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RésuméUne arythmie cardiaque fœtale est dépistée chez 1 à 2 % des grossesses. Un diagnostic précis est important pour adapter la prise en charge thérapeutique, ce qui est le plus souvent possible par l’échocardiographie TM, le mode Doppler et de nouveaux outils diagnostiques. Il s’agit souvent d’extrasystoles intermittentes qui ne justifient pas de traitement. Les arythmies soutenues sont à risque d’anasarque fœtale, de séquelles neurologiques, de prématurité et de mort fœtale in utero. Les BAV complets (1/15 000–20 000 naissances) peuvent être associés aux anticorps anti-SSA/SSB maternels ou à des cardiopathies congénitales (isomérismes gauches, double discordance) de plus mauvais pronostic. L’utilisation d’une corticothérapie maternelle dans les BAV immunologiques est controversée. Les tachycardies fœtales concernent environ 0,4–0,6 % des grossesses. Les causes les plus fréquentes de tachycardie fœtale sont la tachycardie supraventriculaire (TSV) avec intervalle ventriculo-auriculaire court, liée à une réentrée, et le flutter atrial. La tachycardie ventriculaire est rare chez le fœtus. Le choix de la prise en charge dépend de nombreux facteurs différents : âge gestationnel, mécanisme de l’arythmie, fréquence cardiaque, durée de la tachycardie, présence ou non d’insuffisance cardiaque congestive et d’anasarque. La majorité des fœtus avec tachycardie sont traités in utero avec succès par l’administration transplacentaire de médicaments antiarythmiques. La digoxine est largement acceptée comme l’antiarythmique de première ligne en absence d’anasarque. Le sotalol, la flécaïnide et l’amiodarone, ou des associations, sont utilisés comme médicaments de deuxième ligne quand la digoxine échoue à réduire la tachycardie ou en cas d’anasarque qui est le principal marqueur du risque de mort fœtale in utero.SummaryFoetal cardiac dysrhythmias are diagnosed in 1–2 % of pregnancies. Precise diagnosis of cardiac arrhythmias is important for a managed therapeutic approach and is possible using M-mode pulsed-wave Doppler echocardiography and new diagnostic tools. Dysrhythmias are often due to intermittent extrasystoles, which do not require treatment. Sustained forms of arrhythmias may have a significant risk for foetal hydrops, neurological damage, prematurity and intrauterine death. Complete atrioventricular block (1/15 000–20 000 live births) can be associated with maternal anti-SSA/Ro and anti-SSB/la autoantibodies or with congenital heart disease (left atrial isomerism, congenitally corrected transposition of great arteries) and poor prognosis. The use of steroids to treat foetal atrioventricular block is controversial. Foetal tachycardia occurs in approximately 0.4–0.6% of all pregnancies. Common causes of foetal tachycardia are supraventricular tachycardia with short ventriculoatrial interval due to atrioventricular re-entrant and atrial flutter. Ventricular tachycardia is rare in the foetus. The choice of management is correlated to many factors: gestational age, underlying pathophysiology of the arrhythmia, foetal heart rate, duration of the tachyarrhythmia and the presence or absence of congestive heart failure and hydrops. Most foetuses with tachycardia are successfully treated in utero by transplacental administration of antiarrhythmic drugs. Digoxin is widely accepted as a first-line antiarrhythmic drug in the absence of foetal hydrops. Sotalol, flecainide and amiodarone or combinations are used as second-line drugs when digoxin fails to achieve conversion to sinus rhythm or in case of hydrops, which is the main risk factor for intrauterine death.

Purpose When isolated and non immune, prevalence of congenital and childhood atrioventricular blocks (AVB) is extremely low and little is known about their natural history. Methods a multicentric study retrospective from 1980 to 2009 allowed inclusion of 141 children from 13 French referral centers. Included children presented an AVB diagnosed in utero, at birth or during childhood before the age of 15 years, without structural heart abnormalities and without maternal antibodies. Results 26 congenital and 114 childhood AVB were included. Symptoms lead to diagnosis in 15,6% whereas AVB was asymptomatic in 84,4%. 73% AVB were complete and 26,2% AVB were incomplete at first presentation. 21 of these incomplete blocks (56,7%) progressed to permanent complete AVB. Narrow QRS complexes were found in 69,2% congenital and 91,2% childhood AVB. In the 112 (79,4%) implanted children, mean duration between AVB diagnosis and pacemaker implantation was 35 months. Pacemaker primo-implantation occurred during the first year of life for 18 children and 90 children (63.8%) were paced before 10 years old. Pacing was required for symptomatic bradycardia in 37,5% whereas prophylactic cardiac pacing accounted for 61,6%. The median follow-up was 96 months (from 6 to 384 months). 85,1% experienced no complication and neither dilated cardiomyopathy nor death had occurred at last follow-up. Pacemaker-related complications appeared in 11,6%. Conclusion We describe the largest reported experience with isolated and non immune congenital and childhood AVB. Such a block is a nodal damage from unknown origin that may postnatally progress in incomplete forms. Outcome is not influenced by age at diagnosis. Prognosis is very good with no late-onset dilated cardiomyopathy, a few pacemaker-related complications in the modern technological era and no death at last follow-up.

