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.

Position paper concerning the competence, performance and environment required for the practice of ablation in children and in congenital heart disease.

The population of patients with congenital heart disease (CHD) is continuously increasing, and a significant proportion of these patients will experience arrhythmias because of the underlying congenital heart defect itself or as a consequence of interventional or surgical treatment. Arrhythmias are a leading cause of mortality, morbidity and impaired quality of life in adults with CHD. Arrhythmias may also occur in children with or without CHD.

Radiofrequency ablation of right ventricular tachycardia in patients with no femoral access: safety and efficacy of a superior approach.

Aims: Ventricular tachycardia (VT) ablation has been proven to be effective and safe to avoid arrhythmia recurrences in patients with repaired congenital heart disease (CHD). However, some of these patients may present right ventricular (RV) access issues [agenesia or thrombosis of inferior vena cava (IVC)], making impossible to access the right ventricle through an inferior approach. In such patients, only a superior approach would theoretically be feasible.

Outcome of adults with Eisenmenger syndrome treated with drugs specific to pulmonary arterial hypertension: A French multicentre study.

Background: The relationship between pulmonary arterial hypertension-specific drug therapy (PAH-SDT) and mortality in Eisenmenger syndrome (ES) is controversial.

Aims: To investigate outcomes in patients with ES, and their relationship with PAH-SDT.

Methods: Retrospective, observational, nationwide, multicentre cohort study.

Pregnancy outcomes in patients with pulmonary arterial hypertension associated with congenital heart disease.

Objective: There is growing evidence that maternal mortality in pregnant women with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is lower than that in available data. In order to evaluate this hypothesis, we collected data of pregnancies in women with PAH-CHD.

Methods: Women with PAH-CHD followed in seven French referral centres were retrospectively included from 1997 to 2015.

Health-related quality of life of patients with pulmonary arterial hypertension associated with CHD: the multicentre cross-sectional ACHILLE study.

Background: The aim of this study was to assess health-related quality of life in patients with pulmonary arterial hypertension associated with CHD and correlations with clinical status.

Methods: This prospective cross-sectional observational study included CHD patients with pulmonary arterial hypertension in 14 tertiary-care centres in France. We used two health-related quality of life questionnaires - SF-36 and Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) - and one anxiety/depression Hospital Anxiety and Depression Scale (HADS) questionnaire.

Marfan Sartan: a randomized, double-blind, placebo-controlled trial.

Aims: To evaluate the benefit of adding Losartan to baseline therapy in patients with Marfan syndrome (MFS).

Methods And Results: A double-blind, randomized, multi-centre, placebo-controlled, add on trial comparing Losartan (50 mg when <50 kg, 100 mg otherwise) vs. placebo in patients with MFS according to Ghent criteria, age >10 years old, and receiving standard therapy.

Outcomes and safety of transcatheter pulmonary valve replacement in patients with large patched right ventricular outflow tracts.

Background: Although globally accepted, the indication for implantation of the Melody(®) (Medtronic Inc., Minneapolis, MN, USA) transcatheter pulmonary valve is limited to the treatment of haemodynamically dysfunctional right ventricular outflow tract (RVOT) with right ventricle to pulmonary artery (PA) obstruction. The use of the Melody valve for haemodynamically significant isolated pulmonary regurgitation has not been evaluated.

Acquired left ventricular submitral aneurysms in the course of Takayasu arteritis in a child.

A 9-year-old black African boy was hospitalized for heart failure revealing a severe left ventricular dysfunction associated with dilated cardiomyopathy, two submitral aneurysms, occlusion of the circumflex artery and a giant coronary artery aneurysm on the proximal left anterior descending artery. The boy was coinfected with human immunodeficiency virus and Mycobacterium tuberculosis. Though rare, association of Takayasu arteritis and submitral aneurysm leads to rethinking the pathogenesis of submitral aneurysm and suggests that some of them may be acquired.

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.

Transcatheter valve insertion in a model of enlarged right ventricular outflow tracts.

Objective: Transcatheter pulmonary valve insertion has recently emerged as an alternative to surgery. To extend its indications to patients with a large right ventricular outflow tract, we previously developed an intravascular device that reduces the diameter of the main pulmonary artery, allowing the insertion of available valved stents. Here we report its use in a model of animals with an enlarged right ventricular outflow tract and pulmonary valve incompetence.

Off-pump replacement of the pulmonary valve in large right ventricular outflow tracts: a transcatheter approach using an intravascular infundibulum reducer.

We report our experience of pulmonary valve replacement in animals with large right ventricular outflow tracts (RVOTs) using a percutaneous approach. We intended to implant a device to percutaneously reduce the diameter of the pulmonary artery (PA). Following its insertion, we intended to implant a valved stent inside the restriction.