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Background Z scores are the method of choice to report dimensions in pediatric echocardiography. Z scores based on body surface area (BSA) have been shown to cause systematic biases in overweight and obese children. Using aortic valve (AoV) diameters as a paradigm, the aims of this study were to assess the magnitude of z score underestimation in children with increased body mass index z score (BMI-z) and to determine if a predicting model with height and weight as independent predictors would minimise this bias. Methods In this multicentre, retrospective, cross-sectional study, 15,006 normal echocardiograms in healthy children 1-18 years old were analyzed. Residual associations with body size were assessed for previously published z score. BSA-based and alternate prediction models based on height and weight were developed and validated in separate training and validation samples. Results Existing BSA-based z scores incompletely adjusted for weight, BSA, and BMI-z and led to an underestimation of > 0.8 z score units in subjects with higher BMI-z compared with lean subjects. BSA-based models led to overestimation of predicted AoV diameters with increasing weight or BMI-z. Models using height and weight as independent predictors improved adjustment with body size, including in children with higher BMI-z. Conclusions BSA-based models result in underestimation of z scores in patients with high BMI-z. Prediction models using height and weight as independent predictors minimise residual associations with body size and generate well fitted predicted values that could apply to all children, including those with low or high BMI-z.

Background The reported survival of tetralogy of Fallot (TOF) is > 97%. Patients with pulmonary atresia and/or genetic conditions have worse outcomes, but long-term estimates of survival and morbidity for these TOF subgroups are scarce. The objective of this study was to describe the 30-year outcomes of TOF according to native anatomy and the coexistence of genetic conditions. Methods The TRIVIA (Tetralogy of Fallot Research for Improvement of Valve Replacement Intervention: A Bridge Across the Knowledge Gap) study is a retrospective population-based cohort including all TOF subjects born from 1980 to 2015 in Quebec. We evaluated all-cause mortality by means of Cox proportional hazards regression, and cumulative mean number of cardiovascular interventions and unplanned hospitalisations with the use of marginal means/rates models. We computed 30-year estimates of outcomes according to TOF types, ie, classic TOF (cTOF) and TOF with pulmonary atresia (TOF-PA), and the presence of genetic conditions. Results We included 960 subjects. The median follow-up was 17 years (interquartile range, 8-27). Nonsyndromic cTOF subjects had a 30-year survival of 95% and had undergone a mean of 2.8 interventions and 0.5 hospitalisations per subject. In comparison, TOF-PA subjects had a lower 30-year survival of 78% and underwent a mean of 8.1 interventions, with 4 times as many hospitalisations. The presence of a genetic condition was associated with lower survival ( Conclusions The anatomic types and the presence of genetic conditions strongly influence the long-term outcomes of TOF. We provided robust 30-year estimates for key markers of prognosis that may be used to improve risk stratification and provide more informed counselling to families.

Background Aortic valve replacement in children represents an important challenge. Concerns regarding pulmonary autograft and homograft longevity requiring reoperations are well recognized. Very long-term outcomes after the Ross procedure are still unknown. We reviewed our experience with the Ross procedure, aiming to define very long-term survival rate and freedom from reintervention. Methods This was a single-center retrospective cohort including 63 consecutive children who underwent the Ross procedure. Median follow-up duration was 20.5 years. Time-related events were assessed using Kaplan-Meier estimator. Results There were 51 (81%) boys, mean age 10.1 ± 5.8 years. Isolated aortic stenosis was the most common diagnosis (n = 29, 46%) and 34 (54%) patients previously underwent cardiac surgery. There was 1 (1.6%) in-hospital death. Overall survival at 5, 15, and 25 years was 96.7%, 94.4%, and 94.4%, respectively. Freedom from any autograft-related reintervention was 98.1%, 86.4%, and 61.2% at 5, 15, and 25 years, respectively. Fifteen (24%) patients underwent autograft reoperations. Among them, 10 (67%) patients underwent valve-sparing autograft reoperation. Freedom from any pulmonary conduit reintervention was 93.2%, 58.2%, and 28.3% at 5, 15, and 25 years, respectively. Thirty (46.6%) patients underwent conduit reintervention (8 percutaneous, 22 surgical replacements). Conclusions The pediatric Ross procedure is associated with excellent long-term survival. Ross-related reinterventions are more than twice as common on the pulmonary homograft than on the autograft.

