Modified primary sutureless repair of total anomalous pulmonary venous connection in heterotaxy

Early and late mortality remain high after palliative open heart surgery in low-weight neonates. In addition, the need for neonatal repair and the presence of pulmonary venous obstruction are risk factors for mortality in right isomerism. We describe a modified primary sutureless repair of infracardiac total anomalous pulmonary venous connection in a 1,600 g neonate with heterotaxy. Endarterectomy of intimal hyperplasia localized to the anastomotic site was required at bilateral bidirectional cavopulmonary connection 6 months after the initial repair. The patient underwent a successful fenestrated Fontan procedure using an extracardiac conduit at the age of 2 years.