Gestational Age-Specific Markers Associated with Postnatal Intervention in Fetal Suspicion of Coarctation of the Aorta.

OBJECTIVE: Fetal diagnosis of coarctation of the aorta (CoA) is currently associated with a high false-positive rate. Many predictive markers may be gestational age (GA)-specific. We sought to establish GA-specific traditional and speckle-tracking fetal echocardiography (STE) markers predictive of true CoA in neonates with prenatal suspicion. STUDY DESIGN: This is a retrospective case-control study. We compared the fetal ventricular and arch dimensions, as well as the deformation parameters by STE, of infants who required a postnatal intervention for their CoA with those who did not. Cohort was stratified based on GA before or after 30 weeks. Data extractors were masked to the outcome. The first fetal echocardiogram available was used. RESULTS: Seventy-five newborns with a fetal echocardiography performed between October 2013 and May 2022 for an antenatal suspicion of CoA were included, of which 59 (79%) had an aortic arch with nonsignificant obstruction upon ductal closure, and 16 (21%) underwent a neonatal intervention for a confirmed CoA. Before 30 weeks' GA, the right ventricular to left ventricular (RV/LV) end-diastolic width and end-diastolic area (EDA) ratios were most associated with postnatal CoA confirmation (area under the curve [AUCs] = 0.96 and 0.92). After 30 weeks' GA, the RV/LV end-diastolic width ratio (AUC = 0.95), the Z-score for the ascending aorta (AUC = 0.93), and the LV end-diastolic width Z-score (AUC = 0.91) performed the best. A decreased RV peak longitudinal strain was observed in those who developed true CoA and performed well by receiver operating characteristic analysis after 30 weeks (AUC = 0.85). In the overall cohort, the RV/LV EDA ratio was the most sensitive predictor of CoA and identified all cases with CoA. Indeed, a cutoff > 1.24 had a specificity of 69.5% and a sensitivity of 100% (receiver operating characteristic curve with an AUC of 0.88). CONCLUSION: We outlined sensitive and specific fetal markers associated with postnatal CoA based on GA at suspicion. KEY POINTS: · Fetal ventricular disproportion predicts postnatal coarctation.. · A decreased right ventircular contraction was observed in those with coarctation.. · Fetal markers differ based on gestational age at fetal evaluation..

An unusual course of anti-Ro antibody-mediated fetal complete heart block.

Fetal hydrops is a serious complication of immune-mediated congenital complete atrioventricular block. We present the case of a fetus with severe hydrops and profound bradycardia and an unusual favourable outcome. This case enhances the importance of considering the contribution of ventricular ectopic beats to the cardiac output when counselling and predicting outcome of complete heart block.

Pathophysiology, screening and diagnosis of pulmonary hypertension in infants with bronchopulmonary dysplasia - A review of the literature.

Bronchopulmonary dysplasia (BPD) is a common complication of extreme prematurity, which has increased over the last 20 years. BPD is associated with increased morbidities and mortality. It has been increasingly recognized that BPD affects overall lung development including the pulmonary vasculature. More recent studies have demonstrated an increased awareness of pulmonary arterial hypertension (PH) in BPD patients and recent international guidelines have advocated for better screening. This review will describe the current understanding of the pathophysiology of PH in infants with BPD, the in-depth assessment of the available literature linking PH and BPD, and propose an approach of screening and diagnosis of PH in infants with BPD.

The biventricular contribution to chronic pulmonary hypertension of the extremely premature infant.

OBJECTIVE: Evaluate factors associated with significant pulmonary hypertension [PH] (≥2/3 systemic) and its impact on ventricular function at 36 weeks postmenstrual age (PMA). STUDY DESIGN: Retrospective cohort of infants born at <29 weeks who survived to their echocardiography screening for PH at 36 weeks PMA. Masked experts extracted conventional and speckle-tracking echocardiography [STE] data. RESULTS: Of 387 infants, 222 were included and 24 (11%) categorized as significant PH. Significant PH was associated with a decrease in tricuspid annular plane systolic excursion (0.79 vs 0.87 cm, p = 0.03), right peak longitudinal strain [pLS] by STE (-19.6 vs -23.1%, p = 0.003) and left pLS (-25.0 vs -22.7%, p = 0.02). The association between biventricular altered function by STE and significant PH persisted after adjustment for potential confounders - LV-pLS (p = 0.007) and RV-pLS (p = 0.01). CONCLUSION: Our findings are suggestive that premature newborns with significant PH at 36 weeks PMA have a biventricular cardiac involvement to their pathophysiology.

