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Eisenmenger syndrome is the last-stage of congenital heart diseases with untreated left to right shunt. Tolerance and prognosis are better than with idiopathic pulmonary hypertension. However, patients follow-up is often performed in inappropriate non-specialized care centres which makes the collection of epidemiological data difficult. Consequently, population registries are necessary and would increase knowledge of natural history. Finally, complications are often multiple and unexpected with lack of specialized management. Such patients require expert supervision with a multidisciplinary approach in experienced and specialized centres.

Palliative treatment of the hypoplastic left heart syndrome is rarely practiced in France because of the poor prognosis of the first surgical stage of the Norwood procedure. The recent modification proposed by Sano seems to give more promising results. The authors report their experience with the Norwood-Sano procedure compared with the classical Norwood procedure in 13 neonates with hypoplasia of the left heart. From 1999 to 2005, 8 patients underwent the classical Norwood procedure (1999-2001) and 5 had the Sano modification (2002-2005). During the preoperative period, there was 1 case of a very restrictive interatrial septum and 5 patients required mechanical ventilation. The patients were operated at an average age of 8.5 +/- 12 days. The results showed that survival after the first surgical stage was significantly better with the Sano modification (12.5% versus 80%; p= 0.03). All patients who survived the first stage went on to the second stage with a bi-directional right superior cavo-pulmonary derivation at an average of 6.1 +/- 2.4 months. Despite the infundibulotomy of the Sano modification, no arrhythmia or right ventricular dysfunction was observed after an average follow-up of 21.7 +/- 7.6 months. The authors conclude that the Sano modification improved survival of patients with hypoplasia of the left heart after the first palliative surgical procedure. The long-term follow-up of patients operated by this technique should particularly look out for arrhythmias and right ventricular dysfunction.