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Article in Japanese | WPRIM | ID: wpr-825919


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.

Article in Japanese | WPRIM | ID: wpr-375641


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.

Article in Japanese | WPRIM | ID: wpr-375915


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.

Article in Japanese | WPRIM | ID: wpr-374618


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.

Article in Japanese | WPRIM | ID: wpr-374589


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.

Article in Japanese | WPRIM | ID: wpr-367181


A 41-year-old woman with neurofibromatosis (NF) was admitted to our hospital for severe pain and right leg swelling of 5 days duration. Paralysis of the right leg due to compartment syndrome was also recognized. She had been diagnosed as von Recklinghausen's neurofibromatosis, previously. 3 D-computed tomography showed a ruptured anterior tibial artery aneurysm. There was a normal patent posterior tibial artery. Since her complaint of pain was severe, we performed an emergency operation. Under the pneumatic tourniquet technique, the aneurysm was resected, and both the proximal and distal sides of the anterior tibial artery were ligated. A massive hematoma was completely removed. Postoperatively, the dorsalis pedis and posterior tibial pulses remained palpable. The paralysis improved considerably and she was given an ambulatory discharge from our hospital 21 days after the operation. Histological examination revealed proliferating wavy spindle cells infiltrating between the adventitia and mesothelium of the aneurysmal wall and staining positively for S 100 immunoperoxidase.