Early Echocardiography Predicts Intervention Need in Antenatal Suspicion of Coarctation of the Aorta.

Background: Coarctation of the aorta (CoA) is challenging to diagnose in early postnatal life. We aimed to describe the resource utilization and predictors for the need of intervention in an antenatal suspicion of CoA. Methods: A retrospective study of infants with an antenatal suspicion of CoA born at ≥37 weeks was performed. Those not requiring intervention (normal) were compared with those who required cardiac surgery (CoA). Strain was measured using speckle-tracking echocardiography. Results: A total of 51 newborns were included; 40 (78%) were considered normal and 11 (22%) underwent intervention. Echocardiography occurred within the first day of life for both groups. Right ventricular (RV) predominance was present in the CoA group, as demonstrated by the left ventricular (LV) end-systolic eccentricity index (1.60 [0.28] vs 2.16 [0.45]; P < 0.001) and by a larger RV end-diastolic area (EDA) in apical 4-chamber (A4C) relative to LV-EDA-with a ratio of 1.56 [0.23] vs 1.02 [0.2]; P < 0.001. An RV/LV EDA ratio in A4C ≥1.3 had a high probability for CoA (area under the curve = 0.97). Newborns with CoA had a lower RV deformation (peak systolic strain rate: -0.98 [0.17] vs -0.83 [0.2]; P = 0.02). Intraclass correlation coefficient for the EDA ratio revealed a good inter-rater agreement (0.76; 95% confidence interval: 0.55-0.87). Analysis with rater #2 revealed that the EDA ratio ≥1.3 predicted 100% of CoA. Conclusions: The majority of those with an antenatal suspicion of CoA did not require intervention but were high consumers of resources. Within the first day of life, the ventricular EDA ratio in A4C may help predicting those with true CoA requiring intervention.

Contexte: La coarctation de l'aorte (CoA) est difficile à diagnostiquer au début de la vie postnatale. Nous avons voulu décrire l'utilisation de ressources et les facteurs prédictifs à prendre en compte pour déterminer la nécessité d'une intervention en cas de suspicion prénatale de CoA. Méthodologie: Une étude rétrospective portant sur des nourrissons nés à ≥ 37 semaines chez qui il y avait une suspicion prénatale de CoA a été réalisée. Ceux chez qui aucune intervention n'a été nécessaire (groupe de sujets sans anomalie) ont été comparés à ceux chez qui une intervention chirurgicale cardiaque a été nécessaire (groupe de sujets présentant une CoA). L'échocardiographie de suivi des marqueurs acoustiques (speckle-tracking echocardiography) a servi à mesurer la déformation. Résultats: Au total, 51 nouveau-nés ont été inclus; 40 (78 %) ne présentant aucune anomalie selon les évaluateurs, et 11 (22 %) ayant subi une intervention. L'échocardiographie a été réalisée au cours du premier jour de vie dans les deux groupes. Une prédominance ventriculaire droite existait chez les sujets présentant une CoA, comme l'ont démontré l'indice d'excentricité ventriculaire gauche télésystolique (1,60 [0,28] vs 2,16 [0,45]; P < 0,001) et la plus grande surface télédiastolique du ventricule droit (STDVD) en vue apicale 4 cavités (A4C) par rapport à la surface télédiastolique du ventricule gauche (STDVG) ­ le rapport étant de 1,56 [0,23] vs 1,02 [0,2]; P < 0,001. Un rapport STDVD/STDVG en A4C ≥ 1,3 correspondait à une forte probabilité de CoA (aire sous la courbe = 0,97). La déformation ventriculaire droite était moindre chez les nouveau-nés présentant une CoA (taux de déformation systolique maximal : −0,98 [0,17] vs −0,83 [0,2]; P = 0,02). Le coefficient de corrélation intraclasse pour le rapport des surfaces télédiastoliques a révélé une bonne concordance interévaluateurs (0,76; intervalle de confiance à 95 % : 0,55-0,87). L'analyse de l'évaluateur no 2 a révélé qu'un rapport des surfaces télédiastoliques ≥ 1,3 avait une valeur prédictive de 100 % au regard de la CoA. Conclusions: Dans la majorité des cas où il y avait suspicion prénatale de CoA, aucune intervention n'a été nécessaire, mais les ressources médicales mobilisées étaient importantes. Dans ce contexte, le calcul du rapport des surfaces télédiastoliques ventriculaires en A4C au cours du premier jour de vie peut aider à prévoir les cas où une véritable CoA nécessitera une intervention.

