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Article in Chinese | WPRIM | ID: wpr-799061


Objective@#To investigate the clinical outcomes of selective major aortopulmonary collaterals(MAPCAs) unifocalization and report histopathological findings in patients with pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals(PA/VSD/ MAPCAs).@*Methods@#The study enrolled 6 MAPCAs/VSD/PA patients with age ranged from 6 to 96 months and body weight ranged from 5.0 to 23.0 kg. These patients underwent selective MAPCAs unifocalization and primary repairs. Preoperative cardiac catheter, selective arteriography, cardiac CTA and intraoperative pathology were performed to identify different function, anatomic distribution and histopathology of MAPCAs.@*Results@#6 MAPCAs/VSD/PA patients underwent selective MAPCAs unifocalization and primary repair. No death occurred after operation and at follow-up which lasted for 1 to 20 months. Preoperative cardiac catheter, selective arteriography and intraoperative histopathology demonstrated distribution of functional MAPCAs similar to native pulmonary artery arborization and participating in arterial gas exchange. Functional MAPCAs were classified into elastic arteries according to histopathology.@*Conclusion@#There are two histological type of MAPCAs which play different roles. Selective unifocalization to functional MAPCAs which are classified into elastic arteries like native pulmonary artery is a safe and effective treatment approach for PA/VSD/MAPCAs.

Article in Chinese | WPRIM | ID: wpr-450347


Objective Sum up the research of concept of surgical procedure of PA/VSD/MAPCA.Methods Between January 2003 and December 2012,92 children were operated,aged 1 day to 5.2 years ; median age (1.3 ± 0.6) years.There were 26 cases for one stage operation,66 cases were for palliative operation which were divided into 3 stages.The 3 stages of palliative operation included system to pulmonary shunt,conduit from RVOT to MPA or transannular patch from RVOT to MPA,enlargemene of pulmonary artery and ligation of MAPCA.From all the patients,15 cases had been finished the two staged operation.In the end,there were 17 cases who were completed two or three stage radical operation.Results There were 3 cases who died from the first period.There were 9 cases who were completed two stage operation,and 1 case died.There were 8 cases who were completed three stage operation.there was 1 cases who died in this period.Conclusion The surgical treatment for the PA/VSD/MAPCA could be that the connection between RVOT and pulmonary artery should be performed as early as possible which could provide the blood flow to pulmonary artery and promote the growth.Uniforcalization could also be set up as early as possible if it is needed.It could be ligated in different period.The PA/VSD/MAPCA could be repaired by stagesand have satisfied results.

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.