Giant Localized Atheroma Mimicking a Coarctation of the Aorta: A Rare Entity to Consider.

We describe the case of a patient who developed resistant hypertension due to a giant atheroma with acquired physiologic mimic of coarctation of the aorta. This presentation illustrates an extremely rare etiology to consider in adults in whom aortic isthmus stenosis remains often of congenital origin. ().

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.

Cardiovascular events in perimembranous ventricular septal defect with left ventricular volume overload: a French prospective cohort study (FRANCISCO).

Unlabelled: The long-term prospective multi-centre nationwide (French) observational study FRANCISCO will provide new information on perimembranous ventricular septal defect with left ventricular overload but no pulmonary hypertension in children older than 1 year. Outcomes will be compared according to treatment strategy (watchful waiting, surgical closure, or percutaneous closure) and anatomic features of the defect. The results are expected to provide additional guidance about the optimal treatment of this specific population, which is unclear at present.

Patent foramen ovale closure in children without cardiopathy: Child-PFO study.

Background: Closure of patent foramen ovale is well-managed in adults, but is performed less frequently in children.

Aim: To analyse all patent foramen ovale closures performed in the past 20 years in French paediatric centres.

Methods: Retrospective study of patent foramen ovale closures in children without cardiopathy in nine centres between 2000 and 2019.

INTU-related oral-facial-digital syndrome type VI: A confirmatory report.

Oral-facial-digital (OFD) syndromes are a subgroup of ciliopathies distinguished by the co-occurrence of hamartomas and/or multiple frenula of the oral region and digital anomalies. Several clinical forms of OFD syndromes are distinguished by their associated anomalies and/or inheritance patterns, and at least 20 genetic types of OFD syndromes have been delineated. We describe here a child with preaxial and postaxial polydactyly, lingual hamartoma, a congenital heart defect, delayed development and cerebellar peduncles displaying the molar tooth sign.

Isolated severe leftward displacement of the septum primum: anatomic and 3D echocardiographic findings and surgical repair.

Objectives: Leftward displacement of the septum primum is usually described as associated with hypoplastic left heart syndrome or visceral heterotaxy. This rare malformation results in partially or totally anomalous pulmonary venous drainage with a normal connection of the pulmonary veins to the left atrium, depending on the degree of septal shift. We report the 3D echocardiographic and anatomic findings as well as the surgical repair in a series of isolated severe leftward displacement of the septum primum, responsible for totally anomalous pulmonary venous drainage.

Nontoxinogenic corynebacterium diphtheriae as a rare cause of native endocarditis in childhood.

We report a pediatric case of negative blood culture pulmonary valve endocarditis caused by a nontoxinogenic Corynebacterium diphtheriae biotype gravis and review the literature on this disease.

Acute ischemic cardiomyopathy after extreme emotional stress in a child.

Ischemic cardiomyopathy is rare in children. It is usually caused by congenital anomalies of coronary arteries, coronary anomalies after coronary artery transfer, or Kawasaki disease. In recent years, a new cardiac syndrome-named "Tako-Tsubo cardiomyopathy" for the particular shape of the end systolic ventricle-has been described in adults.

[Complications of prostaglandin E1 treatment of congenital heart disease in paediatric medical intensive care].

The authors undertook a retrospective study of the modes of prescription, the tolerance and efficacy of prostaglandin E1 in 62 consecutive neonates with congenital heart disease (average Age 1.6 days: 35 boys: weight: 3.1 +/- 0.

[Paediatric cardiac surgery and autoevaluation: risk score, complexity score and graphic analysis].

The creation of a paediatric surgical unit requires autoevaluation in order to: assess the quality of the results with respect to recognised international standards, answer the family's questions about the results obtained and adhere to criteria of accreditation Between January 2003 and December 2004, 201 consecutive patients, children (N= 164) or operated for adult congenital heart disease (N= 37) were treated. No patient was excluded. The RACHS-1 risk score, the ARISTOTLE scores of complexity and performance and the CUSUM and VLAD graphic analyses were applied to the study of hospital mortality.