Postnatal recurrence and transesophageal inducibility of prenatally treated fetal supraventricular tachycardia.

BACKGROUND: Antiarrhythmic treatment of fetal supraventricular tachycardia (SVT) is used to prevent morbidity and mortality. The postnatal management of survivors is often arbitrary and varied. OBJECTIVE: The purpose of this study was to examine the utility of a risk-based postnatal management strategy. METHODS: Sixty-six prenatally treated newborns with fetal long or short ventriculoatrial tachycardia were reviewed. Postnatal diagnoses included atrioventricular reentrant tachycardia, atrial ectopic tachycardia, and permanent junctional reciprocating tachycardia. Unless SVT persisted to birth, early neonatal observation without treatment was recommended. For newborns without spontaneous arrhythmia after ≥2 days of observation, inducibility was tested by transesophageal pacing study (TEPS). Postnatal therapy was advised for spontaneous or inducible SVT. Characteristics associated with these outcomes were analyzed. RESULTS: Twenty-eight patients (42%) experienced SVT at or early after birth, which was associated with fetal long ventriculoatrial tachycardia (odds ratio [OR] 6.8; 95% confidence interval [CI] 1.88-24.57; P = .0029); delayed in utero cardioversion with treatment (median 11 days vs 5.5 days; P < .0001); prenatal treatment with multiple antiarrhythmics (OR 4.42; 95% CI 1.56-12.55; P = .0059); and postnatal atrial ectopic tachycardia/permanent junctional reciprocating (OR 18.0; 95% CI 2.11-153.9; P = .0013). Of the 38 neonates undergoing TEPS, 19 (50%) had inducible tachyarrhythmias. Recurrence of SVT during infancy or childhood was documented in 4 of 6 patients with SVT at birth (66%), 8 of 22 patients with early neonatal SVT (36%), 4 of 19 patients with inducible SVT (21%), and 0 of 19 untreated patients without inducible SVT (0%) (P = .0032). CONCLUSION: The postnatal risk of SVT is related to the arrhythmia mechanism and prenatal treatment response. In newborns without spontaneous SVT, TEPS may be useful to guide the need for postnatal treatment on the basis of SVT inducibility.

Recent Graduates Experience Difficulty in Finding Positions Despite Apparent Need for More Pediatric Cardiologists in Canada.

There have been reports that pediatric cardiology is an increasingly competitive field and that it has become difficult for recent graduates to find employment. In Canada, empirical observations are consistent with these findings, but this has not been formally evaluated. The aim of this study was to survey pediatric cardiologists (PCs) on their perception of the current workforce in Canada. A survey was sent to PCs and trainee members of the Canadian Pediatric Cardiology Association in November 2017. Eligible nonmembers of the Canadian Pediatric Cardiology Association were also contacted with the help of Canadian program directors and division chiefs. A total of 68 of 83 PCs completed the survey (82%). The proportion of respondents reporting difficulties in finding a stable position in pediatric cardiology was 83% for those finishing training after 2014 compared with 33% between 2010 and 2014. There was a concomitant increase in the duration of additional subspecialization, which more than doubled after 2014. This contrasted with most PCs (57%) and division chiefs (86%) reporting being understaffed, and with a higher number of anticipated openings compared with available trainees. Division chiefs reporting being understaffed mostly attributed this to government regulations or institutional budgetary constraints. We observed a discrepancy between a perceived need for additional PCs and a reported increasing difficulty in finding employment by recent graduates. This coincided with an increasing number of additional subspecialization years by recent graduates. Institutional and government restrictions may contribute to this situation.

