Mid-term Results After Surgical Correction of Total Anomalous Pulmonary Venous Connection

Objective:To evaluate the surgical treatment of total anomalous pulmonary venous connection (TAPVC) and determination of predictors for postoperative death. Method Material: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. There were an atrial communication in all patients, large PFO in 23% and ASD in 77%. 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 degrees 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%. Heart failure and low cardiac output syndrome were the most common causes of death. The incidence of postoperative death was the highest in 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 also 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).