Contemporary outcomes for functional single ventricle with total anomalous pulmonary venous connection

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

This was a single-center review of patients with single ventricle anatomy and total anomalous pulmonary venous connection who were 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.

We included 190 patients with single ventricle anatomy and total anomalous pulmonary venous connection. Unbalanced atrioventricular canal defect (70%) was the most common primary diagnosis. The most common type of total anomalous pulmonary venous connection 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, and then remained stable in Era 3. Overall survival improved from Era 1 to Eras 2 and 3 (P < .001), but not between Era 2 and 3. Survival to 10 years by Eras 1 to 3 was 15%, 51%, and 54%, respectively. The anatomic features associated with worse survival were hypoplastic left heart syndrome diagnosis (hazard ratio, 1.60; 1.04-2.57) and pulmonary venous obstruction (hazard ratio, 1.80; 1.24-2.69).

Overall survival for patients with single ventricle anatomy and total anomalous pulmonary venous connection has plateaued since the early 2000s. Even in the most recent era, survival to age 10 years remains less than 60%. Risk factors for mortality include the diagnosis of hypoplastic left heart syndrome and pulmonary venous obstruction. Further studies should focus on identification of the pathophysiological factors underlying the increased mortality.