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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 ORCID: 0000-0001-8664-9297 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: 16 May 2024 02:42
URI: http://eprints.lse.ac.uk/id/eprint/100450

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