Des études ont montré que la cardiologie pédiatrique est un domaine de plus en plus concurrentiel. Conséquemment, les diplômés actuels ont du mal à trouver de l'emploi. Au Canada, des observations empiriques sont cohérentes avec ces conclusions, mais aucune évaluation formelle n'a été réalisée. L'objectif de la présente étude était de mener une enquête auprès des cardiologues pédiatres (CP) sur leur perception de la main-d'œuvre actuelle au Canada. En novembre 2017, nous avons envoyé un sondage aux CP et aux résidents membres de l'Association canadienne de cardiologie pédiatrique. Nous sommes également entrés en contact avec les non-membres admissibles de l'Association canadienne de cardiologie pédiatrique par l'intermédiaire des directeurs de programmes et des chefs de division. Soixante-huit des quatre-vingt-trois CP ont rempli l'enquête (82 %). Le pourcentage des répondants qui ont rapporté avoir des difficultés à trouver un poste stable en cardiologie pédiatrique était de 83 % chez ceux ayant terminé leur formation après 2014, alors que le pourcentage était de 33 % chez ceux ayant terminé leur formation entre 2010 et 2014. La durée des formations en surspécialité a augmenté de manière concomitante et a plus que doublé après 2014. Ces données entraient en contradiction avec les commentaires de la plupart des CP (57 %) et des chefs de division (86 %) qui, disaient manquer de personnel et avoir un nombre anticipé plus de postes vacants plus élevés que le nombre de résidents disponibles. Les chefs de division qui rapportaient manquer de personnel attribuaient principalement cela à des contraintes gouvernementales ou budgétaires. Nous avons observé des contradictions entre les besoins perçus pour des CP additionnels et la difficulté croissante que rapportent les diplômés actuels à trouver de l'emploi. Ceci coïncide avec un nombre croissant d'années supplémentaires de formation en surspécialisation. Des contraintes institutionnelles ou gouvernementales contribuent possiblement à cette situation.

Aspirin Dose and Prevention of Coronary Abnormalities in Kawasaki Disease.

BACKGROUND: Acetylsalicylic acid (ASA) is part of the recommended treatment of Kawasaki disease (KD). Controversies remain regarding the optimal dose of ASA to be used. We aimed to evaluate the noninferiority of ASA at an antiplatelet dose in acute KD in preventing coronary artery (CA) abnormalities. METHODS: This is a multicenter, retrospective, nonrandomized cohort study including children 0 to 10 years of age with acute KD between 2004 and 2015 from 5 institutions, of which 2 routinely use low-dose ASA (3-5 mg/kg per day) and 3 use high-dose ASA (80 mg/kg per day). Outcomes were CA abnormalities defined as a CA diameter with a z score ≥2.5. We assessed the risk difference of CA abnormalities according to ASA dose. All subjects received ASA and intravenous immunoglobulin within 10 days of fever onset. RESULTS: There were 1213 subjects included, 848 in the high-dose and 365 in the low-dose ASA group. There was no difference in the risk of CA abnormalities in the low-dose compared with the high-dose ASA group (22.2% vs 20.5%). The risk difference adjusted for potential confounders was 0.3% (95% confidence interval [CI]: -4.5% to 5.0%). The adjusted risk difference for CA abnormalities persisting at the 6-week follow-up was -1.9% (95% CI: -5.3% to 1.5%). The 95% CI of the risk difference of CA abnormalities adjusted for confounders was within the prespecified 5% margin considered to be noninferior. CONCLUSIONS: In conjunction with intravenous immunoglobulin, low-dose ASA in acute KD is not inferior to high-dose ASA for reducing the risk of CA abnormalities.

Intrauterine therapy for structural congenital heart disease: Contemporary results and Canadian experience.

Percutaneous, ultrasound-guided fetal cardiac intervention (FCI) is increasingly used to alter the prognosis of specific forms of congenital heart disease. Careful patient selection and postnatal management strategy are essential for optimal outcomes. This article discusses the rationale, patient selection criteria, procedural techniques, and contemporary results of FCI. Sources of information included published patient series, the International Fetal Cardiac Intervention Registry, and the Toronto experience as the Canadian referral center.

Inflammatory myofibroblastic tumor of the aortic valves causing sudden cardiac death: a case report and review of the literature.

Cardiac inflammatory myofibroblastic tumor (IMT) is a rare entity affecting predominantly infants, children, and young adults. Although most tumors have a benign clinical course after complete surgical resection, some have significant clinical effects. We report the case of a 9-year-old girl who had sudden cardiac death as a result of occlusion of the left circumflex coronary artery. A review of 57 cases of cardiac IMTs reported in the literature in terms of epidemiology, clinical presentation, histologic and immunohistologic features, and outcome is presented. Recognition of this rare abnormality is important in order to initiate prompt surgical intervention.