Staged biventricular repair for persistent truncus arteriosus with aortic arch obstruction following bilateral...

Persistent truncus arteriosus associated with aortic arch obstruction (Van Praagh type A4) is a rare combination with an extremely high rate of mortality. Our current approach is staged biventricular repair following bilateral pulmonary artery banding (bPAB). The patient in case 1 underwent bPAB at five days old, then biventricular repair was performed 10 days later because duct became restrictive despite prostaglandin administration. In case 2, the patient underwent bPAB via a left side thoracotomy at eight days old concomitant with an aortic arch repair using an extended direct anastomosis. Complete biventricular repair was delayed until eight months after the bPAB due to respiratory issues, then both banded branch pulmonary arteries were augmented by auto-pericardial patches at that time. A catheter examination three years after the complete biventricular repair showed that both branch pulmonary arteries were widely open. The patient in case 3 underwent emergency bPAB and truncal valve repair with a cardiopulmonary bypass at three days old for severe truncal regurgitation. Banding tapes on both sides were tightened two days after the operation. Although the hemodynamic condition was stable and truncal regurgitation was reduced, the patient died one month later from sudden septic shock.