midterm results after surgical correction of total anomalous pulmonary venous connection

abstract   objective: to evaluate the surgical treatment of total anomalous pulmonary venous connection (tapvc) and determination of predictors for postoperative death. methods: between 1995 and 2005, 80 patients aged from 1 month to 12 years underwent surgical repair for supracardiac (39), cardiac (34), infracardiac (3) or mixed (4) type of tapvc. systemic pulmonary hypertension (ph) in 53.8% of patients, half systemic ph in 26.3% and mild ph (< 40 mmhg) were found by preoperative evaluations. twelve patients (15%) had some degree of pulmonary vein obstruction preoperatively.   results: seven patients (8.7%) died in the operating room. early postoperative mortality (during 48 hours) occurred in 11 cases (13.7%) and nine patients died during first hospitalization. we did not have late mortality in survivors during follow-up period therefore the overall mortality rate was 33.8%. the incidence of postoperative death was highest in the infracardiac type (2/3). approximately two-thirds of dead patients (21/27) had presented with systemic ph and 89% of them had at least half-systemic ph preoperatively. mortality rate in patients with normal pulmonary artery pressure (pap) was zero.   conclusion: in contrast to early surgical results we had excellent mid-term outcome. the role of myocardial protection and surgical technique are the most probable causes of high death rate in our series. however influences of poor preoperative stabilization process as well as anesthetic technique and cardiopulmonary bypass related problems should be considered. pap more than half of systemic pressure and patient age smaller than 3 months were the primary predictive factors for premature death (p< 0.05).