Although cardiovascular magnetic resonance (CMR) imaging measurements are known to differ between sexes and according to age in the normal heart, little is known about measurement variability in patients with congenital heart disease (CHD). Yet, aggregate CMR data, without adjustment for sex or age

Background: Guidelines for surgical management of tetralogy of Fallot (TOF) are often based on low-quality evidence due to the many challenges of congenital heart disease: heterogeneous cardiac anatomy, consequences from surgical interventions arising years later, and scarcity of hard outcomes. The overarching goal of the Tetralogy of Fallot Research for Improvement of Valve replacement Intervention: A Bridge Across the Knowledge Gap (TRIVIA) study is to evaluate the long-term impact of the surgical management strategies in TOF. The specific objectives are: (1) to describe the long-term outcomes of TOF according to the native anatomy and the presence of genetic conditions, (2) to evaluate the long-term outcomes of surgical repair according to associated residual lesions, and (3) to evaluate the impact of paediatric pulmonary valve replacements on the long-term outcomes. Methods: The TRIVIA study is a population-based cohort including all subjects with TOF in the province of Québec between 1980 and 2017. It links patient-level granular clinical data with long-term administrative health care data. We will evaluate mortality, cardiovascular interventions, and hospitalizations for adverse cardiovascular events using survival Cox models and marginal mean/rates models for recurrent events, respectively. Multivariate multilevel models will correct for potential confounders, and risk score matching will mitigate the potential of confounding by indication. Results: The current TRIVIA cohort includes 1001 eligible subjects with TOF with complete lifelong follow-up for > 98%. The median follow-up is 17.1 years, totalling > 17,000 patient-years. Conclusions: Universal health insurance data combined with granular clinical data enable the development of population-based cohorts, to which contemporary statistical methods are applied to address important research questions in congenital heart disease research. Résumé: Contexte: Les lignes directrices pour la prise en charge chirurgicale de la tétralogie de Fallot (TdF) sont souvent basées sur des données de faible qualité en raison des nombreux défis posés par les maladies cardiaques congénitales : anatomie cardiaque hétérogène, répercussions des interventions chirurgicales survenant des années plus tard et rareté de conclusions solides. L'objectif principal de l’étude TRIVIA (Tetralogy of Fallot Research for Improvement of Valve replacement Intervention: A Bridge Across the Knowledge Gap) est d'évaluer l'impact à long terme des stratégies de prise en charge chirurgicale dans les TdF. Les objectifs spécifiques sont les suivants : (1) décrire les issues à long terme de la TdF en fonction de l'anatomie native et de la présence de conditions génétiques, (2) évaluer les conséquences à long terme de la chirurgie réparatrice en fonction des lésions résiduelles associées, et (3) évaluer l'impact des remplacements valvulaires pulmonaires pédiatriques sur le pronostic à long terme. Méthodes: L'étude TRIVIA est une étude de cohorte basée sur une population comprenant tous les sujets atteints de TdF dans la province du Québec entre 1980 et 2017. Elle fait le lien entre les données cliniques détaillées des patients et les données administratives des soins de santé à long terme. Nous évaluerons la mortalité, les interventions cardiovasculaires et les hospitalisations pour troubles cardiovasculaires en utilisant respectivement des analyses de survie par les modèles de Cox et des modèles de moyennes/taux marginaux pour les événements récurrents. Des modèles multivariés à plusieurs niveaux permettront de corriger les facteurs de confusion potentiels, et l'appariement par des scores de risque atténuera le potentiel de confusion par indication. Résultats: La cohorte de l’étude TRIVIA comprend actuellement 1001 sujets admissibles atteints de TdF avec un suivi complet tout au long de la vie pour > 98 %. Le suivi médian est de 17,1 ans, pour un total de > 17 000 patients-années. Conclusions: Les données de l'assurance maladie universelle combinées à des données cliniques détaillées permettent la mise en place de cohortes populationnelles, auxquelles les méthodes statistiques contemporaines pourront être appliquées afin de répondre aux questions importantes pour la recherche portant sur les maladies cardiaques congénitales.

Bicuspid aortic valve (BAV) is the most common congenital heart disease. Since heritability is suspected, actual guidelines recommend to perform an echocardiographic assessment for first-degree relatives (FDR) of patient with BAV. This study aimed to assess the effectiveness and the feasibility of the current guidelines for the screening of FDR of patient with BAV in a pediatric cardiology daily practice. Consecutive patients with BAV and their FDR were prospectively included from January 2015 to March 2018 at Centre Hospitalier Universitaire de Laval, Quebec City (Canada). Data were retrospectively collected and analyzed. A total of 713 FDR of 213 consecutives index cases [median age: 11 (6–20) years] were studied. Up to 32 (6.6%) FDR had a BAV and 26 (5.4%) had an aortic valve dysfunction. A total of 14 (2.9%) FDR had an ascending aorta dilatation according to Z-score including 6 (1.2%) patients with an ascending aorta ≥ 45 mm. No statistically significant differences regarding BAV, aortic valve dysfunction and ascending aorta dilatation prevalence were identified between generations. Screening was done in 482 (67.6%), prescribed but not done in 134 (19%), not prescribed in 92 (13%) and declined in 5 (1%) FDR. The prevalence of BAV in FDR was similar to prospective adult studies and supports actual guidelines in pediatric cardiology practice. Ascending aorta dilatation was rare in our young population. Exhaustiveness and additional burden to implement current guidelines remain a challenge in daily practice.

