Incidence, risk factors, and mortality of neonatal and late-onset dilated cardiomyopathy associated with cardiac neonatal lupus.

Background: Dilated cardiomyopathy (DCM), a well-known complication of cardiac neonatal lupus, is associated with high mortality rate. Its risk factors remain unclear.

Methods: We analyzed occurrence of postnatal DCM among children with high-degree congenital heart block (CHB) and mothers with anti-SSA and/or anti-SSB antibodies.

Description of 214 cases of autoimmune congenital heart block: Results of the French neonatal lupus syndrome.

Background: Cardiac neonatal lupus syndrome is due to anti-SSA or SSB antibodies and mainly includes congenital heart block (CHB) and dilated cardiomyopathy (DCM). Its optimal management is still debated. We report a large series of autoimmune high degree CHB.

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.

Maternal and fetal outcomes of pregnancy with Fontan circulation: A multicentric observational study.

Background: Despite serious long-term sequel, women with Fontan palliation have reached childbearing age. However there is paucity of data on the pregnancy outcomes and management of this condition. We aimed to determine the maternal and fetal outcomes of pregnancy in women with Fontan palliation.

Transcatheter tricuspid valve implantation: a multicentre French study.

Background: Transcatheter valve-in-valve (VIV) implantation in failing bioprosthesis is an emerging field in cardiology.

Aim: To report on a French multicentre experience and a literature review of tricuspid VIV implantation.

Methods: We approached different institutions and collected 10 unpublished cases; a literature review identified 71 patients, including our 10 cases.

Significant persistent ductus arteriosus in infants less or equal to 6 kg: percutaneous closure or surgery?

Background: Percutaneous closure of large persistent ductus arteriosus using the Amplatzer duct occluder is an alternative to surgery. However, this device is not recommended in infants weighing less than 6 kg.

Aim: To evaluate the safety and effectiveness of this procedure in low-body-weight infants.

Elucidation of penetrance variability of a ZIC3 mutation in a family with complex heart defects and functional analysis of ZIC3 mutations in the first zinc finger domain.

We studied a series of 42 cases of transposition of the great arteries (TGA), a complex heart defect (CHD) that is two times more prevalent in males than in females. A mutation in the X chromosome at the ZIC3 gene was found in two affected siblings (one male, one female) and their unaffected mother. A second factor, skewed X-inactivation pattern explained the discrepancy between the daughter/mother phenotype.

Non-invasive detection of coronary artery disease by dobutamine-stress echocardiography in children after heart transplantation.

Background: Coronary vasculopathy is the main cause of cardiac graft failure. Because yearly coronary angiography is invasive in children, a non-invasive method for detecting graft vasculopathy is needed. The aim of this study was to test dobutamine-stress echocardiography in a pediatric population to determine its feasibility, safety and reliability in the detection of graft coronary artery disease.

Rabbit antithymocyte globulin as induction immunotherapy in pediatric heart transplantation.

Background: There is little published data on the use of antithymocyte globulins in children. This retrospective study describes the use of Thymoglobulin (Imtix, SangStat, Lyon, France) in pediatric cardiac transplantation over a 13-year period in a single center that adjusted the dose of Thymoglobulin according to platelet count monitoring and examines the short-term hematological effects as well as longer-term outcomes.

Methods: Data for all children who received a heart transplant at the Hôpital Cardiologique at Lyon from 1984 to 2001 and who were given Thymoglobulin as part of their immunosuppressive protocol were extracted.