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Summary: The value of prenatal diagnosis of major congenital heart diseases (CHDs) has already been proved. In this study, we observed the evolution of the detection rate of CHDs and the quality of the diagnoses over a 15-year period in the Indre-et-Loire department of France. Method: Retrospective analysis of the quantitative and qualitative data of prenatal diagnosis between 2000 and 2005 (period 3) and comparison with studies conducted from 1991 to 1994 (period 1) and from 1995 to 1999 (period 2). The CHDs considered to be detectable are the major CHDs with neonatal symptoms. In order to analyze the quality of prenatal diagnosis, each diagnosis was classified as correct, false, or incomplete during period 3. Results: Period 3: 65.7% detectable CHDs were screened. Using period 1 criteria for prenatal diagnosis, the percentage of CHDs detected for periods 1, 2, and 3 was 43.2, 66.7, and 78%, respectively. Between periods 2 and 3, the detection rate increased from 52 to 74% for CHDs diagnosed with the visualization of the outflow tract. It increased from 63 to 71% for CHDs diagnosed with a four-chamber view. During period 3, diagnoses made by a pediatric cardiologist were correct and complete in 80% of cases. It was less than 25% otherwise. Conclusion: The improvement of prenatal diagnosis over time results from technological progress, the greater experience of ultrasonographers, and the systematic visualization of the outflow tract in addition to the four-chamber view. Despite the improvement in prenatal diagnosis, one-third of major CHDs were not screened before birth. Pediatricians still need to diagnose CHDs after birth. [ABSTRACT FROM AUTHOR]

Résumé Nous rapportons l’observation d’un enfant de 7 ans atteint d’extrasystoles ventriculaires (ESV) nombreuses d’apparence bénigne et d’une dysfonction ventriculaire gauche. Le bilan cardiologique n’a pas mis en évidence d’anomalie structurale du cœur. Dix-huit mois après l’introduction d’un traitement anti-arythmique par bisoprolol à faible dose, l’enregistrement Holter a montré 70 000 ESV/24 h et la fonction ventriculaire gauche restait diminuée. Six mois après l’augmentation de la dose de bisoprolol, les ESV étaient moins fréquentes et la fonction ventriculaire gauche s’était complètement normalisée. Cette évolution est caractéristique d’une myocardiopathie rythmique induite par des ESV fréquentes. Summary We report on the rare case of 7-year-old child presenting with frequent and apparently benign premature ventricular complexes (VPC) and left ventricular dysfunction. No structural disease of the heart was seen at cardiological evaluation. Eighteen months after the introduction of an antiarrhythmic treatment with low-dose bisoprolol, Holter monitoring showed 70,000 VPC in 24 h with left ventricular function remaining decreased. Six months after increasing the dose of bisoprolol, the VPC were less frequent and left ventricular function was completely normalized. This case was characteristic of a cardiomyopathy induced by frequent premature ventricular contractions. [ABSTRACT FROM AUTHOR]

Objectifs: Évaluer le devenir des ESV idiopathiques de l’enfant. Patients et Méthodes: Enfants avec ESV vus entre 1984 et 2004. Paramètres étudiés: sexe, âge, symptômes, ESV par 24h: nombre, type ECG et gravité selon la classification de Lown (classes de 1 à 5). Protocole d’étude prospectif: consultation cardiologique, ECG, Holter. Deux groupes évolutifs constitués: A (absence d’ESV) et B (ESV persistantes). Résultats: Parmi les 74 pts recensés, 31 (10 adultes) ont participé. Les pts non revus étaient plus âgés (p = 0,021) et avaient plus d’ESV monomorphes (p = 0,032). Le recul médian était de 6,7 ans (4-19,5). Groupe A : 17 pts, Groupe B: 14 pts dont 11 avec ESV de classe 1 ou 2, et 3 avec ESV de classe 3 ou 4. Le sex-ratio était plus élevé dans le groupe A (p = 0,045). Les autres facteurs n’avaient pas d’influence sur l’évolution des ESV. Dans le groupe B, les symptômes étaient plus fréquents (p = 0,032), le nombre d’ESV par 24 h était < 1000 chez 8 pts, entre 1000 et 10000 chez 4 pts et > 10 000 chez 2 pts. Par rapport aux données initiales, la classe de gravité était plus faible (n = 5), identique (n = 7) ou augmentée (n = 2). Conclusions: La persistance chez quelques pts d’ESV nombreuses, source potentielle de dysfonction ventriculaire, justifie la surveillance des ESV idiopathiques. [ABSTRACT FROM AUTHOR]

Summary: Pompe disease is an autosomal recessive glycogen storage disorder caused by acid-α-glucosidase deficiency. The infantile form is usually fatal by 1year of age in the absence of specific therapy. We report the cardiac follow-up of a 4-month-old boy treated with enzyme replacement therapy (ERT) for 8months. The patient had no cardiac failure at the age of 1year. Before starting ERT, ECG showed a shortened PR interval, with huge QRS complexes and biventricular hypertrophy; echocardiography demonstrated major hypertrophic cardiomyopathy. The QRS voltage (SV1+RV6) decreased from 13 to 2.9mV after 32weeks of ERT, suggesting a progressive reduction of cardiac hypertrophy and intracellular glycogen excess. The PR interval increased from 60 to 90ms. A block of the right bundle branch appeared after 13weeks of treatment. The indexed left ventricular mass decreased from 240 to 90g/m2 after 30weeks of ERT. The left ventricular ejection fraction decreased transitorily between the 5th and the 15thweeks of treatment. In summary, ERT is an efficient therapeutic approach for the cardiomyopathy of infantile Pompe disease. However, the possible occurrence of a right bundle branch block and a transitory alteration in the ejection fraction highlight the importance of cardiac follow-up. [Copyright &y& Elsevier]

We report on the rare case of 7-year-old child presenting with frequent and apparently benign premature ventricular complexes (VPC) and left ventricular dysfunction. No structural disease of the heart was seen at cardiological evaluation. Eighteen months after the introduction of an antiarrhythmic treatment with low-dose bisoprolol, Holter monitoring showed 70,000 VPC in 24h with left ventricular function remaining decreased. Six months after increasing the dose of bisoprolol, the VPC were less frequent and left ventricular function was completely normalized. This case was characteristic of a cardiomyopathy induced by frequent premature ventricular contractions., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

Heart murmurs are common in children and adolescents. Although most are innocent, an isolated heart murmur in asymptomatic children may be the sole finding indicating serious heart disease. Historical elements of familial heart disease, cardiovascular symptoms and a well-conducted medical examination can identify children with an increased risk of heart disease. The distinction between an innocent heart murmur and a pathologic heart murmur is not always easy for primary care physicians because most of them have little experience with auscultation searching for congenital heart malformation. Echocardiography provides a definitive diagnosis of heart disease but is not required in case of innocent murmur. Inappropriate pediatric cardiologist and echocardiographic referral leads to useless and expensive examinations, resulting in a work overload for pediatric cardiologists. The objective of this review is to provide the keys to differentiate innocent and pathologic murmurs, and to help physicians decide on the optimal diagnostic strategy., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

Fetal intrapericardial teratomas are uncommon and usually benign. Their histology is the same as that of teratomas located elsewhere. They may cause death from non-immune hydrops fetalis and cardiac tamponade. We report a case that was successfully managed prenatally by placement of a pericardial amniotic shunt., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)