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To study the long-term outcome after surgery for pulmonary atresia and ventricular septal defect (PA-VSD), and to determine association between the contribution of major aorto-pulmonary collateral arteries (MAPCAs) to the pulmonary blood flow, comorbidity and cause of death. Patients who had undergone surgery for PA-VSD from January 1st 1994 to December 31st 2017 were studied retrospectively. Survival was cross-checked against the Swedish National Population Register. Seventy patients were identified, giving an incidence of 5.3 newborns p