Fibrin sheath formation around long-term indwelling central venous catheters is common and usually benign. Fibrin sheath can persist after catheter removal and rarely leads to complications. This is a report of three pediatric oncology patients that required cardiac surgery for cardiac embolization of a "ghost" catheter several years after catheter removal. One case required tricuspid valve replacement for complete tricuspid valve destruction and two had erosion through the atrial wall. The severity of these rare complications mandates follow-up of "ghost" catheters in pediatric oncology patients.

Reference values for cardiac magnetic resonance imaging (cMRI) in children and young adults are scarce. This leads to risk stratification of patients with congenital heart diseases being based on volumes indexed to body surface area (BSA). We aimed to produce cMRI Z score equations for ventricular volumes in children and young adults and to test whether indexing to BSA resulted in an incorrect assessment of ventricular dilation according to sex, body composition, and growth.We retrospectively included 372 subjects aged26 years with either normal hearts or conditions with no impact on ventricular volumes (reference group), and 205 subjects with repaired tetralogy of Fallot (TOF) aged26 years. We generated Z score equations by means of multivariable regression modelling. Right ventricular dilation was assessed with the use of Z scores and compared with indexing to BSA in TOF subjects.Ventricular volume Z scores were independent from age, sex, and anthropometric measurements, although volumes indexed to BSA showed significant residual association with sex and body size. In TOF subjects, indexing overestimated dilation in growing children and underestimated dilation in female compared with male subjects, and in overweight compared with lean subjects.Indexed ventricular volumes measured with cMRI did not completely adjust for body size and resulted in a differential error in the assessment of ventricular dilation according to sex and body size. Our proposed Z score equations solved this problem. Future studies should evaluate if ventricular volumes expressed as Z scores have a better prognostic value than volumes indexed to BSA.

Background Heart failure in adult with congenital heart disease is an important cause of morbi-mortality. Sacubitril/Valsartan has been validated for the treatment of heart failure in adults with left ventricular dysfunction. Purpose The aim of this study was to assess safety and efficacy of Sacubitril/Valsartan in symptomatic adults with systemic right ventricle dysfunction. Methods This is a retrospective and single center study (Quebec Heart and Lung Institute, Quebec City, Canada). Results Eight patients (mean age 41 ± 9.2 years, 5 males) with a significant systemic right ventricle dysfunction in New-York functional class II-III despite optimal heart failure medication were included. Sacubitril/Valsartan was up titrated at the maximal dosage tolerated. The mean dosage reached was 85 ± 22 mg/90 ± 24 mg. During a median follow-up of 248 (213 - 430) days, systolic (121 ± 13 vs. 116 ± 13 mmHg, P = 0.238) and diastolic (76 ± 6 mmHg vs. 72 ± 5 mmHg, P = 0.105) blood pressure, potassium (4.1 ± 0.4 mmol/l vs. 3.9 ± 0.2 mmol/l, P = 0.111) and creatinine (71.7 ± 14.5 μmol/l vs. 68.9 ± 14.7 μmol/l, P = 0.516) levels remained stable. No discontinuation, reduction in dose or side effects were noted. A significant improvement in functional status was observed (P = 0.03): 6 patients had a New-York Heart Association class improvement and 2 remained in the same class ( Fig. 1 ). Maximal oxygen uptake, NTproBNP levels and echocardiographic data did not reach significant improvement. Conclusion Sacubitril/Valsartantitration in a population of patients with congenital heart disease and significant right ventricle dysfunction was well tolerated and associated with significant improvement in functional status class. Whether Sacubitril/Valsartan improved right ventricle remodeling or decrease heart failure hospitalization or mortality in that population remained to determined.

