The Association of Single Ventricle Morphology with Long Term Quality of Life Among Survivors: A Report from CHD PULSE (Congenital Heart Disease Project to Understand Lifelong Survivor Experience)

BACKGROUND Among individuals with single ventricle (SV) congenital heart disease (CHD), systemic right ventricle morphology is associated with decreased survival compared to systemic left ventricle morphology. We aimed to determine the association of ventricular morphology with quality of life and medical, neurocognitive, socioeconomic outcomes and compare these between SV CHD patients and siblings. METHODS We performed a cross-sectional cohort study using data from CHD PULSE (Congenital Heart Disease Project to Understand Lifelong Survivor Experience), a survey conducted from 2021-2023 among adults with CHD interventions and siblings across 11 US Pediatric Cardiac Care Consortium centers. Quality of life was assessed by PROMIS (Patient-Reported Outcomes Measurement Information System) surveys. RESULTS We included 226 SV patients (146 left and 80 right ventricular) and 40 siblings. No outcome differences were found between left and right ventricular groups. Compared to siblings, SV patients reported more medical comorbidities (p<0.001), future health concerns (0.008), disability benefits (p<0.001), and work affected by health (p<0.001). Siblings had higher rates of college/graduate degrees (p=0.002) and employment (p=0.044). However, 95.6% of SV patients completed high school, 45.6% had college/graduate degrees, and 73.9% were employed. SV patients had lower PROMIS scores for global mental health and physical function but similar scores for global physical health, cognitive function, anxiety, depression, and ability to participate in social roles. CONCLUSIONS Ventricular morphology was not associated with differences in long-term outcomes. Adults with SV CHD have medical, neurocognitive, and socioeconomic differences compared to siblings, but similar PROMIS scores compared to the general population.