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. 2021 Dec:239:187-192.e1.
doi: 10.1016/j.jpeds.2021.08.034. Epub 2021 Aug 24.

Thirty-Year Survival after Cardiac Surgery for Patients with Turner Syndrome

Sabikha Alam  1 , J'Neka S Claxton  2 , Michael Mortillo  3 , Leandros Sassis  4 , Pelagia Kefala-Karli  4 , Michael Silberbach  5 , Lazaros Kochilas  6 , Stephanie Burns Wechsler  7
Affiliations

Thirty-Year Survival after Cardiac Surgery for Patients with Turner Syndrome

Sabikha Alam et al. J Pediatr. 2021 Dec.

Abstract

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, a US-based registry of congenital heart surgery, for patients with Turner syndrome undergoing congenital heart surgery at <21 years of age between 1982 and 2011. Outcomes were obtained from the Pediatric Cardiac Care Consortium and from national death and transplant registries through 2019. Survival of patients with Turner syndrome and nonsyndromic patients with similar LHOL was compared by Kaplan-Meier survival curves and Cox regression adjusted for age, congenital heart disease, and era.

Results: We identified 179 patients with Turner syndrome operated for LHOL: 161 with 2-ventricle lesions (coarctation n = 149, aortic stenosis n = 12) and 18 with hypoplastic left heart (HLH) variants. There were 157 with 2-ventricle LHOL and 6 with HLH survived to discharge. Among survivors to hospital discharge, the 30-year transplant-free survival was 90.4% for Turner syndrome with 2-ventricle lesions and 90.9% for nonsyndromic comparators (adjusted hazard ratio [aHR] 1.15, 95% CI 0.64-2.04). The postdischarge survival for HLH was 33% for Turner syndrome and 51% for nonsyndromic patients, with these numbers being too small for meaningful comparisons. There was a higher risk for cardiovascular disease events in patients with Turner syndrome 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 Turner syndrome and nonsyndromic comparators with operated 2-ventricle LHOL without excess congenital heart disease risk. However, patients with Turner Syndrome still face increased cardiovascular disease morbidity, stressing the importance of lifelong comorbidity surveillance in this population.

Keywords: Turner syndrome; congenital heart disease; left heart obstructive lesions; mortality.

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Figures

Figure 1.
Figure 1.
Study cohort flow diagram leading to 179 patients with Turner syndrome and LHOL. *Indicates similar criteria for the selection of the nonsyndromic comparator cohort (n = 6895).
Figure 2.
Figure 2.
Kaplan-Meier survival plots for patients with Turner syndrome and comparators after congenital heart surgery for 2-ventricle LHOL. Thirty-year transplant-free survival of patients with Turner syndrome with 2-ventricle LHOL Compared with nonsyndromic comparators: Kaplan-Meier curves for patients with Turner syndrome and nonsyndromic comparators with 2 ventricles and LHOL, conditioned on being discharged alive after first congenital heart surgery. Survival estimates given in % with 95% CI in parentheses for all groups.
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