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Heart Failure in Systemic Right Ventricle: Mechanisms and Therapeutic Options

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Abstract

d-loop transposition of the great arteries (d-TGA) and congenitally corrected transposition of the great arteries (cc-TGA) feature a right ventricle attempting to sustain the systemic circulation. A systemic right ventricle (sRV) cannot support cardiac output in the long run, eventually decompensating and causing heart failure. The burden of d-TGA patients with previous atrial switch repair and cc-TGA patients with heart failure will only increase in the coming years due to the aging adult congenital heart disease population and improvements in the management of advanced heart failure. Clinical data still lags behind in developing evidence-based guidelines for risk stratification and management of sRV patients, and clinical trials for heart failure in these patients are underrepresented. Recent studies have provided foundational data for the commencement of robust clinical trials in d-TGA and cc-TGA patients. Further insights into the multifactorial nature of sRV failure can only be provided by the results of such studies. This review discusses the mechanisms of heart failure in sRV patients with biventricular circulation and how these mediators may be targeted clinically to alleviate sRV failure.

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References
1.
Brida M, Diller G, Gatzoulis M . Systemic Right Ventricle in Adults With Congenital Heart Disease: Anatomic and Phenotypic Spectrum and Current Approach to Management. Circulation. 2018; 137(5):508-518. DOI: 10.1161/CIRCULATIONAHA.117.031544. View

2.
Szymanski P, Klisiewicz A, Lubiszewska B, Lipczynska M, Konka M, Kusmierczyk M . Functional anatomy of tricuspid regurgitation in patients with systemic right ventricles. J Am Soc Echocardiogr. 2010; 23(5):504-10. DOI: 10.1016/j.echo.2010.01.007. View

3.
Warnes C . Transposition of the great arteries. Circulation. 2006; 114(24):2699-709. DOI: 10.1161/CIRCULATIONAHA.105.592352. View

4.
Akiyama-Uchida Y, Ashizawa N, Ohtsuru A, Seto S, Tsukazaki T, Kikuchi H . Norepinephrine enhances fibrosis mediated by TGF-beta in cardiac fibroblasts. Hypertension. 2002; 40(2):148-54. DOI: 10.1161/01.hyp.0000025443.61926.12. View

5.
Khairy P . Sudden cardiac death in adults with transposition of the great arteries and systemic right ventricles: preventing (night)MAREs. Eur Heart J. 2022; 43(28):2695-2697. DOI: 10.1093/eurheartj/ehac228. View