We report a rare case of double outlet right ventricle (DORV) with sub-pulmonary type ventricular septal defect (VSD). The great arteries were almost side-by-side, and the ascending aorta was located slightly posterior to the right of the pulmonary artery. We performed complete repair at the age of 25 days. Intra-cardiac rerouting (VSD closure) was carried out through the tricuspid valve. Arterial switch procedure was performed without the Lecompte maneuver. His postoperative course was uneventful and he was discharged 19 days after the operation without any complications.

We performed bilateral pulmonary artery banding (BPAB) through a median sternotomy on a four-day-old male infant with a double-outlet right ventricle (DORV) and interrupted aortic arch (IAA) who was delivered at 40 weeks of gestation. After urinary output improved, definitive repair was carried out 5 days later. Intra-ventricular rerouting was followed by arterial switch with the Lecompte maneuver. The aortic arch was reconstructed with direct anastomosis and the right ventricular outflow tract was augmented with a patch. The sternum was left open at the end of the procedure and the chest was closed on post-operative day (POD) 4. The patient was discharged from hospital on POD 78 after receiving treatment for pneumonia and chylothorax.

A 37-year-old male patient who had previously undergone left original Blalock-Taussig shunt, original Glenn shunt, left pulmonary artery patch plasty, and a Björk procedure was referred to our hospital due to protein-losing enteropathy. Because he suffered from severe low-cardiac output syndrome immediately after the Björk procedure, mechanical circulatory support and construction of the bypass between the right atrial appendage and the innominate vein using an artificial graft were required. We performed a Fontan-revision operation : total cavopulmonary connection with extra-cardiac conduit, right atrial ablation, pacemaker lead implantation, construction of fenestration between the conduit and the atrium, and reconstruction of the left pulmonary artery in front of the ascending aorta successfully. His postoperative course was uneventful and protein-losing enteropathy had not recurred 3 years after the operation.

The case is an infant referred to the mother after a fetal ultrasound at 22 gestational weeks revealed a left renal cyst and congenital heart disease. He was born at 36 weeks and five days, weighing 2,282 g, with Apgar 8/8 points, and diagnosed with tetralogy of Fallot, absent pulmonary valve, pulmonary regurgitation, persistent left superior vena cava, right aortic arch, and cervical arch. He was placed on ventilatory management immediately after birth, but was extubated at age nine days. On approximately day 26, since the SpO2 markedly decreased and bronchoscopy showed only mild stenosis, we concluded that decreased pulmonary blood flow mainly caused cyanosis. Because of the low body weight and presence of a single coronary artery, we decided to proceed with a palliative surgery. However, since the patient had a cervical arch and the usual Blalock-Taussig shunt was anatomically difficult, we performed a central shunt＋main pulmonary artery ligation on day 49. Cyanosis improved and his respiratory condition was stable, but contrast-enhanced computed tomography showed a tendency for left pulmonary artery enlargement and left main bronchus compression. On day 87, the Rastelli procedure＋bilateral pulmonary artery plication was performed because worsening airway symptoms were anticipated. The patient’s respiratory condition stabilized postoperatively, and he was extubated 10 days postoperatively. On day 136, the patient was discharged with a home high-flow nasal cannula.