Total Anomalous Pulmonary Venous Connection: The Results of 23 Consecutive Patients

ABSTRACT

PURPOSE: Surgical risk of total anomalous pulmonary venous connection (TAPVC) is high, especially in patients with early presentation resulting from pulmonary venous obstruction or with advanced pulmonary vascular obstructive disease (PVOD). We reviewed our experience of primary correction of TAPVC and examined the surgical outcome and risk factors. METHODS: From June 1991 to Sept. 1996, 23 neonates or infants who were diagnosed simple TAPVC and underwent primary correction at Asan Medical Center were included. They were 16 males and 7 females. We analyzed clinical records, operative records, preoperative and postoperative echocardiographic findings or cardiac catheterization angiographic data and follow-up data and analyzed risk factors for late death. RESULTS: Age at operation ranged from 1 week to 9 months (mean 66 days). 11 cases (49%) were less than 1 month and 19 cases (83%) were less than 3 months. Weight at operation ranged from 2.2kg to 6.4kg (mean 4.0 +/- 1.3kg). The anatomical types of TAPVC was supracardiac in 17, infracardiac in 4, cardiac in 1, mixed type in 1. There were 10 preoperative pulmonary venous obstructions. There were 2 cases of hospital death; one died of persistent low cardiac output from postoperative complete AV block, the other died unsuspectedly. Late death were 5 cases (23.8%). Anastomotic stenosis was suspected in 4 cases on postoperative echocardiography (1 case was confirmed by cardiac catheterization and angiography). one case died of progressive PVOD. Risk factor analysis revealed that only anastomotic stenosis was related to late death (P=0.005). CONCLUSION: From our series, we concluded that earlier surgical intervention is important to prevent PVOD especially in cases with pulmonary hypertension preoperatively and we are alert to the development of late pulmonary venous stenosis especially in cases with intimal irregularity at operative field or vague symptoms postoperatively. Finally prompt reintervention is necessary in cases with correctable pulmonary venous stenosis to decrease late death.