ABSTRACT
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.
ABSTRACT
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.
ABSTRACT
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.
ABSTRACT
A 4-year-old boy was born with cyanosis and was given a diagnosis of tetralogy of Fallot and pulmonary atresia. Echocardiography showed membranous atresia of the pulmonary trunk that was connected to the left side of the ascending aorta via an aortopulmonary window 3 mm in diameter. Four major aortopulmonary collateral arteries (MAPCAs) were detected by cardiac catheterization and computed tomography angiography prior to undergoing surgery at 4 years of age. We performed one-stage complete unifocalization and definitive repair via a median sternotomy. The MAPCA supplying the left lower lobe was anastomosed to the true left pulmonary artery and the pulmonary artery trunk was augmented with an autologous pericardium patch. We then reconstructed the right ventricular outflow tract using a transannular patch and simultaneously patch-closed the VSD. The right/left ventricle pressure ratio after weaning from cardiopulmonary bypass was 0.8. The postoperative course was uneventful and the patient was discharged 26 days later. Seven months after the procedure, the right/left ventricle pressure ratio was decreased to 0.56 on cardiac catheterization.
ABSTRACT
A baby girl delivered at 41 weeks of gestation with persistent truncus arteriosus (PTA) and interrupted aortic arch (IAA) type A was referred to our institute for surgical intervention. Bilateral pulmonary artery banding (BPAB) proceeded through a median sternotomy at the age of 11 days to control excessive pulmonary blood flow. Thereafter, she gained weight under continuous prostaglandin E1 (PGE 1) infusion. Definitive repair proceeded at the age of 2 months. Cardiopulmonary bypass was established through a redo-median sternotomy, with two arterial cannulae (brachiocephalic artery and descending aorta). The aortic arch was reconstructed with direct anastomosis. The orifice of the pulmonary artery was removed from the arterial trunk and the defect in the aortic wall was directly closed. A ventricular septal defect was closed under cardioplegic arrest via a right ventriculotomy. The continuity from the right ventricle to the pulmonary artery was made using a hand-made, extended polytetrafluoroethylene (ePTFE) conduit with a bicusp. The sternum was left open at the end of the procedure and the chest was closed on post-operative day (POD) 3. She was weaned from mechanical ventilation on POD 4 and the postoperative course was uneventful. She was discharged on POD 49.
ABSTRACT
Recent progress in chemotherapy has prolonged the survival of patients with malignant biliary strictures, leading to increased rates of stent occlusion. Even we employed metallic stents which contributed to higher rates and longer durations of patency, and occlusion of covered metallic stents now occurs in about half of all patients during their survival. We investigated the complication and patency rate for the removal of covered metallic stents, and found that the durations were similar for initial stent placement and re-intervention. In order to preserve patient quality of life, we currently recommend the use of covered metallic stents for patients with malignant biliary obstruction because of their removability and longest patency duration, even though uncovered metallic stents have similar patency durations.