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Coronary artery fistulae (CAF) are rare forms of congenital heart disease with an incidence of one in 50 000 live births. The authors present the case of an asymptomatic neonate with a precordial murmur. Pre and postductal saturations, blood pressure and ECG were normal. Echocardiography revealed a large right coronary artery fistula to the right ventricle (4.5 mm). At 11 months, transcatheter occlusion of the fistula with a vascular plug was performed. A year on, the child was thriving, ECG and echocardiogram remained normal. CAF complications and symptoms (including aneurysm, myocardial ischaemia, angina, heart failure and dyspnoea) are commoner in older patients, so traditionally we intervene early. With increasing case reports of spontaneous closure of even large and symptomatic fistulae, management of especially asymptomatic children is unclear. Long-term complications of intervention also remain largely unknown. As such more information is required on the conditions natural history to better manage patients and counsel parents. [ABSTRACT FROM AUTHOR]

Currently, a three-stage surgical palliation remains the treatment of choice at Birmingham Children's Hospital. After initial introduction of the classical Norwood with pulmonary blood flow provided by a modified Blalock-Taussig shunt, a right ventricular to right pulmonary artery conduit at stage 1 Norwood palliation is now used in most cases, a bi-directional 'Glenn' shunt at second stage and an extra-cardiac Fontan completion at third stage. Mortality and morbidity has improved after modification of the technique. Thirty-day mortality was 32.4% (79/244) for the 'classical' Norwood procedure, 25.0% (7/28) for the left-sided RV-PA conduit and 12.7% (22/173) for the right-sided RV-PA conduit. Interstage mortality was 8.6% (21/244) for the 'classical' Norwood procedure, 14.3% (4/28) for the left and 10.1% (15/148) for right-sided RV-PA conduit. After stage II, 30-day mortality was 3.0% (10/335) for all groups. Stage III 30-day mortality was 0.9% (1/115) for all groups.