Total anomalous pulmonary venous connection: outcome of surgical correction and management of recurrent venous obstruction.

ABSTRACT

OBJECTIVE: Total anomalous pulmonary venous connection (TAPVC) can be corrected with low mortality and good outcome. If complicated by pulmonary vein stenosis (PVS), either at presentation or secondary to the repair, the long-term outcome is compromised. We have evaluated an institutional experience with TAPVC, with particular regard to the evolving management of PVS. METHODS: Retrospective analysis of 85 consecutive patients with non-isomeric TAPVC undergoing surgical correction over a 10-year period (1988-1997). In addition, three patients were referred to us with secondary PVS, having had their primary procedure elsewhere. Attention was focused on incidence of PVS, and strategies for management. RESULTS: Median age at first operation was 33 days (range 1-533). Site of drainage was supracardiac (43/88), infracardiac (20/88), cardiac (17/88), and mixed (8/88). On presentation, 35% of patients were ventilated. Early mortality was 7% (6/85), with one late non-cardiac death. 82% of the original patients (70/85) are currently well at a median follow-up of 64 months (range 6-119). The incidence of PVS requiring intervention was 11% (9/85). Median time to PVS was 41 days. In these patients, 18 balloon angioplasties, four endovascular stent placements (in two patients), and a further 23 surgical procedures were performed. Of the nine patients undergoing re-intervention after initial surgery at our institution, five (56%) survived. Two of these have no residual obstruction and right ventricular pressure (RVP) < 50% systemic, two have unilateral obstruction and RVP < 50% systemic, and one has bilateral obstruction and RVP 80% systemic. Of the three patients referred to us with secondary PVS, two are alive and well, and one died early after the first re-operation. CONCLUSIONS: Intrinsic obstruction (endocardial sclerosis or thickening) is associated with worse prognosis and earlier re-intervention than extrinsic (anatomical) obstruction. We advocate an early, aggressive approach to the management of patients with TAPVC, especially in the presence of PVS. This complication is most appropriately managed by a combination of re-operation and repeated balloon dilation.