The right ventricle in tetralogy of Fallot: adaptation to sequential loading

Frontiers in Pediatrics

Pediatrics

Lotte M. Zandbergen¹  Daphne Merkus²  Yannick J. H. J. Taverne³  Willem A. Helbing⁴  Beatrijs Bartelds⁴  Wouter J. van Genuchten⁴  Rahi S. Alipour Symakani⁵  Surya Henry⁶ 

[1] Department of Cardiology, Division of Experimental Cardiology, Erasmus Medical Center, Rotterdam, Netherlands;Walter Brendel Center of Experimental Medicine (WBex), University Clinic Munich, Munich, Germany;Department of Cardiology, Division of Experimental Cardiology, Erasmus Medical Center, Rotterdam, Netherlands;Walter Brendel Center of Experimental Medicine (WBex), University Clinic Munich, Munich, Germany;German Center for Cardiovascular Research (DZHK), Partner Site Munich, Munich Heart Alliance (MHA), Munich, Germany;Department of Cardiothoracic Surgery, Erasmus Medical Center, Rotterdam, Netherlands;Department of Pediatrics, Division of Pediatric Cardiology, Erasmus Medical Center, Sophia Children’s Hospital, Rotterdam, Netherlands;Department of Pediatrics, Division of Pediatric Cardiology, Erasmus Medical Center, Sophia Children’s Hospital, Rotterdam, Netherlands;Department of Cardiology, Division of Experimental Cardiology, Erasmus Medical Center, Rotterdam, Netherlands;Department of Cardiothoracic Surgery, Erasmus Medical Center, Rotterdam, Netherlands;Department of Pediatrics, Division of Pediatric Cardiology, Erasmus Medical Center, Sophia Children’s Hospital, Rotterdam, Netherlands;Department of Cell Biology, Erasmus Medical Center, Rotterdam, Netherlands;

关键词: tetralogy of Fallot;    right ventricular dysfunction (RV dysfunction);    myocardial adaptation;    pulmonary regurgitation;    congenital heart disease;    ventricular hypertrophy;    animal models;   

DOI  :  10.3389/fped.2023.1098248

 received in 2022-11-14, accepted in 2023-02-27,  发布年份 2023

来源: Frontiers

【 摘 要 】

Right ventricular dysfunction is a major determinant of outcome in patients with complex congenital heart disease, as in tetralogy of Fallot. In these patients, right ventricular dysfunction emerges after initial pressure overload and hypoxemia, which is followed by chronic volume overload due to pulmonary regurgitation after corrective surgery. Myocardial adaptation and the transition to right ventricular failure remain poorly understood. Combining insights from clinical and experimental physiology and myocardial (tissue) data has identified a disease phenotype with important distinctions from other types of heart failure. This phenotype of the right ventricle in tetralogy of Fallot can be described as a syndrome of dysfunctional characteristics affecting both contraction and filling. These characteristics are the end result of several adaptation pathways of the cardiomyocytes, myocardial vasculature and extracellular matrix. As long as the long-term outcome of surgical correction of tetralogy of Fallot remains suboptimal, other treatment strategies need to be explored. Novel insights in failure of adaptation and the role of cardiomyocyte proliferation might provide targets for treatment of the (dysfunctional) right ventricle under stress.

【 授权许可】

Unknown   
© 2023 Alipour Symakani, van Genuchten, Zandbergen, Henry, Taverne, Merkus, Helbing and Bartelds.