Heart failure in systemic right ventricle: Mechanisms and therapeutic options

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 decompensati...

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Published inFrontiers in cardiovascular medicine Vol. 9; p. 1064196
Main Authors Sabbah, Belal Nedal, Arabi, Tarek Ziad, Shafqat, Areez, Abdul Rab, Saleha, Razak, Adhil, Albert-Brotons, Dimpna Calila
Format Journal Article
LanguageEnglish
Published Switzerland Frontiers Media S.A 10.01.2023
Subjects
atrial switch repair
Cardiovascular Medicine
congenitally corrected transposition of the great arteries
dextro-transposition of the great arteries
heart failure
systemic right ventricle
tricuspid regurgitation
congenitally corrected transposition of the great arteries
dextro-transposition of the great arteries
heart failure
tricuspid regurgitation
systemic right ventricle
congenital heart disease
pediatric cardiology
atrial switch repair
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Summary: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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Edited by: Xiaolei Xu, Mayo Clinic, United States
These authors have contributed equally to this work and share first authorship
This article was submitted to Pediatric Cardiology, a section of the journal Frontiers in Cardiovascular Medicine
Reviewed by: Zahra Saadatian, Gonabad University of Medical Sciences, Iran; Ewa Kowalik, National Institute of Cardiology, Poland; Maruti Haranal, U.N. Mehta Institute of Cardiology and Research, India
ISSN:2297-055X
2297-055X
DOI:10.3389/fcvm.2022.1064196