This cohort study compares the long-term outcomes associated with valve-sparing procedures vs transannular patches for correction of tetralogy of Fallot., Key Points Question Do valve-sparing (VS) operations have better long-term outcomes than transannular patches (TAP) for correction of tetralogy of Fallot (TOF) when stratifying for residual lesions after surgical treatment? Findings In this cohort study of 528 patients with repaired TOF in the province of Quebec, Canada, from 1980 to 2015, individuals who underwent a VS procedure had statistically significantly increased 30-year survival compared with individuals who underwent TAP. Moreover, individuals who underwent a VS procedure had statistically significantly fewer reinterventions, even in the presence of moderate residual pulmonary stenosis. Meaning These findings suggest that it is beneficial to perform a VS procedure for correction of TOF, even in the presence of moderate residual stenosis, compared with TAP repair., Importance The choice of the right surgical technique for correction of tetralogy of Fallot (TOF) is contentious for patients with a moderate to severe right outflow tract obstruction. The use of a transannular patch (TAP) exposes patients to chronic pulmonary regurgitation, while valve-sparing (VS) procedures may incompletely relieve pulmonary obstruction. Objective To compare 30-year outcomes of TOF repair after a VS procedure vs TAP. Design, Setting, and Participants This retrospective population-based cohort study was conducted among all patients with TOF born in the province of Quebec, Canada, from 1980 to 2015 who underwent complete surgical repair. Patients who received a TAP or VS procedure were matched using a propensity score based on preoperative factors in a 1:1 ratio. Data were analyzed from March 2020 through April 2021. Exposures The study groups were individuals who received TAP and those who received VS. The VS group was further stratified by the presence of residual pulmonary stenosis. Main Outcomes and Measures The primary outcome was all-cause mortality, with 30-year survival evaluated using Cox proportional-hazards models. Secondary outcomes included the cumulative mean number of cardiovascular interventions, pulmonary valve replacements (PVRs), and cardiovascular hospitalizations were evaluated using marginal means/rates regressions. Results Among 683 patients with TOF (401 patients who underwent TAP [58.7%] and 282 patients who underwent a VS procedure [41.3%]), adequate propensity score matching was achieved for 528 patients (264 patients who underwent a VS procedure and 264 patients who underwent TAP). Among this study cohort, 307 individuals (58.1%) were men. The median (interquartile range [IQR]) follow-up was 16.0 (8.1-25.4) years, for a total of 8881 patient-years, including 63 individuals (11.9%) followed up for more than 30 years. Individuals who received a VS procedure had an increased 30-year survival of 99.1% compared with 90.4% for individuals who received TAP (hazard ratio [HR], 0.09 [95% CI, 0.02-0.41]; P = .002). Patients who underwent TAP had an increased 30-year cumulative mean number of cardiovascular interventions compared with patients who underwent a VS procedure without residual pulmonary stenosis (2.0 interventions [95% CI, 1.5-2.7 interventions] vs 0.7 interventions [95% CI, 0.5-1.1 interventions]; mean ratio [MR], 0.36 [95% CI, 0.25-0.50]; P

In recent years, there has been a shift in the type of repair for tetralogy of Fallot (ToF) with a preference for valve-sparing (VS) surgery over transannular patching (TAP). However, long-term outcomes evaluation is lacking. The objective was to compare long-term outcomes of patients with ToF

Cardiac hemangioma is rare, even more when leading to a cardiovascular collapse in a seemingly healthy newborn. A 6-day-old neonate had a tamponade caused by a basolateral hemangioma of the left ventricle. Partial surgical resection was performed. A congenital lobular capillary hemangioma was diagnosed upon histologic examination. The patient recovered completely and shows normal development at the 12-month follow-up.

Introduction: In addition to understanding causes of morbidity and mortality following tetralogy of Fallot repair (rTOF), the importance of patient-reported outcomes (PROs) such as quality of life (QOL) is increasingly being recognized. Hypothesis: We hypothesized that PROs in rTOF would be associated with selected sociodemographic factors, functional status, and/or clinical variables. Methods: As part of a prospective study of patients ≥12 years with rTOF and significant pulmonary regurgitation, participants completed PRO surveys: SF-12 Health Status (physical component summary [PCS] and mental component summary [MCS] t-scores), EQ-5D (5 dimensions: mobility, self-care, usual activities, pain/discomfort, and anxiety/depression), and a 0-100 QOL linear analogue scale (QOL-LAS). With multivariable regression analysis, we determined the association between PROs and selected predictors. Results: We studied n=627 participants (55% male, 26±13 years, 75% adult) at 14 sites (North America, Europe and Asia). Median age at repair was 1.6 years (IQR 0.6, 4.2). In general, scores on the SF-12 and QOL-LAS suggested good outcomes: SF-12 PCS Conclusions: Age and NYHA functional class at enrollment were found to be predictors of PROs in rTOF. While global measures of health status and QOL (SF-12 and QOL-LAS) suggest relatively good PROs, this study demonstrates the importance of inquiring about specific health problems (EQ-5D) to achieve a richer understanding of PROs in order to potentially maximize patient well-being.