[Feasibility and significance of a transoesophageal electrophysiological investigation in children and adolescents with Wolff-Parkinson-White syndrome].

Unlabelled: An electrophysiological investigation is the most reliable means of detecting malignant forms of Wolff-Parkinson-White syndrome (WPW). However, an endocavity investigation is an invasive procedure, especially in young subjects with few symptoms. The aim of this study was to examine the feasibility and results of an electrophysiological study performed by the transoesophageal route in children with WPW.

[Grave's disease in children with 22q11 deletion. Report of three cases].

Hypothyroidism is a well recognized complication of 22q11.2 deletion syndrome. Auto-immune hyperthyroidism is less common.

[Late presentation of type A aortic arch blockage with a large type I aortopulmonary window].

We report the case of a 39 day old infant, hospitalised for congenital cardiopathy associated with type A blockage of the aortic arch with a large type I aortopulmonary window. The infant was in cardiogenic shock with pulmonary systemic hypertension and a tightly stenosed arterial canal (< 2 mm). With no possibility of re-opening the arterial canal under PGE1 at this stage, complete repair was performed as an emergency.

[Kawasaki disease in newborns and infants: refractory forms to immunoglobulin therapy].

Unlabelled: We studied 52 consecutive patients with Kawasaki disease hospitalized (1984 -2003) during the acute phase (mean age 2.5 + 2.4 years; range 0.

Value of programmed ventricular stimulation after tetralogy of fallot repair: a multicenter study.

Background: Studies have consistently shown that ventricular tachycardia (VT) and sudden cardiac death (SCD) complicate the long-term outcome after tetralogy of Fallot repair, yet the diagnostic and predictive value of electrophysiological testing in this population is uncertain.

Methods And Results: A multicenter cohort of 252 patients with repaired tetralogy of Fallot undergoing programmed ventricular stimulation was followed up for 18.5+/-9.

Evidence of early impairments in both right and left ventricular inotropic reserves in children with Duchenne's muscular dystrophy.

In Duchenne's muscular dystrophy (DMD), cardiac function deteriorates with time and heart failure is one of the major causes of death. The aim of the study was to determine if a decrease in the ventricular inotropic reserves could be an early sign of cardiac dysfunction in these children. Nineteen children with DMD (aged 9 to 18 years, mean age 13.

[Cardiac stimulation in the infant and young child].

This study evaluates the problems and the evolution of cardiac stimulation in infants (aged < 3.5 years) by comparing the endocavity and epicardial routes in a retrospective series of 37 patients. Thirty seven patients aged 1.

[Isolated congenital complete atrio-ventricular block].

The objective of this work was to study the long term evolution of a retrospective series of 54 patients affected with congenital isolated complete atrio-ventricular block (CAVB) and to analyse the value of the different methods used for surveillance. Our series included 54 patients affected with isolated CAVB, without associated cardiopathy, diagnosed at an average age of 5.3 +/- 5.

[Junctional tachycardia in adolescents: nodal reentry is the most frequent cause].

Unlabelled: Ventricular preexcitation syndromes are classically more common in the pediatric age group than in adults, and a latent Kent bundle may explain most cases of paroxysmal junctional tachycardia (PJT). These data stem from the results of intracardiac electrophysiologic testing, which is performed only in those patients at the most severe end of the symptom spectrum. The recent introduction of transesophageal testing has expanded the indications of electrophysiologic testing for PJT.

[Role of pediatric cardiologists in the management of neonates with congenital heart disease].

Whether diagnosed before birth or suspected in a newborn, congenital heart diseases rapidly require an evaluation by the pediatric cardiologist. The role of the pediatric cardiologist is threefold: 1) to realize a careful anatomical examination using color Doppler echocardiography; 2) in many cases to perform a palliative or curative therapeutic act based upon interventional catheterization, such as Rashkind atrioseptotomy or percutaneous balloon valvuloplasty; and 3) whenever necessary, to take a rapid decision of cardiac surgery in concert with the cardiac surgeon, and to prepare the child for surgery.