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Methods: We approached different institutions and collected 10 unpublished cases; a literature review identified 71 patients, including our 10 cases.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

[Intravenous thrombolysis in pediatric arterial ischemic stroke: a case report and a review of the literature].

Pediatric arterial ischemic stroke (AIS) is little known by pediatricians, as it is rare and difficult to diagnose. Current therapeutic approaches include platelet aggregation inhibitors, anticoagulation using heparin or thrombolysis with rt-PA, and therapeutic abstention in the absence of consensus, in contrast with stroke in adults. We report the case of a 3-year-old child who benefited from intravenous thrombolysis with rt-PA 2h after a major AIS of the anterior and middle left arteries due to embolism from the heart.

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.

Current patterns of infective endocarditis in congenital heart disease.

Objective: To assess the changing profile of infective endocarditis in patients with congenital heart disease.

Methods: All cases diagnosed from 1966 to 2001 (revised Duke criteria) were retrospectively reviewed and categorised in periods I (< 1990) and II (>or= 1990).

Results: 153 episodes occurred, 81 in period I and 72 in period II.

[Characteristics of infectious endocarditis in ventricular septal defects in children and adults].

The aim of this retrospective study was to analyse cases of infectious endocarditis (IE) of native or repaired ventricular septal defects (VSD) to determine its incidence, the circumstances of its occurrences, the outcome and prognosis of this complication. From 1966 to 2002, 36 IE occurred in 19 boys and 17 girls: the age at diagnosis was 13.4 +/- 11.

Primary cardiac Burkitt lymphoma in a child.

Primary cardiac lymphoma (PCL) is a rare and usually fatal malignancy, seldom reported in children. This report describes the case of a 10-year-old boy who presented with multiple intracardiac masses which, when biopsied, proved to be small non-cleaved cell (Burkitt's) lymphoma. The first two cycles of chemotherapy according to the LMB 96 protocol were given under close cardiological supervision, with good response.

[Genetics and congenital heart diseases].

We describe in this article the recent data on the genetics of congenital heart defects (CHD) organised by type of CHD although each predisposing genetic factor is associated with a whole variety of CHD types. The recent progress resulting from animal models, molecular cytogenetics and CHD familial cases studies allow a better understanding of the determinism of CHD. This lead in term to improved counselling of parents of affected children and of CHD adults who would like to become parents.

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.

[Pediatric cardiac transplantation. Long-term results and problems].

The aim of this study was to establish an assessment for cardiac transplantation by retrospective analysis of data from two French centres. Screening for acute rejection was ensured by systematic myocardial biopsies for patients aged over 10 years, and by clinical non-invasive follow up, ECG and Doppler echo in the younger ones. Coronaropathy was screened for by coronarography or stress echography.

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.

[Variation in cholesterol, lipoproteins and triglycerides after heart transplantation in children].

Total cholesterol, HDL and LDL-cholesterol and triglyceride levels may contribute to the development or progression of coronary artery disease of the transplanted heart. The aim of this retrospective study was to determine the short and long-term lipid profiles of transplanted children and to identify factors influencing these dyslipidemias. Twenty-three patients aged 9.

[Dobutamine echocardiography in children after heart transplantation].

Coronary disease of the transplanted heart is the principal cause limiting long-term survival of patients and grafts. In view of the invasive nature of coronary angiography, dobutamine echocardiography has been proposed as a non-invasive diagnostic method for this disease. The aim of this study was to determine the feasibility and reliability of this investigation in transplanted children.

The left retro-aortic brachiocephalic vein: morphologic data and diagnostic ultrasound in 27 cases.

A left retro-aortic brachiocephalic vein is a rare anatomic entity. A retrospective study was made of 5218 congenital cardiopathies treated between 1982 and 1998 in a medico-surgical department of paediatric cardiology. A left retro-aortic brachiocephalic vein was demonstrated in 27 patients, i.

[The Ross procedure in the acute phase of infectious endocarditis in childhood].

The Ross procedure of aortic valve replacement with a pulmonary autograft has several advantages in childhood over mechanical prostheses or homografts, especially in infectious endocarditis requiring early surgery. Between January 1997 and July 1998, 3 children with no known previous cardiac disease, aged 14 months, 10 and 11 years, had aortic valve infectious endocarditis. The causal organism was not identified in 1 case and the other two were due to staphylococcus aureus and corynebacterium diphteriae.

Long-term clinical and hemodynamic evaluation of porcine valved conduits implanted from the right ventricle to the pulmonary artery.

Objective: This retrospective study was initiated to evaluate the long-term results of valved prosthetic conduits implanted in the right ventricular outflow tract in patients with complex ventricular-pulmonary discontinuity.

Methods: A cohort of 103 patients out of 127 (24 early deaths, 19%) operated on between 1973 and 1996 with porcine valved conduits was available for evaluation, with a follow-up ranging from 1 to 21.6 years (mean follow-up 8.

[Prevention of infective endocarditis in the child. Current status and protocols].

Infective endocarditis remains a severe, potentially lethal disease, which justifies a rigorous prevention schedule. Children with cyanotic congenital heart disease, mitroaortic valvulopathies, prosthetic valve and uncorrected ventricular septal defect are the most susceptible. Dental care is the main cause of bacterial graft, followed by upper respiratory tract and cutaneous infections.