Between 1994 and 2011, we performed 133 Fontan conversions with arrhythmia surgery. Most patients had tricuspid atresia or double-inlet left ventricle with prior atriopulmonary connection. Operative mortality was 1.5%, and mean length of stay was 14 days. A total of eight patients (6%) have had late cardiac transplantation. Freedom from arrhythmia recurrence is 85% at 10 years. For properly selected patients with a functionally univentricular heart who have had an atriopulmonary Fontan procedure, Fontan conversion with arrhythmia surgery significantly improves quality of life.

We report here the case of a 27-year-old woman with Noonan syndrome presenting with ventricular fibrillation. After successful defibrillation, echocardiography revealed hypertrophic cardiomyopathy associated with left ventricular outflow tract obstruction. Normal echocardiographic cardiac structure and function were reported 11 years ago. This case emphasises the importance of regular follow-up in patients with congenital disorders in which cardiac manifestations might develop in early adulthood or later.

Congenital venolobular or scimitar syndrome is a rare congenital cardiopulmonary anomaly consisting in a partial anomalous pulmonary venous drainage, lung hypoplasia, and anomalous systemic arterial supply to the lung. It can associate with other congenital disorders which will confer the clinical presentation and prognosis of these patients. In most of the cases, the therapeutic approach is partial, as anatomy allows only aberrant arterial embolisation. We present a 6-year-old girl with recurrent pulmonary infections, diagnosed as scimitar syndrome with double collector drainage to the inferior caval vein and left atrium, undergoing interventional catheterisation for complete correction of her disorder. The anomalous systemic artery supply was embolised and the anomalous venous drainage was occluded. The patient was asymptomatic during follow-up, which supports the interventional catheterisation approach as a valid therapeutic option in cases of scimitar syndrome with double venous drainage.

Pentalogy of Cantrell is characterised by a combination of severe defects in the middle of the chest including the sternum, diaphragm, heart, and abdominal wall. Mortality rate after cardiac surgery is usually high. We report a successful total correction of the cardiac defects in a case of Pentalogy of Cantrell with a double-outlet right ventricle prior to abdominal wall defect repair.

We report a case of two brothers with Marfan syndrome and coexistent anomalous aortic origin of a coronary artery from the wrong sinus of Valsalva. To our knowledge, this is the first ever report of siblings with Marfan syndrome who also have anomalous aortic origin of a coronary artery. The treatment and management options in the context of sudden death risk are discussed.