Surgical management of the neonate with heterotaxy and long-term outcomes of heterotaxy.

A review of the many challenges facing the neonate with heterotaxy has identified total anomalous pulmonary venous connection, atrioventricular valve abnormalities, pulmonary atresia, and arrhythmias including heart block as particular risk factors for the child who will pursue a single-ventricle pathway. Experience varies widely between different centers as to the percentage of patients who are suitable for biventricular repair, ranging from less than 20% to greater than 50%. Biventricular repair may only require simple baffling of anomalous systemic or pulmonary veins or may involve complex intraventricular baffle repair of double-outlet right ventricle with common atrioventricular valve. The long-term complications of heterotaxy include accelerated development of pulmonary arteriovenous malformations after the Kawashima procedure, high mortality and morbidity for the Fontan procedure (although improving results have been reported more recently), and the development of late arrhythmias. Extracardiac problems include a high risk of volvulus if malrotation is present, suggesting the need for an elective Ladd procedure. The presence of associated ciliary dyskinesia appears to be associated with an increased risk of postoperative morbidity, particularly ventilator dependence and other respiratory complications. The child with heterotaxy faces many challenges that are often underappreciated by both caregivers and families.