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Sudden death in pulmonary atresia and intact ventricular septum with right ventricular dependent coronary circulation is a well-established complication, and is thought to be caused by myocardial ischaemia. We report a case of paroxysmal complete atrioventricular block that raises the possibility of an additional mechanism of sudden death.

Paroxysmal complete atrioventricular block in a patient with pulmonary atresia and intact ventricular septum