Introduction: Late complications after tetralogy of Fallot repair (rTOF) often arise from chronic pulmonary regurgitation (PR). Prospective studies of outcomes in those with rTOF and significant PR are lacking. Hypothesis: We hypothesized that cardiovascular magnetic resonance imaging (CMR) imaging measurements would be associated with poor outcomes in an “at risk” population with rTOF and ≥moderate PR. Methods: This was a prospective, international study (North America, Europe and Asia) of children and adults. Inclusion criteria were rTOF in childhood, age ≥12 years, ≥moderate PR, and CMR within 18 months of enrollment. All CMR analysis occurred in a central core laboratory. Patient reported outcomes were collected using standardized tools. The primary clinical outcome was death or sustained (>30 seconds) ventricular tachycardia (VT) recorded at 3 year follow-up. Results: We enrolled 629 patients (55% male) at 29±14 years (2013-2017). Primary rTOF was done at 1.6 years (IQR 0.6,4.2) with a transannular patch in 55%. By standardized symptom assessment, 78% were asymptomatic (NYHA 1). Baseline CMR findings included PR fraction 38±14%, right ventricular (RV) end-diastolic volume indexed (EDVi) 155±41mL/m2, RV ejection fraction (EF) 44±7%, RV mass indexed 59±16 g/m2, left ventricular (LV) EDVi 85±18mL/m2, LVEF 55±7% and LV mass indexed 88±27 g/m2. Outcomes at 3 years were recorded (available in n=101). During follow-up, surgical pulmonary valve replacement (PVR) occurred in 38 patients (38%). In the PVR group compared with the non-PVR group, baseline RVEDVi was larger (189 vs. 152 mL/m2, p Conclusions: Despite significant PR, most patients with rTOF were asymptomatic. At 3 year follow-up, PVR occurred in more than one third of patients. Death or VT was observed in a small number of patients who had higher RV and LV mass on baseline CMR.

Background Chronic hemodynamically relevant pulmonary regurgitation (PR) resulting in important right ventricular dilation and ventricular dysfunction is commonly seen after tetralogy of Fallot (TOF) repair. Late adverse clinical outcomes, including exercise intolerance, arrhythmias, heart failure and/or death accelerate in the third decade of life and are cause for considerable concern. Timing of pulmonary valve replacement (PVR) to address chronic PR is controversial, particularly in asymptomatic individuals, and effect of PVR on clinical measures has not been determined. Methods Canadian Outcomes Registry Late After Tetralogy of Fallot Repair (CORRELATE) is a prospective, multicentre, Canada-wide cohort study. Candidates will be included if they are ≥ 12 years of age, have had surgically repaired TOF resulting in moderate or severe PR, and are able to undergo cardiovascular magnetic resonance imaging. Enrollment of > 1000 individuals from 15 participating centres (Toronto, Montreal, Quebec City, Sherbrooke, Halifax, Calgary, Edmonton, and Vancouver) is anticipated. Clinical data, health-related quality of life metrics, and adverse outcomes will be entered into a web-based database. A central core lab will analyze all cardiovascular magnetic resonance studies (PR severity, right ventricular volumes, and ventricular function). Major adverse outcomes (sustained ventricular tachycardia and cardiovascular cause of death) will be centrally adjudicated. Results To the best of our knowledge, CORRELATE will be the first prospective pan-Canadian cohort study of congenital heart disease in children and adults. Conclusions CORRELATE will uniquely link clinical, imaging, and functional data in those with repaired TOF and important PR, thereby enabling critical evaluation of clinically relevant outcomes in those managed conservatively compared with those referred for PVR.

Several articles have proposed echocardiographic reference values in normal pediatric subjects, but adequate validation is often lacking and has not been reviewed. The aim of this study was to review published reference values in pediatric two-dimensional and M-mode echocardiography with a specific focus on the adequacy of the statistical and mathematical methods used to normalize echocardiographic measurements. All articles proposing reference values for transthoracic pediatric echocardiography were reviewed. The types of measurements, the methods of normalization, the regression models used, and the methods used to detect potential bias in proposed reference values were abstracted. The detection of residual associations, residual heteroscedasticity, and departures from the normal distribution theory predictions were specifically analyzed. Fifty-two studies met the inclusion criteria. Most authors (87%) used parametric normalization to account for body size, but their approaches were very heterogeneous. Linear regression and indexing were the most common models. Heteroscedasticity was often present but was mentioned in only 27% of studies. The absence of residual heteroscedasticity and residual associations between the normalized measurements and the independent variables were mentioned in only 9% and 22% of the studies, respectively. Only 14% of studies documented that the distribution of the residual values was appropriate for Z score calculation or that the proportion of subjects falling outside the reference range was appropriate. Statistical suitability of the proposed reference ranges was often incompletely documented. This review underlines the great need for better standardization in echocardiographic measurement normalization.

