Mitral annular disjunction and Pickelhaube sign in children with mitral valve prolapse: A prospective cohort study.

BACKGROUND: Mitral annular disjunction (MAD) and the Pickelhaube sign are identified as risk factors for malignant ventricular arrhythmias (VAs) and sudden cardiac death in adults with mitral valve prolapse (MVP); their prevalence and consequences in children have never been studied. OBJECTIVES: To determine the proportion of MAD in children with MVP, and its potential link with VAs. METHODS: A cohort of 49 consecutive children (mean age 12.8±3.0 years; 33 females) with MVP and comprehensive clinical arrhythmia (24-hour monitoring) and Doppler echocardiographic characterization, including pulsed-wave tissue Doppler (PWTD) of the lateral mitral annulus, was identified. The relationship between clinical and echocardiographic data and presence of VAs was studied. RESULTS: MAD was common (n=25; 51%). Only five patients had significant VAs (Lown grade>2) characterized by polymorphic premature ventricular contractions or couplets. MAD was not associated with VAs on 24-hour Holter monitoring, but an association was found between VAs and spiked high-velocity midsystolic signal>16cm/s on PWTD (Pickelhaube sign) (P=0.004), myxomatous mitral valve (P=0.004) and left ventricular dilatation (P=0.01). T-wave inversion in inferolateral leads on electrocardiogram was more frequent in patients with versus without the Pickelhaube sign (P=0.03). No difference was found between patients with or without MAD regarding sex, history of palpitation, severity of mitral regurgitation, aortic root diameter and incidence of connective tissue disorders. Myocardial fibrosis was detected in two of three patients who underwent a complementary cardiac magnetic resonance examination. CONCLUSIONS: MAD is common in children with MVP; its presence was not associated with significant VAs on 24-hour Holter monitoring, but the Pickelhaube sign and presence of myxomatous mitral valve may help to detect patients prone to significant VAs. Myocardial fibrosis can be detected by cardiac magnetic resonance in children with significant VAs.

Prevalence and Associated Factors of Long-term Growth Failure in Infants with Congenital Heart Disease Who Underwent Cardiac Surgery Before the Age of One.

Long-term growth failure can have negative impact on health (by increasing morbidity and mortality) and on neurodevelopmental outcomes. Its prevalence among children with congenital heart disease (CHD) is not well described. The aim of our study was to evaluate the prevalence of growth failure in a population of infants with CHD away from cardiac surgery and identify associated factors. We conducted a retrospective and multicentric study that included infants from the North of France who underwent cardiac surgery before the age of one, between January 2013 and December 2017. 331 infants were included among which 48% had a prenatal diagnosis, 15% had a genetic syndrome, and 15% were premature infants. Mean birth weight was 3 ± 0.6 kg. At surgery, 35% presented feeding difficulties (need for enriched formula and/or feeding tube) and 14% had growth failure (defined by Z-score weight for age < -2SD). 6-12 months after surgery, 16% still presented growth failure. Several associated factors were identified: prenatal diagnosis, genetic syndrome association, birth weight ≤ 3 kg, complex CHD (≥ 2 significative lesions, or double outlet right ventricle or single ventricle physiology), surgery after 30 days, and need for diuretic drug before surgery and/or still needed 1 month after surgery. Growth failure persists between 6 and 12 months after surgery in 16% of infants with CHD. More studies are needed to link growth failure and neurodevelopment, which is the new challenge for this aging population.

Screening for neurodevelopmental disorders in children with congenital heart disease.

The aim of this study was to evaluate the frequency of neurodevelopmental disorders (NDD) in children with significant congenital heart disease (CHD) and to determine associated factors to NDD and frequency of follow-up in developmental therapies. Two hundred and ten children with significant CHD aged from 6 to 66 months were enrolled over a period of six months. The Ages & Stages Questionnaire Third Edition in French (ASQ-3) was used to assess neurodevelopmental domains. NDD were defined if cut-off scores were ≤ - 1SD. - 1SD corresponded to "Monitor" range: children with minor or emerging disorders; - 2SD corresponded to "Refer" range: children exhibiting neurodevelopmental delays. Forty children were in "Monitor" range and 86 in "Refer" range. NDD rate was 60.0% (n = 126, 95% CI, 53.4 to 66.6%). There was no difference regarding CHD severity (p = 0.99). Only the presence of non-cardiac disease (OR = 2.14; 95% CI, 1.11 to 4.20) was associated with NDD. Forty-six children with NDD had no developmental follow-up (among them 21 were in "Refer" range (10%)) despite this being available.Conclusion: Children with significant CHD are at risk for NDD regardless of CHD severity. Systematic and early monitoring in a specific care program is required. Barriers that prevent access of care must be identified.Trial registration: Neurodevelopmental Disorders in Children With Congenital Heart Disease. NeuroDis-CHD. NCT03360370. https://clinicaltrials.gov/ct2/show/NCT03360370 What is Known: • Children with CHD are at risk for neurodevelopmental disorders and behavioural problems impacting their social adaptation, academic achievements and quality of personal and family life even in adulthood. What is New: • Children with CHD are at risk for neurodevelopmental disorders regardless of the complexity of the CHD. • Even with the availability of appropriate developmental services, children with CHD are not correctly followed, highlighting the need of a specific program of care for a better outcome. Local barriers that prevent access of care of those children must be identified.