Right Ventricular Remodeling in Complex Congenital Heart Disease.

Matthieu Ruiz, François-Pierre Mongeon, Paul Khairy, Nabil Dib, Annie Dore, Marie-A Chaix, Réda Ibrahim, Seema Mital, Blandine Mondésert, Marilee Demonceaux, Juva Benseba, Maxime Tremblay Gravel, Stéphanie Tan, Nancy Poirier

Journal: The Canadian journal of cardiology 2025;41(6):1067-1080

PMID: 39800187

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Abstract

In congenital heart diseases (CHDs) of moderate to great complexity involving the right ventricle (RV), the morphologic RV can be exposed to significant stressors across the lifespan, either in a biventricular circulation in a subpulmonary or subaortic position or as part of a univentricular circulation. These include pressure and/or volume overload, hypoxia, ischemia, and periprocedural surgical stress, leading to remodeling, maladaptation, dilation, hypertrophy, and dysfunction. In this review we examine the macroscopic remodeling of the RV in various forms of CHD and explore remodeling trajectories, along with the effects of surgeries and residual lesion repair, in tetralogy of Fallot, Ebstein anomaly, congenitally corrected transposition of the great arteries, transposition of the great arteries with atrial switch surgery, and single ventricle palliated by Fontan. In addition, the role of metabolism, genetic markers, and imaging criteria of RV remodeling are explored. Finally, the optimal timing for addressing residual lesions in CHD through surgery or percutaneous interventions is discussed, along with advanced heart failure management strategies and medical therapy aimed at preventing further RV dilation and/or systolic deterioration or promoting reverse remodeling.

Address: Research Centre, Montréal Heart Institute, Department of Medicine, Université de Montréal, Montréal, Québec, Canada.; Adult Congenital Heart Centre, Montréal Heart Institute, Université de Montréal, Montréal, Québec, Canada.; Research Centre, Montréal Heart Institute, Department of Medicine, Université de Montréal, Montréal, Québec, Canada; Metabolomic Centre, Montréal Heart Institute, Department of Nutrition, Université de Montréal, Montréal, Québec, Canada.; Research Centre, Montréal Heart Institute, Department of Medicine, Université de Montréal, Montréal, Québec, Canada; Adult Congenital Heart Centre, Montréal Heart Institute, Université de Montréal, Montréal, Québec, Canada.; Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.; Research Centre, Montréal Heart Institute, Department of Medicine, Université de Montréal, Montréal, Québec, Canada; Advanced Heart Failure and Transplantation Program Centre, Montréal Heart Institute, Université de Montréal, Montréal, Québec, Canada.; Radiology Department, Montréal Heart Institute, Department of Medicine, Université de Montréal, Montréal, Québec, Canada.; Research Centre, Montréal Heart Institute, Department of Medicine, Université de Montréal, Montréal, Québec, Canada; Adult Congenital Heart Centre, Montréal Heart Institute, Université de Montréal, Montréal, Québec, Canada. Electronic address: [email protected].

DOI: 10.1016/j.cjca.2025.01.005

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MeSH Terms: Ventricular Remodeling, Heart Ventricles, Heart Defects, Congenital, Humans, Metabolomics