Abstract
BACKGROUND: Pulmonary venous obstruction after repair of total anomalous pulmonary venous connection (TAPVC) results in substantial morbidity and mortality. Risk factors for postoperative obstruction remain ambiguous. In addition, the existing literature has no standard definition for preoperative obstruction, making patient counseling difficult. METHODS: All patients undergoing repair of TAPVC at our institution from January 1, 2006, to October 23, 2017, were identified. The primary outcome was the development of postoperative obstruction, analyzed as a time-to-event outcome. Clinical information was extracted to assess risk factors. Degrees of preoperative obstruction were defined based on echocardiographic, catheterization, and clinical findings. Univariable and multivariable Cox proportional hazard regression methods were used to identify factors associated with the primary outcome. RESULTS: During the study interval, 119 patients underwent repair of TAPVC (40% single ventricle), and postoperative obstruction developed in 25 patients (21%). Risk factors associated with obstruction were heterotaxy syndrome, single-ventricle heart disease, additional procedures at the time of vein repair, mixed-type TAPVC, and preoperative obstruction. Having even mild preoperative obstruction (≥1.2 m/s by Doppler echocardiography) was predictive of postoperative obstruction. A multivariable model showed mixed-type TAPVC and the presence of preoperative obstruction were associated with a more than twofold greater hazard of obstruction. CONCLUSIONS: TAPVC in the setting of heterotaxy and a single ventricle remains challenging, with high rates of postoperative obstruction. Mixed-type TAPVC is an independent risk factor for postoperative obstruction, particularly in patients with isolated TAPVC. Even mild preoperative obstruction is a risk factor for postoperative obstruction. These results may help risk-stratify TAPVC patients.