Background The left pulmonary artery (LPA) contributes more than the right (RPA) to total pulmonary regurgitation (PR) in patients after tetralogy of Fallot (TOF) repair, but the mechanism of this difference is not well understood. This study aimed to analyze the interplay between heart and lung size, mediastinal geometry, and differential PR. Methods Forty-eight Cardiovascular Magnetic Resonance (CMR) studies in patients after TOF repair were analyzed. In addition to the routine blood flow and ventricular volume quantification cardiac angle between the thoracic anterior-posterior line and the interventricular septum, right and left lung areas as well as right and left hemithorax areas were measured on an axial image. Statistical analysis was performed to compare flow parameters between RPA and LPA and to assess correlation among right ventricular volume, pulmonary blood flow parameters and lung area. Results There was no difference between LPA and RPA diameters. The LPA showed significantly less total forward flow (2.49 ± 0.87 L/min/m2 vs 2.86 ± 0.89 L/min/m2; p = 0.02), smaller net forward flow (1.40 ± 0.51 vs 1.89 ± 0.60 mL/min/m2; p =

BACKGROUND Les anneaux vasculaires complets (AV) sont des anomalies rares des gros vaisseaux. Ils sont souvent symptomatiques en bas âge et nécessitent une chirurgie dans la grande majorité des cas. Les deux formes les plus communes sont les double arc aortique (DAA) et arc aortique droit avec ligament artériel et artère sous-clavière gauches aberrante (AADSCGA). L’approche chirurgicale et la prise en charge à long terme demeurent débattues dans la littérature, en plus, certains patients demeurent symptomatiques après la chirurgie. OBJECTIVES Évaluation à long terme des patients opérés pour un AV. DESIGN/METHODS Étude rétrospective observationnelle des patients diagnostiqués avec un AV. RESULTS Soixante-six patients ont été diagnostiqués avec un AV (16 patients avec DAA et 50 avec AADSCGA +/- diverticule de Kommerell (DK)). Au diagnostic, 61% étaient symptomatiques (39% symptômes respiratoires, 27% symptômes digestifs hauts, 6% les deux), alors que 20% ont été diagnostiqués dans le cadre d’une anomalie cardiaque associée; chez les autres, il s’agissait d’une découverte fortuite. Trente-huit pourcent des patients avaient une anomalie cardiaque associée. Vingt-trois pourcent des patients avaient un syndrome génétique, dont 53% étaient des syndromes de DiGeorge. Douze patients ont eu un diagnostic concomitant de trachéomalacie. Les patients avec DAA ont été diagnostiqués plus tôt (1.8 ± 3.6 vs 5 ± 8.3 ans, p=0.038) et opérés plus précocement après le diagnostic (82 ± 123 vs 264 ± 416 jours, p=0.036) que les patients avec AADSCGA. Une chirurgie a été effectuée chez 67% des cas, avec 34 patients (77%) subissant une division avec aortopexie. Aucun patient n’est décédé des suites de sa chirurgie. Deux patients ont dû avoir une seconde chirurgie pour des symptômes secondaires à l’AV. Au dernier suivi, 16 patients demeuraient symptomatiques au niveau respiratoire (aucun symptôme digestif), dont 9 patients opérés (20%). Sept patients ont eu une investigation supplémentaire par spirométrie, par contre, aucun n’a eu d’imagerie additionnelle. CONCLUSION Les AV sont des anomalies rares et se présentent habituellement dans la première année de vie. Une chirurgie est généralement curatrice et sécuritaire. Néanmoins, plusieurs patients opérés demeurent symptomatiques au dernier suivi. Il serait probablement judicieux d’investiguer ces patients par imagerie pour préciser la cause de la symptomatologie résiduelle.

Introduction: The left pulmonary artery (LPA) contributes more than the right (RPA) to total pulmonary regurgitation (PR) in patients after tetralogy of Fallot (TOF) repair, but the mechanism of this difference is not well known. We hypothesized that unilaterally increased pulmonary vascular resistance (PVR), resulting from lung compression by the enlarged and levorotated heart leads to greater PR in the LPA. This study aimed to analyze the interplay between heart and lung size, mediastinal geometry, and differential PR. Methods: This is a single-center retrospective analysis of 50 magnetic resonance studies in patients after TOF repair. Patients with more than mild discrete branch pulmonary artery stenosis were excluded. Blood flow was measured by phase-contrast velocity encoding within the branch pulmonary arteries. On the axial image with the largest total cardiac surface area, cardiac angle (α) between the thoracic anterior-posterior line and the interventricular septum, right and left lung areas as well as right and left hemithorax areas were measured (Figure). Results: There was no difference in LPA and RPA diameters. The LPA showed significantly less total forward flow (p=0.04), smaller net forward flow (p= Conclusions: An enlarged and levorotated heart - as a result of PR - is associated with smaller left lung size, and augments diastolic flow reversal in the LPA, presumably via increased left PVR. By imposing a further volume load on the RV, LPA regurgitation may thus close a positive feed-back loop of PR and RV dilatation.

