RESULTS OF REPAIR OF TOTAL ANOMALOUS PULMONARY VENOUS CONNECTION IN INFANCY USING DEEP HYPOTHERMIC CIRCULATORY ARREST

Abstract

In order to determine the results of repair of total anomalous pulmonary venous connection (TAPVC) utilizing deep hypothermic circulatory arrest and to observe the hemodynamic changes following relief of pulmonary venous obstruction, we obtained early and/or late postoperative(postop)data on 13 of 15 infants who underwent correction in infancy. Seven had supracardiac drainago(SC), 4 had cardiac drainage(C)and in 4 tho drainage was infracardiac(IC). Additional lesions included PDA(8), VSD(1), hypoplastic LV(1). Pulmonary artery hypertennion(PAH)was present in 13. Median age was 27 days(1.5d-8mo)and mean body weight was 3.68Kg(2.22-5.25Kg)at surqery. Three infanta died(20%)within the initial hospitalization(2 SC, 1 IC) (there were 2 late deaths, 3 and 10 mo postop, both with IC. Combined mortality was 33%. Systolic pulmonary artery pressure(PAp)was measured within 24 hours postop in 6, all with SC or C, and had decreased from an average of 65 nmHg down to 30 ramHg. Among the 10 long-term survivors (moan 20mo poston)3 have not been restudied, but are well clinically. Six of the 7 infants restudied(average 15 mo postop)had normal PAp(avoraqe 22mmHg). One with IC had residual PAH and we suspect this infant has pulmonary vascular obstructive disease. None had residual shunts. We believe, on the basis of these observations, that physiological correction may be accomplished at an acceptable risk in Infants with TAPVC utilizing deep hypothermia circulatory arrest.