Management of pulmonary venous obstruction after correction of TAPVC: risk factors for adverse outcome

Recurrent pulmonary venous obstruction (PVO) occurs in 0-18% of infants undergoing correction of total anomalous pulmonary venous connection (TAPVC). Limited published data suggest that PVO usually develops within 6 months of primary repair, and that outcomes of reoperations are poor. This study aimed to review our experience of reoperations for PVO post-TAPVC repair and to identify risk factors for adverse outcome.

Twenty patients underwent reoperation for PVO between 1982 and 2002. Clinical data were reviewed. TAPVC was mostly infracardiac (11 patients). TAPVC was obstructed in nine patients. PVO developed early (<6 months) in seven patients, and late in 13 (>6 months). Time of presentation was unrelated to type of PVO (anastomotic vs. ostial). Repair was accomplished using various techniques (anastomotic enlargement with native atrial tissue, enlargement with pericardium, free or in situ, or other prosthetic material). Follow-up ranged from 1 month to 15 years (average 44 months).

Thirteen patients received one reoperation, while seven had multiple reoperations. In 13 patients, PVO was defined as new onset (no obstruction post-TAPVC repair), and in seven patients as residual (minimal obstructive changes post-TAPVC repair that progressed to PVO). Ten patients presented with anastomotic PVO, six with anastomotic and ostial PVO (involving the PVs), three with ostial PVO, and one with coronary sinus-left atrial junction stenosis. Mortality was 25% (5/20). Six of the ten patients with anastomotic PVO underwent one reoperation (2/6 died); the other four developed ostial PVO after reoperation, requiring multiple procedures (2/4 died). Mode of presentation (new onset vs. residual), site of obstruction (anastomotic vs. ostial), preoperative RV pressure (<0.8 vs. >0.8 systemic), number of reoperations (single vs. multiple), residual obstruction (presence or absence), and operative approach (Gore-tex or not) did not seem to affect outcomes. Risk factors for death were early presentation (<6 months) and persistence of pulmonary hypertension after reoperation; early presentation was also a risk factor for multiple reoperations.

Our findings support the conclusion that early presentation and postoperative pulmonary hypertension have the greatest adverse impact on outcome. Of these, failure to achieve a low-pressure pulmonary vascular system seems to be the variable that most strongly prevents survival. In our series, neither ostial PVO nor multiple re-interventions significantly increased surgical risk. The negative impact of postoperative residual obstruction on outcome was not striking. However, an aggressive surgical approach to this disease is still warranted. Although the role of each technique in obtaining long-lasting relief of PVO remains to be established, the use of artificial material seems unwise.