Post-Surgical Transplant-Free Survival Among Children with Turner Syndrome and Congenital Heart Disease Pubblico
Mortillo, Michael (Spring 2019)
Published
Abstract
Background: Turner syndrome (TS) is a neurogenetic disorder affecting approximately 1 in 2,000 live-born females, characterized by partial or complete monosomy-X. A high rate of morbidity exists among the TS population, primarily due to congenital heart disease (CHD) conditions acquired at birth. People with CHD often undergo various corrective surgical procedures, and depending on the efficacy of the surgery, a subsequent transplant may be required. Though there is much knowledge regarding TS in concert with CHD, little is known about how TS affects post-surgical transplant-free survival (TFS) in people with CHD.
Objective: Assess post-surgical TFS among children with TS and CHD (cases) compared to children with just CHD (controls), to determine if TS increases risk of transplant/mortality.
Methods: This matched cohort study enrolled 704 patients (< 21 years of age) from the Pediatric Cardiac Care Consortium (PCCC), a multi-institutional registry of interventions for CHD from 1982-2011 in 47 US centers. Controls were matched to cases 3:1 by age, primary CHD diagnosis, and year of first surgery. Survival analysis was used to compare TFS between cases and all controls, cases and male controls, and cases and female controls.
Results: Cases had a slightly higher risk of death/transplant during follow-up compared to all controls (HR: 1.27; 95% CI: 0.65 – 2.50; p-value: 0.4872), male controls (HR: 1.40; 95% CI: 0.68 – 2.91; p-value: 0.3610), and female controls (HR: 1.03; 95% CI: 0.45 – 2.39; p-value: 0.9365). Interaction assessment between TS and CHD found no evidence of interaction (X2: 0.6583; p-value: 0.4172).
Conclusions: TS does not appear to increase risk of transplant/mortality in children with CHD, as no significant differences in survival were seen between cases and controls. We recommend further studies to better confirm the association between TS and CHD in TFS.
Table of Contents
Background…………………………………………………………………………………1
Methods………………………………………………………………………………….....3
Results……………………………………………………………………………………...7
Discussion…………………………………………………………………………………10
References…………………………………………………………………………………15
Tables…………………………………………………………………………………...…17
Figures……………………………………………………………………………………..20
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