JOURNAL ARTICLE
Add like
Add dislike
Add to saved papers

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

Journal of Pediatrics 2021 August 25
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.

Full text links

For the best experience, use the Read mobile app

Group 7SearchHeart failure treatmentPapersTopicsCollectionsEffects of Sodium-Glucose Cotransporter 2 Inhibitors for the Treatment of Patients With Heart Failure Importance: Only 1 class of glucose-lowering agents-sodium-glucose cotransporter 2 (SGLT2) inhibitors-has been reported to decrease the risk of cardiovascular events primarily by reducingSeptember 1, 2017: JAMA CardiologyAssociations of albuminuria in patients with chronic heart failure: findings in the ALiskiren Observation of heart Failure Treatment study.CONCLUSIONS: Increased UACR is common in patients with heart failure, including non-diabetics. Urinary albumin creatininineJul, 2011: European Journal of Heart FailureRandomized Controlled TrialEffects of Liraglutide on Clinical Stability Among Patients With Advanced Heart Failure and Reduced Ejection Fraction: A Randomized Clinical Trial.Review

Get seemless 1-tap access through your institution/university

For the best experience, use the Read mobile app

Read by QxMD is copyright © 2021 QxMD Software Inc. All rights reserved. By using this service, you agree to our terms of use and privacy policy.

Get seemless 1-tap access through your institution/university

For the best experience, use the Read mobile app