Quality of Life and Outcomes of Adults with Troncus Arteriosus

Truncus arteriosus (TA) is a rare lesion, defined by a single great vessel from which the pulmonary arteries, aorta, and coronary arteries arise. Operative correction has dramatically reduced mortality. However, the adult life of these patients is still affected by the burden of reinterventions (specifically for right ventricular outflow tract obstruction and truncal valve dysfunction). Quality of life (QoL) has become an important issue in patients with congenital heart disease (CHD). Accordingly, the focus has shifted from mere survival to a better QoL. Despite a good understanding of the long-term surgical outcomes, there are no data on the health-related quality of life following TA repair in those who reach adulthood. This study aimed to explore QOL and physical activity in a cohort of adults with TA, the association between patient's characteristics and QOL. QOL was assessed using the 5-level EQ-5D version (EQ-5D-5L). Physical activity was assessed using the Ricci-Gagnon self-questionnaire. Ninety-three adult patients (age > 16 years on 31 Dec 2019) with TA were identified in our centre for this study. Of these, 64 (69%) participated and completed the questionnaires. Medical records were reviewed. Associations of QOL with patients’ characteristics were assessed. Mean age was 24 years (±6.5) and 27 (41.5%) were male. Three of them did not undergo repair and were in Eisenmenger status. Age at repair was 136 days (±418). All repaired patients underwent reinterventions in childhood either by surgery or catheterism (from 1 to 10 reinterventions/patient) whereas 17 (26%) underwent reinterventions in adulthood. Overall, they reported a good QOL comparable to French population norms with a mean EQ-5D Index of 0.84 (±0.21). Forty-one percent patients reported to be active. Reinterventions in adulthood was the only variable associated with better QOL (P < 0.01). The distribution of the index values (QOL) according to the number of reinterventions in adulthood is shown in Fig. 1. TA patients experience a generally good QOL with a positive association of QOL with reinterventions in adulthood.