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Risk factors for death or heart transplantation in single ventricle physiology (tricuspid atresia, pulmonary atresia and heterotaxy): a systematic review and meta-analysis

Kulkarni, Aparna, Patel, Nishali, Singh, Tajinder P., Mossialos, Elias and Mehra, Mandeep R. (2019) Risk factors for death or heart transplantation in single ventricle physiology (tricuspid atresia, pulmonary atresia and heterotaxy): a systematic review and meta-analysis. Journal of Heart and Lung Transplantation, 38 (7). pp. 739-747. ISSN 1053-2498

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Identification Number: 10.1016/j.healun.2019.04.001

Abstract

BACKGROUND: In this study we sought to evaluate risk factors (RFs) for death or heart transplantation (D-HT) in single-ventricle (SV) physiology due to tricuspid atresia (TA), pulmonary atresia‒intact ventricular septum (PA-IVS), and heterotaxy with SV (HX), clinical conditions for which outcome data are limited. METHODS: To conduct a systematic review, we included citations that evaluated occurrence of D-HT in SV physiology of TA, PA-IVS, and HX in English articles published between January 1998 and December 2017 based on inclusion and exclusion criteria, following the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines. The Cochrane Risk of Bias in Non-Randomized Studies-Interventions (ROBINS-I) tool for non-randomized studies was used to assess the risk of bias. Meta-analysis was performed if RF data were available in more than 3 studies. RESULTS: Of 11,629 citations reviewed, 30 met inclusion criteria. All 30 were observational, retrospective studies. In all, 1,770 patients were included, 481 died and 21 underwent HT (63 lost to follow-up); 723 patients reached Fontan completion. We found that systemic ventricular dysfunction (odds ratio [OR] 20.7, confidence interval [CI] 10.0–42.5, I 2 = 0%) and atrioventricular valve regurgitation (AVR) were associated with risk of D-HT (OR 3.7, CI 1.9–6.9, I 2 = 14%). RF associations with D-HT could not be derived for right ventricle‒dependent coronary circulation, pulmonary arteriovenous malformations, total anomalous pulmonary venous return, arrhythmias, and pulmonary atresia. CONCLUSIONS: This systematic review and meta-analysis has identified a high mortality rate in children born with non-HLHS SV heart disease and points to potential under-utilization of HT. Systemic ventricular dysfunction and AVR were identified as RFs for D-HT in this subset of patients SV with TA, PA-IVS, and HX.

Item Type: Article
Additional Information: © 2019 International Society for Heart and Lung Transplantation
Divisions: LSE Health
Health Policy
Subjects: R Medicine
Date Deposited: 12 Apr 2019 23:10
Last Modified: 24 Jan 2020 00:13
URI: http://eprints.lse.ac.uk/id/eprint/100450

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