia, and arch hypoplasia with either interruption or coarctation, and a small left ventricle undergo Norwood palliation followed classically by a bidirectional cavopulmonary shunt and eventual modified Fontan. However, a subset of patients, usually neonates with a ventricular septal defect, may have adequate left ventricle and mitral valve sizes making them candidates for future biventricular repair (BVR). In view of the long-term advantage of BVR, the feasibility and outcome of this approach was studied. Additionally, echocardiographic data were reviewed in an attempt to develop objective prognostic criteria for selection of patients suitable for BVR.
Methods:
During a 4-year period, 8 of 58 infants undergoing Norwood palliation were identified as potential two-ventricle candidates. Their mean age was 6 days. Diagnoses included aortic atresia (n = 1), or aortic valve stenosis and subaortic stenosis (n = 7), with an interrupted aortic arch in 3 and coarctation in 4. All patients had a ventricular septal defect and a left ventricle that was considered to be apex forming. Mean mitral valve size was 11 mm (z-score = -1.7). Mean aortic valve size was 4.1 mm (mean z-score = -8.4).
Results:
All 8 patients survived Norwood palliation. Six subsequently underwent BVR with ventricular septal defect closure and a right ventricle to pulmonary artery conduit at a mean age of 7 months. One patient is awaiting repair, and 1 underwent a cavopulmonary shunt. At the time of BVR, mean mitral valve z-score was essentially unchanged at -1.4 (14 mm). No early deaths or late deaths occurred during a mean follow-up of 32 months.
Conclusions:
A small subset of patients requiring Norwood palliation as newborns may be candidates for eventual BVR with low risk. In general, patients suitable for BVR have a mitral valve z-score of more than -3 and a normal-sized left ventricle. Recognition of neonatal BVR candidates enables consideration of complete neonatal repair. However, single-stage repair needs to be compared with the excellent results obtainable with the staged approach.
Preventing the progression of fetal aortic stenosis (AS) to hypoplastic left heart syndrome (HLHS) requires identification of fetuses with salvageable left hearts who would progress to HLHS if left untreated, a successful in utero valvotomy, and demonstration that a successful valvotomy promotes left heart growth in utero. Fetuses meeting the first criterion are undefined, and previous reports of fetal AS dilation have not evaluated the impact of intervention on in utero growth of left heart structures.
We offered fetal AS dilation to 24 mothers whose fetuses had AS. At least 3 echocardiographers assigned a high probability that all 24 fetuses would progress to HLHS if left untreated. Twenty (21 to 29 weeks' gestation) underwent attempted AS dilation, with technical success in 14. Ideal fetal positioning for cannula puncture site and course of the needle (with or without laparotomy) proved to be necessary for procedural success. Serial fetal echocardiograms after intervention demonstrated growth arrest of the left heart structures in unsuccessful cases and in those who declined the procedure, while ongoing left heart growth was seen in successful cases. Resumed left heart growth led to a 2-ventricle circulation at birth in 3 babies.
Fetal echocardiography can identify midgestation fetuses with AS who are at high risk for developing HLHS. Timely and successful aortic valve dilation requires ideal fetal and cannula positioning, prevents left heart growth arrest, and may result in normal ventricular anatomy and function at birth.
Basic fluid dynamic principles were used to derive a theoretical model of optimum cardiovascular allometry, the relationship between somatic and cardiovascular growth. The validity of the predicted models was then tested against the size of 22 cardiovascular structures measured echocardiographically in 496 normal children aged 1 day to 20 yr, including valves, pulmonary arteries, aorta and aortic branches, pulmonary veins, and left ventricular volume. Body surface area (BSA) was found to be a more important determinant of the size of each of the cardiovascular structures than age, height, or weight alone. The observed vascular and valvar dimensions were in agreement with values predicted from the theoretical models. Vascular and valve diameters related linearly to the square root of BSA, whereas valve and vascular areas related to BSA. The relationship between left ventricular volume and body size fit a complex model predicted by the nonlinear decrease of heart rate with growth. Overall, the relationship between cardiac output and body size is the fundamental driving factor in cardiovascular allometry.
Transcatheter balloon aortic valvuloplasty (BAVP) has become the first-line treatment for critical aortic stenosis (AS) in neonates. However, little is known about the growth and function of left heart structures or about patterns of reintervention on the left heart after neonatal BAVP.
Between 1985 and 2002, 113 patients underwent neonatal BAVP at < or =60 days of age. There were 16 early deaths (14%), with a significant decrease from 1985 to 1993 (22%) to 1994 to 2002 (4%), and 6 patients had successful early conversion to a univentricular circulation. In the short term, the mean relative gradient reduction was 54+/-26%, and significant aortic regurgitation (AR) developed in 15% of patients. The 91 early survivors with a biventricular circulation were followed up for 6.3+/-5.3 years, during which time there was a steady increase in the frequency of significant AR. Freedom from moderate or severe AR was 65% at 5 years. In almost all patients with a baseline aortic annulus z score less than -1, the annulus diameter increased to within the normal range within 1 to 2 years. Similarly, left ventricular (LV) end-diastolic dimension z scores, which ranged from -5 to 7.5 before BAVP, normalized within 1 to 2 years in nearly all patients with a predilation z score less than -1. Among early survivors with a biventricular circulation, reintervention-free survival on the LV outflow tract was 65% at 1 year and 48% at 5 years, with younger age, higher pre- and post-BAVP gradients, and a larger balloon-annulus diameter ratio associated with decreased reintervention-free survival (P<0.01). Seventeen surgical interventions were performed on the aortic valve in 15 patients, including replacement in 7. Survival free from aortic valve replacement was 84% at 5 years.
BAVP for AS during the first 60 days of life results in short-term relief of AS in the majority of patients. Among early survivors, initially small left heart structures may be associated with worse subacute outcomes but typically normalize within 1 year. Reintervention for residual/recurrent AS or iatrogenic AR is relatively common, particularly during the first year after BAVP, but aortic valve replacement during early childhood is seldom necessary.
Neonatal aortic stenosis is a complex and heterogeneous condition, defined as left ventricular outflow tract obstruction at valvular level, presenting and often requiring treatment in the first month of life. Initial presentation may be catastrophic, necessitating hemodynamic, respiratory and metabolic resuscitation. Subsequent management is focused on maintaining systemic blood flow, either via a univentricular Norwood palliation or a biventricular route, in which the effective aortic valve area is increased by balloon dilation or surgical valvotomy. In infants with aortic annular hypoplasia but adequately sized left ventricle, the Ross-Konno procedure is also an attractive option. Outcomes after biventricular management have improved in recent years as a consequence of better patient selection, perioperative management and advances in catheter technology. Exciting new developments are likely to significantly modify the natural history of this disorder, including fetal intervention for the salvage of the hypoplastic left ventricle; 3D echocardiography providing better definition of valve morphology and aiding patient selection for a surgical or catheter-based intervention; and new transcutaneous approaches, such as duel beam echo, to perforate the valve.
Critical aortic stenosis in the neonate
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