Purpose When isolated and non immune, prevalence of congenital and childhood atrioventricular blocks (AVB) is extremely low and little is known about their natural history. Methods A multicentric study retrospective from 1980 to 2009 allowed inclusion of 141 children from 13 French referral centers. Included children presented an AVB diagnosed in utero, at birth or during childhood before the age of 15 years, without structural heart abnormalities and without maternal antibodies. Results 26 congenital and 114 childhood AVB were included. Symptoms lead to diagnosis in 15,6% whereas AVB was asymptomatic in 84,4%. 73% AVB were complete and 26,2% AVB were incomplete at first presentation. 21 of these incomplete blocks (56,7%) progressed to permanent complete AVB. Narrow QRS complexes were found in 69,2% congenital and 91,2% childhood AVB. In the 112 (79,4%) implanted children, mean duration between AVB diagnosis and pacemaker implantation was 35 months. Pacemaker primo-implantation occurred during the first year of life for 18 children and 90 children (63.8%) were paced before 10 years old. Pacing was required for symptomatic bradycardia in 37,5% whereas prophylactic cardiac pacing accounted for 61,6%. The median follow-up was 96 months (from 6 to 384 months). 85,1% experienced no complication and neither dilated cardiomyopathy nor death had occurred at last follow-up. Pacemaker-related complications appeared in 11,6%. Conclusion We describe the largest reported experience with isolated and non immune congenital and childhood AVB. Such a block is a nodal damage from unknown origin that may postnatally progress in incomplete forms. Outcome is not influenced by age at diagnosis. Prognosis is very good with no late-onset dilated cardiomyopathy, a few pacemaker-related complications in the modern technological era and no death at last follow-up.

Background Subaortic membrane (SAM) is a fibrous membrane located below the aortic valve. Severe obstruction and/or lesions of valve leaflets require surgical resection of the SAM. Proximity of the SAM to the aortic valve predicts recurrent SAM requiring reoperation. 2D echocardiography (2DE) failed to describe precisely the SAM and its adjacent structures. Aim To assess the accuracy of transthoracic real-time 3D echocardiography (3DE) to describe the SAM. Methods Twenty-two patients (median age 8 years, 3 to 40 years) with SAM were prospectively enrolled. 2DE and Doppler assessed aortic regurgitation grade, left ventricular to aorta Doppler mean gradient. 3DE was performed using the matrix probe (×1-3 in patients over 7 years old; ×7-2 in younger patients). Q-Lab system (Philips) allowed (1) measurements of 3D sub aortic obstruction surface and distance between SAM and aortic leaflets; (2) description of attachment of the SAM to the mitral valve. Ten patients underwent surgical resection of the SAM. Surgical views were compared with 3DE views. Results Aortic regurgitation assessed by 2DE and Doppler was present in 14 patients (grade 1 in 10, grade 2 in 4); left ventricular to aorta Doppler mean gradient ranged from 10 to 60 mmHg (mean 25 +/- 10 mmHg). Sub aortic obstruction surface assessed by 3DE ranged from 12 to 65% (mean 22 +/- 15%); distance between SAM and aortic leaflets ranged from 0.5 to 12 mm (mean 4 +/- 4 mm). Linear relation between Doppler gradient and 3D obstruction surface was found (r = 0.7). Attachment of the SAM to the mitral valve was present in 14 patients and confirmed by surgical view in 7 patients. Conclusion 3DE introduces new pre-operative description of the SAM. Such views could optimize the surgical indication and procedure. Download : Download full-size image

