[Bronchopulmonary dysplasia-associated pulmonary arterial hypertension of very preterm infants].

Bronchopulmonary dysplasia (BPD) of very preterm infants is a multifactorial chronic lung disease and its incidence has not decreased despite improvements in neonatal intensive care, including lung protective strategies. Pulmonary hypertension (PH) can complicate the course of BPD. Mortality in infants with BPD-associated PH is thought to be very high, but its incidence is unknown and a standard diagnostic and therapeutic strategy has not been well defined.

Maternal transmission of interstitial 8p23.1 deletion detected during prenatal diagnosis.

We report on the first prenatally diagnosed interstitial 8p23.1 maternally inherited deletion. At 20 weeks of gestation (WG) the fetus was diagnosed with a complete atrioventricular canal.

Reduction of left ventricular hypertrophy in children undergoing hemodialysis.

Left ventricular hypertrophy (LVH) is related to a 1,000-fold increased risk of cardiovascular morbidity and mortality in young adults with end-stage renal disease (ESRD) treated with hemodialysis (HD) or peritoneal dialysis. We report a series of 17 children (5 girls, 12 boys), with a median (range) age of 11 (2-18) years, all treated by HD, who presented with an increased left ventricular mass (LVM) index of 54.8+/-4.

Burkholderia cepacia is associated with pulmonary hypertension and increased mortality among cystic fibrosis patients.

The aim of the study was to evaluate the impact of Burkholderia cepacia on cardiovascular status and mortality in cystic fibrosis. Seven patients infected with B. cepacia were matched with 31 patients not infected with this organism for gender, age, height, weight, genotype, and percent predicted forced expiratory volume in one second, partial arterial oxygen pressure, and pancreatic sufficiency status.

[Aneurism of the subclavian artery associated with Turner's syndrome].

Unlabelled: Arterial aneurysms associated with Turner's syndrome are rare.

Case Report: We report a case of aneurysm of the left subclavian artery in a 16-year-old girl with Turner's syndrome. This patient was operated on: resection of the aneurysm, suture of the aortic arch and reimplantation of the subclavian artery in the left common carotid were performed.

[Myocardiopathy and isolated glucocorticoid deficit with ACTH resistance: a fortuitous association?].

Unlabelled: Hereditary syndrome of unresponsiveness to ACTH is a rare autosomal recessive disorder characterized by an isolated glucocorticoid deficiency which is exceptionally associated to regressive cardiomyopathy.

Case Report: A male newborn had iterative episodes of hypoglycemia since the first hours of life. Acute bronchiolitis at the age of 14 days was associated with transitory dilated cardiomyopathy.

Value of radionuclide assessment with thallium 201 scintigraphy in carnitine deficiency cardiomyopathy.

The case of a 30 month-old boy who presented with isolated severe dilated cardiomyopathy is reported. The diagnosis of systemic carnitine deficiency was confirmed by low serum and tissue carnitine levels. During oral L-carnitine therapy, dramatic improvement of the cardiac function was assessed by radionuclide methods.

[A rare cause of neonatal ulcero-necrotizing enterocolitis: aortic coarctation syndrome].

The authors report a case of necrotizing enterocolitis which appeared in the first hours of life of a full-term neonate without signs of sepsis. This neonate presented with a severe hypoplasia of the horizontal aorta and very tight coarctation responsible for hepatic, renal and mesenteric ischemia. Reports of enterocolitis as a complication of congenital heart disease are rare and related most often to hypoplastic left heart than to coarctation of the aorta.

[Coronarocardiac fistula disclosed neonatally. Apropos of 2 cases].

Two cases of fistula between a coronary artery and the cardiac cavities (right coronary artery-right atrium, and right coronary artery-right ventricle) are reported. They were revealed by cardiac failure developed shortly after birth. The diagnosis was confirmed by two-dimensional echocardiography and angiography.

[Regressive hypertrophic myocardiopathy in an infant with Recklinghausen's neurofibromatosis].

A case of neonatal hypertrophic cardiomyopathy (HCM) without obstruction is reported. Von Recklinghausen neurofibromatosis in the mother and several relatives and the occurrence of café-au-lait spots in the child at 6 weeks of life led to the association of HCM with this phacomatosis. The spontaneous regression of the myocardial hypertrophy after 6 weeks makes this first report of neonatal HCM in the course of Von Recklinghausen neurofibromatosis peculiar.

[Outcome of infants operated on for aortic coarctation before 3 months of age].

Between January 1966 and June 1982, 177 infants less than 3 months of age underwent surgical cure of coarctation of the aorta. Seventy-one percent had associated cardiac lesions not including a patent ductus arteriosus. With the cure of the coarctation either by resection anastomosis or angioplasty, 45% underwent associated surgical procedure, mainly pulmonary artery banding.