During the fall, a 17-day-old boy was brought to the emergency department because of progressive feeding difficulty and lethargy after developing nasal congestion three days before presentation. The infant was born following an uncomplicated full term pregnancy and delivery. The family history was notable only for a five-year-old brother with a bicuspid aortic valve. Both his parents and brother had been recently ill with a cold. The infant was hypotonic and pale with signs of respiratory distress. His heart rate was 140 beats/min, respiratory rate was 64 breaths/min, systolic blood pressure was approximately 80 mmHg, oxygen saturation was 90% on room air and his rectal temperature was 36.5°C. Physical examination revealed hepatomegaly (4 cm to 5 cm below the costal margin) and diminished femoral and radial pulses; no rash was noted. Fluid resuscitation was begun immediately. However, after receiving 60 mL/kg of crystalloid solution, the patient became bradycardic (60 beats/min), requiring chest compressions, intubation and continuous infusion of vasopressors for stabilization. Initial laboratory findings included a mixed respiratory and metabolic acidosis, lymphocytosis, elevated transaminases and evidence of early disseminated intravascular coagulopathy. A chest x-ray demonstrated cardiomegaly (Figure 1). Blood, cerebrospinal fluid (CSF) and urine samples were sent for bacterial culture. Broad-spectrum antibiotics were begun, and the patient was admitted to the paediatric intensive care unit. Further investigations revealed the diagnosis. Figure 1) Chest radiographs at presentation. Anteroposterior (A) and lateral (B) views demonstrate cardiomegaly and mildly increased vascular markings. There is no evidence of parenchymal pulmonary disease.

Pediatric heart failure (HF) is an important cause of morbidity and mortality in childhood. This article presents guidelines for the recognition, diagnosis, and early medical management of HF in infancy, childhood, and adolescence. The guidelines are intended to assist practitioners in office-based or emergency room practice, who encounter children with undiagnosed heart disease and symptoms of possible HF, rather than those who have already received surgical palliation. The guidelines have been developed using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology, and are accompanied by practical Recommendations for their application in the clinical setting, supplemented by online material. This work does not include Recommendations for advanced management involving ventricular assist devices, or other device therapies.

The use of a fenestration in the Fontan pathway remains controversial, partly because its hemodynamic effects and clinical consequences are insufficiently understood. The objective of this study was to quantify the magnitude of fenestration flow and to characterize its hemodynamic consequences after an intermediate interval after surgery.Twenty three patients with a fenestrated extracardiac conduit prospectively underwent investigation by cardiac magnetic resonance (CMR), echocardiography, and invasive manometry under the same general anesthetic 12 ± 4 months after Fontan surgery. Fenestration flow was determined using phase contrast CMR by subtracting flow in the Fontan pathway above the fenestration from Fontan flow below the fenestration.Fenestration flow constituted a mean of 31 ± 12% (range 8-50%) of ventricular preload. It was associated with a lower Qp/Qs (r = -0.64, p=0.001) and oxygen saturation (r = -0.74, p0.0001). Fenestration flow volume was correlated with pulmonary vascular resistance (r = 0.45, p = 0.04) and markers of ventricular diastolic function (early diastolic strain rate r = 0.57, p = 0.008 and ventricular untwist rate r = 0.54, p = 0.02). In 14 patients (61%) all of the net inferior vena cava flow and part of the superior vena cava flow were diverted into the systemic atrium and did not reach the lungs.Fenestration flow can be measured accurately with CMR. In two-thirds of the patients not only all of the inferior vena cava flow, but also some of the superior vena cava flow is diverted through the fenestration. Fenestration flow is driven by a balance between pulmonary vascular resistance and early diastolic ventricular function.

Objective Aortopulmonary collaterals are a frequent phenomenon in patients after bidirectional cavopulmonary connection. The aortopulmonary collateral flow volume can be quantified using cardiac magnetic resonance imaging. However, the significance of aortopulmonary collateral flow for the postoperative outcome after total cavopulmonary connection is unclear and was sought to be determined. Methods The data from 33 patients were prospectively studied with cardiac magnetic resonance, echocardiography, and cardiac catheterization before the total cavopulmonary connection operation. The early postoperative outcomes after total cavopulmonary connection completion were recorded. Results Aortopulmonary collateral flow was 1.59 L/min/m 2 ± 0.65 L/min/m 2 (range, 0.54 L/min/m 2 –3.34 L/min/m 2 ), constituting 43% ± 13% (range, 12–87%) of pulmonary blood flow and 35% ± 12% (range, 11–62%) of the cardiac index, resulting in a pulmonary blood flow/systemic blood flow ratio of 1.06 ± 0.17 (range, 0.79–1.55). The aortopulmonary collateral flow correlated with pulmonary blood flow/systemic blood flow ratio ( r = 0.69, P r = 0.42, P = .018), and cardiac index ( r = 0.53, P = .002). Of the 36 patients, 24 underwent fenestrated total cavopulmonary connection during the study period. The aortopulmonary collateral flow, relative to the cardiac index, correlated with the duration of hospital stay ( r = 0.48, P = .02) and pleural drainage ( r = 0.45, P = .03). Patients whose pleural drainage lasted 1 week or less had less aortopulmonary collateral flow before the Fontan operation than those with a longer period until chest tube removal (1.23 L/min/m 2 ± 0.38 L/min/m 2 vs 1.73 L/min/m 2 ± 0.76 L/min/m 2 ; P = .03). Compared with a contemporary group of total cavopulmonary connection patients with fenestration in their extracardiac conduit who were studied prospectively, with a similar protocol, the bidirectional cavopulmonary connection had a greater amount of aortopulmonary collateral flow (1.59 L/min/m 2 ± 0.65 L/min/m 2 vs 1.30 L/min/m 2 ± 0.57 L/min/m 2 , P = .04). Conclusions Patients after bidirectional cavopulmonary connection routinely acquire a large amount of aortopulmonary collateral flow. The hemodynamic consequences of aortopulmonary collateral flow translate into adverse outcomes early after total cavopulmonary connection completion.

