Long-term outcomes of single-ventricle palliation for pulmonary atresia with intact ventricular septum: Fontan survivors remain at risk of late myocardial ischaemia and death

Abstract

OBJECTIVES: The specific outcomes of patients with pulmonary atresia with intact ventricular septum late after Fontan palliation are unknown. Patients with smaller right ventricles and myocardial sinusoids are known to have worse survival in the first years of life. Whether the potential for coronary ischaemia affects the long-term outcomes of these patients after Fontan palliation is still unknown. METHODS: All patients with pulmonary atresia with intact ventricular septum who underwent the Fontan procedure from 1984 to 2016 in Australia and New Zealand were identified, and preoperative, perioperative and follow-up data were collected. RESULTS: Late follow-up data were available for 120 pat..