ISSN : 1301-5680
e-ISSN : 2149-8156
Turkish Journal of Thoracic and Cardiovascular Surgery     
Cevahir HABERAL, * Emin TİRELİ, * Enver DAYIOĞLU, * Ertan ONURSAL
Koşuyolu Kalp ve Araştırma Hastanesi, İSTANBUL
*İ.Ü. İstanbul Tıp Fakültesi Göğüs, Kalp ve Damar Cerrahisi Anabilim Dalı, İSTANBUL
Pulmonary atresia with ventricular septal defect (VSD) hawing major aortopulmonary collateral arteries is a rare congenital cardiac disease. These cases differ from type IV Truncus arteriosus for having central pulmonary arteries and from pseudotruncus for having MAPCA’s and they are classified as ‘’VSD+PA+MAPCA’’ in new surgical concept.

Seven ‘’VSD+PA+MAPCA’’ cases were admitted to the department of Thoracic and Cardiovascular Surgery, Istanbul Medical Faculty, University of Istanbul. Six cases had hypoplastic confluent pulmonary arteries and 2 cases had no native pulmonary artery. Out of 21 MAPCA’s, only 1 MAPCA had a communication with the native pulmonary artery. Five cases had right arcus aorta. Patients had average 3 MAPCA’s, ranging between 2, 6. Two cases had right unifocalization through right thoracotomy, 2 cases had left unifokalization through left thoracotomy, 2 cases had bilateral unifokalization through median sternotomy and 2 cases were considered to be inoperable. MAPCA’s were unifocalized using polytetrafluoroethylene graft in 1 case and in 7cases, MAPCA’s were unifocalized with direct anastomosis. There was no mortality.

Although unifocalization for VSD+PA+MAPCA cases are limited today, we think that staged surgical repairs are suitable for these cases.

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