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Published on: December 11, 2017
Thirty-Year Survival after Cardiac Surgery for Patients with Turner Syndrome
Sabikha Alam1, J'Neka S Claxton2, Michael Mortillo3
1Department of Pediatrics, Emory University School of Medicine, Atlanta, GA; Children's Healthcare of Atlanta and Sibley Heart Center Cardiology, Atlanta, GA.
Insights
Long-term survival after congenital heart surgery for left heart obstructive lesions is similar for patients with Turner syndrome and those without. However, Turner syndrome patients have increased cardiovascular disease risks, necessitating lifelong monitoring.
Area of Science:
- Cardiology
- Genetics
- Pediatric Surgery
Background:
- Turner syndrome is associated with congenital heart defects, particularly left heart obstructive lesions (LHOLs).
- Long-term outcomes after surgical repair of these defects in Turner syndrome patients require further evaluation.
Purpose of the Study:
- To assess the long-term survival rates of patients with Turner syndrome following surgical correction of LHOLs.
- To compare survival and cardiovascular disease morbidity between Turner syndrome patients and non-syndromic individuals with similar LHOLs.
Main Methods:
- Utilized the Pediatric Cardiac Care Consortium registry for patients with Turner syndrome undergoing congenital heart surgery (1982-2011).
- Followed outcomes through national death and transplant registries until 2019.
- Employed Kaplan-Meier curves and Cox regression for comparative survival analysis.
Main Results:
- Identified 179 Turner syndrome patients with LHOLs; 161 had 2-ventricle lesions, 18 had hypoplastic left heart variants.
- 30-year transplant-free survival for 2-ventricle LHOLs was comparable (90.4% Turner vs. 90.9% non-syndromic).
- Turner syndrome patients exhibited a significantly higher risk of non-heart failure cardiovascular events.
Conclusions:
- Patients with Turner syndrome and operated 2-ventricle LHOLs demonstrate similar long-term transplant-free survival to non-syndromic peers.
- Increased cardiovascular disease morbidity in Turner syndrome highlights the need for ongoing surveillance and management of comorbidities.
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.
