Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2019 Oct;31(5):604-610.
doi: 10.1097/MOP.0000000000000804.

Right ventricular failure in congenital heart disease

Mark K Friedberg  1 , Sushma Reddy  2
Affiliations
Review

Right ventricular failure in congenital heart disease

Mark K Friedberg et al. Curr Opin Pediatr. 2019 Oct.

Abstract

Purpose of review: We aim to review select literature pertaining to congenital heart disease (CHD)-induced right ventricular (RV) function and failure.

Recent findings: We review recent findings pertaining to children and adults with repaired tetralogy of Fallot (rTOF), systemic RV and hypoplastic left heart syndrome (HLHS). We emphasize pathophysiological mechanisms contributing to RV dysfunction in these conditions, the risk factors for adverse outcomes and the continuing challenges in treating these patients. We discuss how recent pathology findings, as well as developments in imaging and computer modeling have broadened our understanding of the pathophysiology of these conditions. We further review developments in the molecular and cellular basis of RV failure; and in particular, the RV molecular response to stress in repaired tetralogy of Fallot (rTOF). We highlight some of the genetic complexities in HLHS and how these may influence the long-term outcomes in these patients.

Summary: Recent literature has led to new understandings in the pathology, pathophysiology, risk factors for adverse outcomes, molecular and genetic basis for RV dysfunction and failure in CHD. Although these findings provide new therapeutic targets, the treatment of RV failure at this time remains limited.

PubMed Disclaimer

Conflict of interest statement

Conflicts of interest

There are no conflicts of interest.

References

    1. Yu C, Moore BM, Kotchetkova I, et al. Causes of death in a contemporary adult congenital heart disease cohort. Heart 2018; 104:1678–1682. - PubMed
    1. Annavajjhala V, Punn R, Tacy TA, et al. Serial assessment of postoperative ventricular mechanics in young children with tetralogy of Fallot: comparison of transannular patch and valve-sparing repair. Congenit Heart Dis 2019; Doi: 10.1111/chd.12772. - DOI - PubMed
    1. Geva T, Mulder B, Gauvreau K, et al. Preoperative predictors of death and sustained ventricular tachycardia after pulmonary valve replacement in patients with repaired tetralogy of Fallot enrolled in the INDICATOR cohort. Circulation 2018; 138:2106–2115. - PubMed
    1. He F, Feng Z, Chen Q, et al. Whether pulmonary valve replacement in asymptomatic patients with moderate or severe regurgitation after tetralogy of Fallot repair is appropriate: a case- control study. J Am Heart Assoc 2019; 8:e010689. - PMC - PubMed

    2. Studies have not conclusively demonstrated that pulmonary valve replacement, which reduces pulmonary regurgitation and RV volumes has improved survival or the incidence of ventricular tachyarthymias. Thus, other factors, such as increased RV fibrosis and RV electromechanical dyssynchrony likely contribute to RV failure.

    1. Lumens J, Fan CS, Walmsley J, et al. Relative impact of right ventricular electromechanical dyssynchrony versus pulmonary regurgitation on right ventricular dysfunction and exercise intolerance in patients after repair of tetralogy of Fallot. J Am Heart Assoc 2019; 8:e010903. - PMC - PubMed

MeSH terms