Postnatal outcome following fetal aortic valvuloplasty for critical aortic stenosis.

Objective: To report our experience of fetal aortic valvuloplasty (FAV) for critical aortic stenosis (AS), with a focus on the postnatal evolution of the patients.

Methods: This was a retrospective study including all fetuses with critical AS which underwent FAV in a single center between January 2011 and June 2022. FAV was performed under ultrasound guidance.

[Fetal aortic valvuloplasty in critical aortic stenosis: indication, technique and postnatal outcomes].

Aortic stenosis is a complex heart disease that involves the aortic valve and the left ventricle. Impairment of the left ventricle, abnormalities in its size, systolic and diastolic function determine the postnatal outcomes in the same way as the aortic valve. In the most severe forms, the left ventricle cannot provide systemic circulation at birth and the physiology is that of hypoplastic left heart syndrome.

Accuracy and impact of prenatal diagnosis of common arterial trunk.

Objectives: Outcome of common arterial trunk (CAT) depends mainly on truncal valve function, presence of coronary artery abnormalities and presence of interrupted aortic arch. The main objective of this study was to evaluate the accuracy of prenatal diagnosis of CAT by analyzing prenatal vs postnatal assessment of: (1) anatomic subtypes and (2) truncal valve function. The secondary objective was to assess the potential impact of prenatal diagnosis of CAT on postnatal mortality and morbidity by comparing prenatally vs postnatally diagnosed patients.

Management and Outcomes of Neonatal Arteriovenous Brain Malformations with Cardiac Failure: A 17 Years' Experience in a Tertiary Referral Center.

Objectives: To assess the management and outcomes of neonatal arteriovenous brain malformations (mostly vein of Galen malformations) complicated by cardiac failure in the era of prenatal diagnosis and endovascular treatment in a tertiary referral center.

Study Design: This observational study included 77 living newborn infants with arteriovenous brain malformations with cardiac failure, admitted to our referral center from 2001 to 2017. All infants underwent cardiovascular evaluation including echocardiogram and brain magnetic resonance imaging.

Successful in utero transesophageal pacing for severe drug-resistant tachyarrhythmia.

Sustained fetal tachyarrhythmia can evolve into a life-threatening condition in 40% of cases when hydrops develops, with a 27% risk of perinatal death. Several antiarrhythmic drugs can be given solely or in combination to the mother to achieve therapeutic transplacental concentrations. Therapeutic failure could lead to progressive cardiac insufficiency and restrict therapeutic options to either elective delivery or direct fetal administration of antiarrhythmic drugs, which may increase the risk of death.

Discordances Between Pre-Natal and Post-Natal Diagnoses of Congenital Heart Diseases and Impact on Care Strategies.

Background: Pre-natal diagnosis of congenital heart disease (CHD) allows anticipation of urgent neonatal treatment and provides adequate information to the parents on cardiac outcomes.

Objectives: This study sought to analyze the discordances between expert fetal cardiac diagnosis and final diagnosis of CHD and their impact on neonatal and long-term care strategies.

Methods: We included 1,258 neonates with a pre-natally diagnosed CHD and 189 fetopsies following termination of pregnancy at our tertiary center over a 10-year period.

Critical Congenital Heart Diseases in Preterm Neonates: Is Early Cardiac Surgery Quite Reasonable?

Prematurity is a recognized risk factor for morbidity and mortality following cardiac surgery. Postoperative and long-term outcomes after cardiac surgery performed in the preterm period are poorly described. The aim of this study was to analyze a population of preterm neonates operated on for critical congenital heart disease (CHD) before 37 weeks of gestational age (wGA) with special attention given to early and late mortality and morbidity.

Repeat mitral valve repair for haemolysis in children.

Background: Severe haemolysis is a rare complication after mitral valve repair in congenital heart disease.

Aim: We describe four children with severe mitral regurgitation who underwent valve repair and subsequently developed profound haemolytic anaemia.

Methods: Clinical, echocardiographic and surgical data were collected retrospectively from a surgical centre in France during a 5-year period.

Case of a healthy infant born following antenatal enterovirus myocarditis and hydrops.

Fetal hydrops and myocarditis were diagnosed in a woman at 32 weeks of gestation (WG). Transplacental enterovirus infection was suspected because all other causes of myocarditis and hydrops were excluded, it was during an endemic period, and there was a setting of maternal infection (fever a few days before). We opted for in utero treatment because of the risk of resuscitating a neonate with myocarditis and hydrops.

A standardized repair-oriented strategy for mitral insufficiency in infants and children: midterm functional outcomes and predictors of adverse events.

Objective: Surgical management of mitral regurgitation (MR) in children remains a challenge because of the heterogeneity of the anatomy, growth potential, and necessity to avoid valve replacement. Our objective was to assess the functional outcomes and prognostic factors of a standardized strategy of mitral valve (MV) repair for children with MR.

Methods: Consecutive patients aged <18 years who had undergone surgery for severe MR from 2001 to 2012 were studied retrospectively.

