Contemporary Outcomes for Functional Single Ventricle with Total Anomalous Pulmonary Venous Connection.

OBJECTIVE: In 2004, we reported improved early survival for patients with functional single ventricle anatomy and total anomalous pulmonary venous connection (SV/TAPVC). This study sought to discover if outcomes have ameliorated in the contemporary era.

METHODS: This was a single-center review of patients with SV/TAPVC admitted from 1984 to 2021. The cohort was divided into similarly sized groups by date of admission: Era 1- 1984 to 1992, Era 2- 1993 to 2007, and Era 3- 2008 to 2021. Survival was compared and Cox proportional hazards models were used to evaluate the likelihood of mortality.

RESULTS: We included 190 patients with SV/TAPVC. Unbalanced atrioventricular canal defect (70%) was the most common primary diagnosis. The most common type of TAPVC was supracardiac (49%). Approximately one-third (32%) of patients had pulmonary venous obstruction. There were no significant differences in patient characteristics across eras. Early survival after initial palliative operation improved between Eras 1 and 2, then remained stable in Era 3. Overall survival improved from Era 1 to Eras 2 and 3 (p < 0.001), but not between Era 2 and 3. Survival to 10 years by era was 15%, 51%, and 54%. The anatomic features associated with worse survival were HLHS diagnosis (Hazard Ratio [HR] 1.60; 1.04- 2.57) and pulmonary venous obstruction (HR 1.80; 1.24-2.69).

CONCLUSIONS: Overall survival for patients with SV/TAPVC has plateaued since the early 2000s. Even in the most recent era, survival to age 10 remains less than 60%. Risk factors for mortality include the diagnosis of HLHS and pulmonary venous obstruction. Further studies should focus on identification of the pathophysiological factors underlying the increased mortality.