We describe two cases with pulmonary atresia, intact ventricular septum (PA/IVS), and right ventricle-dependent coronary circulation, who suffered from myocardial ischemic symptoms. Case 1 : A female infant diagnosed with PA/IVS during the fetal period was born after 40 weeks of gestation, weighing 3,078 g. Aortography demonstrated that the right coronary artery (RCA) was interrupted at the proximal segment, and the left coronary artery (LCA) did not show any stenosis. Right ventriculogram revealed sinusoidal connections filling the distal part of the right coronary artery and fistulous communications to the left anterior descending artery. Temporary ST segmental depression in ECG during milk feeding or crying improved spontaneously with time. Bidirectional Glenn shunt (BDG) was performed at 3 months old, and she underwent Fontan repair at 4 years old. She is doing well with no evidence of myocardial ischemic change. Case 2 : A female infant was born after 39 weeks of gestation, weighing 3,062 g. Aortography demonstrated that the RCA was interrupted at the middle segment, and the distal area depended on the right ventricle. LCA ostium at the ascending aorta was absent. Multiple sinusoidal connections were seen in the right ventricular myocardium with filling of the LCAs but no reflux into the aortic sinuses. After ventricular tachycardia occurred at 2 months old, left ventricular function deteriorated. BDG with ligation of the arterial duct was performed at age 3 months. The arterial blood pressure was 76/53 (62) mmHg and pressure in SVC was 21 mmHg immediately after the operation. However, arterial oxygen saturation, right atrium venous oxygen saturation and left ventricular ejection fraction were 50%, 16%, and 10%, respectively. Although extracorporeal membrane oxygenation was established for circulatory and myocardial oxygenation support on the postoperative day 2, her left ventricular function did not recover and she died on postoperative day 5. It is difficult to rescue such a case in which almost all coronary circulation depends on the right ventricle filled with desaturated blood.

We report two surgical cases with corrected transposition of the great arteries associated with ventricular septal defect and pulmonary atresia undergoing total correction including reconstruction of the central pulmonary artery after reconstruction of the left pulmonary artery for non-confluent pulmonary arteries. Both patients underwent reconstruction of the left pulmonary artery using 13 or 12mm diameter heterologous pericardial conduit at age of 5 year, respectively. At surgery, after the left pulmonary artery was exposed between the upper and lower lobe of the left lung, the conduit was connected with the left pulmonary artery along the pericardium. Continuity between the conduit and the left subclavian artery or the ascending aorta was established with 5 or 6mm diameter Micronit grafts, respectively. Total correction was performed at 2 years and 10 months after the initial surgery, respectively. In a patient with {I, D, D} type corrected transposition of the great arteries, the central pulmonary artery was established with another 16mm diameter heterologous pericardial conduit, which ran in front of the left superior vena cava. The ventricular septal defect was closed via the right atrium. In another patient with {S, L, L}, the central pulmonary artery was established with the reconstructed conduit of the left pulmonary artery, which ran behind the left phrenic nerve. The ventricular septal defect was closed via the right atrium with the De Leval procedure. In both patients, continuities between the left ventricle and the central pulmonary artery were established with tricuspid valved porcine pericardial conduit and equine pericardial conduit. Postoperatively both patients had uneventful recovery with left ventricular/right ventricular systolic pressure ratios of 0.4 and 0.35, respectively.

We report a successful staged repair of anomalous origin of right pulmonary artery from the ascending aorta in a neonate. A two-day-old girl, who suffered from severe circulatory failure, was admitted. In spite of all medical treatment, acidosis and systemic hypotension developed. Right pulmonary artery banding was performed in an emergency procedure, resulting in immediate elevation of systemic blood pressure. Definitive operation was subsequently performed on the 48th day after birth. The right pulmonary artery, which was de-banded and divided from aorta, was anastomosed directly to the pulmonary trunk in a side-to-end manner. The postoperative course was uneventful and the pulmonary artery pressure was within the normal range.