INTRODUCTION: We previously reported the clinical outcomes of a large pediatric cohort with discrete subaortic stenosis (SubAS) 1 . While patients with more significant left ventricular outflow tract obstruction (LVOTO) at diagnosis progressed rapidly others with mild LVOTO at presentation showed little progression of obstruction or aortic insufficiency (AI). A limitation of our previous study was a relatively short follow-up period. OBJECTIVE: To extend the follow-up of our previously described cohort with SubAS. METHODS: Information was gathered retrospectively on 71 previously identified patients diagnosed with SubAS from 1985–1998. RESULTS: Current data was available on 61/71 patients. At diagnosis the average age was 5.2±0.5 years (mean±SEM), peak LVOT Doppler gradient (Echograd) was 30±3 mmHg, and AI (all mild) was present in 25%. At last assessment the average age was 15.7±0.4 years. Twenty patients were followed only medically and were last reassessed 9.2±1.0 years after diagnosis at 15.8±0.7 years of age. The mean Echograd was unchanged from diagnosis (19±2 vs. 19±3 mmHg). The incidence of AI on echocardiography (all mild) increased from 5% to 55% although none was detectable by auscultation. Forty-one patients underwent surgery 3.0±0.5 years after diagnosis at 7.4±0.6 years of age. The Echograd at diagnosis was 35±3 and increased to 61±6 mmHg pre-operatively. Ten patients were re-operated 5.7±0.7 years after the first surgery and 2 patients had a second re-operation. The last follow up of the operated patients occurred at 15.7±0.6 years of age. At that time the Echograd was 16±3 mmHg and the incidence of AI by echocardiography had increased to 98% (36 mild & 3 moderate) but was evident on auscultation in only 25%. CONCLUSIONS: This study represents the largest and longest contemporary follow-up of pediatric patients with discrete SubAS. Our results indicate that some patients with SubAS show significant LVOTO at diagnosis and subsequent progression. Surgery in these patients provides good relief of LVOTO but does not eliminate AI. The one third of patients presenting with mild LVOTO and AI show little progression through childhood and adolescence. We suggest that the latter can be safely followed medically. 1) Rohlicek et al. Heart 82:708;1999

Background The management of pediatric discrete subaortic stenosis remains controversial. Objectives Document the natural history and surgical outcomes for discrete subaortic stenosis to adolescence. Methods Retrospective review of clinical and echocardiographic findings in 74 patients diagnosed in childhood between 1985 and 1998. Results Twenty-five patients were followed only medically for 9.4 ± 0.9 years to 15.9 ± 0.6 years of age. Their echocardiographic left ventricular outflow peak gradient did not progress, 19 ± 1.4 (SEM) vs 20 ± 2.3 mm Hg. The proportion with aortic insufficiency (AI) increased (4% to 52%). Forty-nine patients were operated for discrete subaortic stenosis at 7.8 ± 0.6 years. Their peak gradient at diagnosis was 36 ± 3 mm Hg with AI in 33%. Preoperatively their peak gradient progressed to 60 ± 5 mm Hg with AI in 82%. Assessment 6.2 ± 0.5 years postoperativly showed a peak gradient of 14 ± 2 mm Hg with AI in 88%. Ten patients required reoperation for recurrent discrete subaortic stenosis, 3 acquired complete heart block, and 1 developed endocarditis. There was no mortality. At diagnosis, surgical patients were younger, had greater peak gradients, and greater incidence of AI, than those followed only medically. The progression of discrete subaortic stenosis was positively associated with severity of obstruction and negatively associated with older age at diagnosis. The risk of having surgery over time was associated with greater preoperative obstruction and presence of AI. Conclusions Many pediatric patients with mild discrete subaortic stenosis exhibit little progression of obstruction and need not undergo immediate surgery. Others with more severe stenosis may progress precipitously and will benefit from early resection.