30-year Survival after Cardiac Surgery for Patients with Turner Syndrome.

OBJECTIVE: To evaluate long-term survival in patients with Turner Syndrome after congenital heart surgery with a focus on left heart obstructive lesions (LHOLs).

STUDY DESIGN: We queried the Pediatric Cardiac Care Consortium (PCCC), a US-based registry of CHS, for patients with TS undergoing CHS at <21 years of age between 1982 and 2011. Outcomes were obtained from the PCCC and from national death and transplant registries through 2019. Survival of patients with Turner Syndrome and non-syndromic patients with similar LHOL was compared by Kaplan-Meier survival curves and Cox regression adjusted for age, congenital heart disease (CHD) and era.

RESULTS: We identified 179 patients with TS operated for LHOL: 161 with two-ventricle lesions (coarctation n=149, aortic stenosis n=12) and 18 with hypoplastic left heart (HLH) variants. There were 157 with two-ventricle LHOL and 6 with HLH survived to discharge. Among survivors to hospital discharge, the 30-year transplant-free survival was 90.4% for TS with two-ventricle lesions and 90.9% for non-syndromic comparators (aHR 1.15, 95%CI:0.64-2.04). The post-discharge survival for HLH was 33% for TS and 51% for non-syndromic patients, with these numbers being too small for meaningful comparisons. There was a higher risk for cardiovascular disease (CVD) events in patients with TS vs. male (aHR 3.72, 95%CI:1.64-8.39) and female comparators (aHR 4.55, 95%CI:1.87-11.06) excluding heart-failure deaths.

CONCLUSIONS: The 30-year transplant-free survival is similar for patients with TS and non-syndromic comparators with operated two-ventricle LHOL without excess CHD risk. However, patients with TS still face increased CVD morbidity, stressing the importance of lifelong comorbidity surveillance in this population.