[Severe hypoplastic left heart syndrome: palliative care after prenatal diagnosis]. / Hypoplasies sévères du ventricule gauche: soins palliatifs après un diagnostic prénatal.

We analyzed 16 cases of hypoplastic left heart syndrome (HLHS) submitted to the multidisciplinary center at Rennes Teaching Hospital from 2006 to 2010 for prenatal diagnosis. The information given to parents at the moment of choice is capital for them to make their own decision: in our team the real choice for parents stands between termination of pregnancy (TOP) and palliative care (PC). The Norwood procedure is rarely proposed to parents in France and it is performed in very few centers. Heart transplant is never proposed nor done at this age. The objectives of our study were to understand the reasons for the choice of PC, take stock of our experience of PC, and relate the benefits but also the disadvantages of PC. Over the 16 patients whose fetus had HLHS, 9 requested TOP, while 7 others wanted to live their pregnancy and meet their child at birth, therefore requesting neonatal PC. No family asked for the Norwood procedure. Four children died within the first days (D1, D2, D4, D9), 2 others died at 5 and 7 months, 1 child was operated on for coarctation of the aorta (unknown before birth) and is still alive 1.5 years later. Maternal motivations to continue the pregnancy were clearly described for 2 of the 7 cases: religious prohibition of TOP in 1 case, negative experiences of previous abortions in the second case. In another case, the parents hesitated between PC and Norwood surgery. For the other women, the reasons were less clearly expressed. In our series, HLHS is the first indication for PC from prenatal diagnosis (7/16 cases in the same period) while in the literature, heart diseases are the second cause of TOP after the neurological causes. The overrepresentation of this pathology in the families who opt for PC may be due to the unconscious image that both professionals and families have of HLHS: severity of an inevitably fatal disease, rapid postnatal death, and no suffering. Our study may change this view: a child was in fact carrying a curable defect which was discovered 12 days after birth, 2 children died at 5 and 7 months, and 5 of 6 children had major analgesics at the end of life. Nevertheless, the families were supported and followed by the PC network, except 1 who ruptured all contacts in a context of presumed intense suffering, but the other 6 do not regret their choice despite the difficulties.

[Percutaneous closure of veno-venous fistulae after total cavopulmonary derivation: a case report]. / Fermeture percutanée de fistules veino-veineuses après dérivation cavo-pulmonaire totale: à propos d'un cas.

The authors report the case of a boy with a complex congenital heart malformation with a dextro-isomerism or Ivemark's syndrome. After several palliative procedures, a total cavopulmonary derivation was performed at the age of 11 years with implantation of a fenestrated tube between the inferior vena cava and the right pulmonary artery. The fenestration was closed two years later. At about 15 years of age, progressive systemic desaturation was observed; cardiac catheterisation with angiography showed a large number of intra-hepatic veno-venous collaterals draining into a large left suprahepatic vein which drained into the right atrium connected to the systemic ventricle. The largest fistula was embolised with a 20 mm Amplatzer Duct Occluder and two others with 14 x 12 and 10 x 8 Amplatzer Duct Occluders. The saturation rose from 75% to 94%. After 6 months' follow-up, the child is asymptomatic and the systemic saturation is 90%.