International audience; Objectives Risk markers of cardio-vascular events in children with Marfan syndrome remain little known. We assessed the prognostic value of aortic root z-score in patients with Marfan syndrome diagnosed during childhood. Methods From the French multicenter database, 457 patients with Marfan syndrome, diagnosed before 18 y.o., were prospectively included in this cohort study. Echocardiographic measurements of aortic root diameters were performed at each visit. We calculated the Z-score of aortic root measurements using the Bichat formula. Mean z-score was defined as the mean of the z-score (MeanZS15) calculated for each measurement before the age of 16. Results Median age at end of FU was 15.9years (interquartile 10.9-20.3). FU was complete for 69.5% of patients. Median FU was 4.6years. A cardiovascular event occurred in 17 patients (3.7%, prophylactic aortic surgery n=14, aortic dissection n=1 and deaths n=2). Survival free of cardiac events was 85.1% in patients with a meanZS15 of the Valsalva diameter

BackgroundRV systolic strain evolution during peri-operative management of congenital heart diseases (CHD) is unknown.MethodsIn this prospective study, RV peak systolic strain (PSS) was measured using 2D speckle tracking echocardiography (Qlab10.0 software, Philips) in 39 children undergoing surgery of a CHD (Median age: 17 months, min 6 day-old, max 14.3 year-old). Three measures were performed the day before surgery, few hours after the surgery and before discharge and compared to conventional echocardiographic parameters of RV and left ventricular (LV) function. The relationships between the evolution of RV-PSS, peri-operative parameters and the type of CHD were assessed.ResultsMean RV-PSS at baseline was –19.5±4.8. RV-PSS was moderately correlated with the heart rate (r=0.49), the LV Tmad (r=–0.48), the TAPSE (r=–0.54) and the tricuspid S’ wave (r= –0.44)(all p

Background 3D echocardiography is a validated method to evaluate left ventricular (LV) volumes and function. Because of its peculiar morphology, the right ventricle (RV) volumes can not be calculated with 3D softwares used for LV quantification. Recently, new 3D software adapted for RV morphology was introduced. Aim To evaluate the feasibility of 3D RV analysis in a pediatric population and to compare RV 3D data with LV 3D measurements. Methods Fifty-one pediatric patients (median age 10 years) with normal cardiac anatomy and function were included. 3D transthoracic echocardiography was performed with the X4-2 or X7-2 matrix probe (ie33, Philips Medical Systems, Andover, MA). Three LV and RV full volume sweeps were acquired from apical views. The volumes were analysed with the LV and the dedicated RV analysis softwares (TomTec Imaging Systems, Munich, Germany). Results Measurements feasibility was 100% for LV and 95% for RV. The RV had higher volumes but smaller ejection fraction compared to LV (RV end-diastolic volume 59 ml, LV end-diastolic volume 46 ml; RV end-systolic volume 29 ml, LV end-systolic volume 17 ml; RV ejection fraction 53%, LV ejection fraction 66%).The stroke volumes of the two ventricles were highly related with a mean difference of 1+/-2 ml (r= 0,95). The intra- and interobserver variabilities ranged from RV 3D volumes (0.4%-2%, and 0.2%-4%). Conclusion 3D echocardiography using dedicated software is a feasible and adequate method for RV volume quantification in a normal pediatric population. Further studies are needed to validate the accuracy of the method to calculate enlarged RV volumes in patients with congenital heart diseases. Download : Download full-size image

Background The bicuspid aortic valve (BAV) is the most common congenital heart defect. Children with BAV are more likely to have valve dysfunction and to require intervention during childhood. According to the subtype of BAV, the prognosis and the treatment may be different. Objective The aim of this study was to assess the accuracy of 3D echocardiography (3DE) in order to diagnose BAV and to depict accurately the leaflets morphology. Methods 40 consecutive children with suspicion of BAV were included in a prospective monocentric study. 2DE and 3DE views were recorded by the same investigator. Images were reviewed separately by two confirmed pediatric cardiologists in order to assess BAV. Then, we compared 2DE and 3DE for the description of the spatial position of cusps and raphes. The association with aortic aneurysms, aortic valve insufficiency or stenosis and other cardiac malformation were also reported. Results The median age was 4.8 years (±7.3). For 19 patients (47%), 3DE allows a better visualization of the leaflets morphology leading to a reclassification of the BAV. Raphes are better distinguished by 3D live (sensitivity = 100% [CI95%, 88–100], specificity = 100% [CI95%, 67–100]) or biplane echocardiography (sensitivity = 96% [CI95%, 80–99], specificity = 63% [CI95%, 35–85]) than 2DE (sensitivity = 86% [CI95%, 62–96], specificity = 28% [14–50]). Inter-observer variability is almost null. Conclusion 3D live and biplane echocardiography is a simple, rapid and reliable method for the diagnosis and the accurate description of BAV in children. This technique may be particularly helpful in order to precise the prognosis or to guide the surgeon. Download full-size image Bicuspid aortic valve assessed by 3DE