Reoperations for left atrioventricular valve dysfunction after repair of atrioventricular septal defect.

Objectives: Postoperative left atrioventricular valve (LAVV) dysfunction is known to be the principal risk factor influencing outcome after repair of all types of atrioventricular septal defect (AVSD). The purpose of the present study was to identify the risk factors for reoperation and to assess the outcomes after reoperation for LAVV dysfunction.

Methods: Records of 412 patients who underwent anatomical repair for different types of AVSD from January 2000 to July 2012 were reviewed.

Surface-length index: a novel index for rapid detection of right ventricles with abnormal ejection fraction using cardiac MRI.

Objective: To validate a new index, the surface-length index (SLI) based on area change in a short-axis view and length reduction in the horizontal long-axis view, which is used to quickly (<1 min) detect right ventricles with an abnormal ejection fraction (EF) during a cardiac MRI examination. SLI can be used to avoid a complete delineation of the endocardial contours of normal right ventricles.

Methods: Sixty patients (group A) were retrospectively included to calibrate the SLI formula by optimisation of the area under the ROC curves and SLI thresholds were chosen to obtain 100 % sensitivity.

Arterial switch for transposition with left outflow tract obstruction: outcomes and risk analysis.

Background: The long-term results and indications of the arterial switch operation (ASO) for transposition of the great arteries (TGA) and anatomic left ventricular outflow tract obstruction (LVOTO) remain undetermined. The aims of this study were to determine long-term outcomes and prognostic factors in this specific population.

Methods: Between 1986 and 2011, 55 patients with TGA and anatomic LVOTO underwent ASO.

Right ventricular systolic strain is altered in children with sickle cell disease.

Background: Several adult studies have shown that sickle cell disease is associated with cardiac abnormalities and premature death. The aim of this study was to use speckle-tracking strain, a relatively load independent parameter, to evaluate systolic left ventricular (LV) and right ventricular (RV) function in a pediatric sickle cell disease population.

Methods: Twenty-eight patients with sickle cell disease (mean age, 10.

Echocardiographic right ventricle longitudinal contraction indices cannot predict ejection fraction in post-operative Fallot children.

Aims: To examine in a population of post-operative tetralogy of Fallot patients, the correlation between right ventricle (RV) ejection fractions (EF) computed from magnetic resonance imaging (MRI) and three echocardiographic indices of RV function: TAPSE, longitudinal strain and strain rate. Indeed, these patients present a pulmonary regurgitation which is responsible for progressive dilatation of the RV. An echocardiographic assessment of the RV function would be very useful in determining the timing of pulmonary revalvulation for Fallot patients.

[Prenatal diagnosis of aorta coarctations].

Coarctations of the aorta can be associated with severe neonatal consequences. Screening for and diagnosis of this prenatal malformation remain difficult. We review the various tools currently available to us, and their respective limits, to minimize the rate of false negatives and false positives associated with prenatal screening for this situation.

Laronidase for cardiopulmonary disease in Hurler syndrome 12 years after bone marrow transplantation.

A patient with severe mucopolysaccharidosis type I (Hurler syndrome) underwent bone marrow transplantation twice (at the ages of 2 and 2.5 years), both times with his HLA-identical heterozygous brother as the donor. Between the ages of 10 and 14 years, despite 92% donor engraftment and 50% normal α-L-iduronidase activity, he developed progressive respiratory failure with severe pulmonary arterial hypertension, upper airway obstruction, and interstitial lung disease.

Right ventricular failure secondary to chronic overload in congenital heart disease: an experimental model for therapeutic innovation.

Objective: Mortality and morbidity related to right ventricular failure remain a problem for the long-term outcome of congenital heart diseases. Therapeutic innovation requires establishing an animal model reproducing right ventricular dysfunction secondary to chronic pressure-volume overload.

Methods: Right ventricular tract enlargement by transvalvular patch and pulmonary artery banding were created in 2-month-old piglets (n = 6) to mimic repaired tetralogy of Fallot.

Health-e-Child project: mechanical dyssynchrony in children with dilated cardiomyopathy.

Objective: Left ventricular mechanical dyssynchrony has been poorly studied in the pediatric population with dilated cardiomyopathy. We investigated the degree of dyssynchrony in children with dilated cardiomyopathy using tissue Doppler imaging and speckle tracking strain.

Methods: Twenty-five children with dilated cardiomyopathy were compared with healthy subjects.

Supracardiac total anomalous pulmonary venous connection: the transaortopulmonary approach.

We have been confronted with patients in whom classical techniques did not offer optimum exposure to correct supracardiac forms of total anomalous pulmonary venous connection, especially in neonates. Therefore, we present a surgical modification of the superior approach for enhanced exposure as a result of transection of the ascending aorta associated or not with the transection of the pulmonary trunk. The transaortopulmonary approach ensures a perfect exposition without any need to pull on the surrounding structures.