RESUMO
TAPVc is a rare and known cardiac malformation in which there is no direct connection between any pulmonary vein and the left atrium, rather, all pulmonary veins connect to the right atrium,thus the ASD or PFO is necessary for survival after birth. This anomaly has four types that include: cardiac -supra cardiac-infra cardiac and mixed type. Based on drainage of pulmonary veins, that treatment in all types, soon after diagnosis is made is surgical treatment. The prescribed surgical technique, after median sternotomy and hypothermic circulatory arrest, the first stage is ligation and division of vertical vein [connection between left pulmonary vein and innominat vein] and then total correction based on the anatomy. If total correction was done in neonates and infancy period, operative mortality seems to be low but in old age is high because of pulmonary artery hypertension. The aim of this study is to introduce a two stage repair in patients who dont tolerate one stage repair. The vertical vein is kept intact and snared, If pulmonary hypertension and failure occur, opening and appropriately banding this vein will relieve the pulmonary hypertension and associated failure. Two patients with TAPVC types cardiac and Supra cardiac in GHAEM HOSPITAL had cardiac surgery with CPB as usual manner, we ligated vertical vein but not divided it, and then total correction had been done via right atrium and in final of operation we closed ASD, but weaning from CPB was impossible and pulmonary edema and hypotention occurred. With opening of vertical vein, all symptoms were obscured and weaning from CPB was done very well and patients were transferred to ICU division with hemodynamically stability condition. Because of older age of these patients and occurred pulmonary hypertension [PA-pressure = 50 mmgh] and lower left atrial cavity we had hypertensive pulmonary artey crises,and with opening of vertical vein, all symptoms resolve,therfore, recommended: in older children with TAPVC, first, vertical vein should not be closed and in latest stage of operation if condition of patient is good then closure of it done