Tetralogy of Fallot across the lifespan: a focus on the right ventricle
File(s)
Author(s)
Type
Journal Article
Abstract
Tetralogy of Fallot is a cyanotic congenital heart disease, for which various surgical techniques allow patients to survive to adulthood. Currently, the natural history of corrected tetralogy of Fallot is underlined by progressive right ventricular (RV) failure due to pulmonic regurgitation and other residual lesions. The underlying cellular mechanisms that lead to RV failure from chronic volume overload are characterized by microvascular and mitochondrial dysfunction through various regulatory molecules. On a clinical level, these cardiac alterations are commonly manifested as exercise intolerance. The degree of exercise intolerance can be objectified and aid in prognostication through cardiopulmonary exercise testing. The timing for reintervention on residual lesions contributing to RV volume overload remains controversial; however, interval assessment of cardiac function and volumes by echocardiography and magnetic resonance imaging may be helpful. In patients who develop clinically important RV failure, clinicians should aim to maintain a euvolemic state through the use of diuretics while paying particular attention to preload and kidney function. In patients who develop signs of cardiogenic shock from right heart failure, stabilization through the use of inotropes and pressor is indicated. In special circumstances, the use of mechanical support may be appropriate. However, cardiologists should pay particular attention to residual lesions that may impact the efficacy of the selected device.
Date Issued
2023-12-01
Date Acceptance
2023-10-17
Citation
CJC Pediatric and Congenital Heart Disease, 2023, 2 (6, Part A), pp.283-300
ISSN
2772-8129
Publisher
Elsevier BV
Start Page
283
End Page
300
Journal / Book Title
CJC Pediatric and Congenital Heart Disease
Volume
2
Issue
6, Part A
Copyright Statement
© 2023 The Author(s). Published by Elsevier Inc. on behalf of the Canadian Cardiovascular Society. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Identifier
https://www.ncbi.nlm.nih.gov/pubmed/38161676
S2772-8129(23)00160-4
Publication Status
Published
Coverage Spatial
United States
Date Publish Online
2023-10-20