Congenital Heart Disease and Systemic Right Ventricular Function
Summary
Congenital heart disease encompasses a spectrum of structural cardiac anomalies present at birth. Among these, transposition of the great arteries (TGA) corrected by an atrial switch procedure and congenitally corrected transposition of the great arteries (ccTGA) place the morphologically right ventricle in the systemic circulation. The systemic right ventricle (sRV) is not optimally designed to withstand systemic pressures over a lifetime, and it often undergoes progressive remodelling, fibrosis and decline in contractile function. Late complications include heart failure, systemic atrioventricular valve regurgitation, arrhythmias and sudden cardiac death. Lifelong surveillance is essential, combining imaging modalities such as echocardiography and cardiac MRI with biomarkers and functional testing. Therapeutic strategies range from surgical or percutaneous valve interventions to medical therapies targeting neurohormonal activation, yet evidence for optimal management remains limited by the rarity and heterogeneity of these patient cohorts.
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Congenital Heart Disease and Systemic Right Ventricular Function publication trend
The graph below shows the total number of articles in congenital heart disease and systemic right ventricular function across all publications each year (not limited to Nature Index journals).
Technical terms
Systemic right ventricle (sRV): A morphologic right ventricle supporting systemic circulation, as seen in atrial switch and ccTGA.
Congenitally corrected transposition of the great arteries (ccTGA): A rare malformation with atrioventricular and ventriculoarterial discordance, placing the right ventricle in systemic position without surgical redirection.
Atrial switch operation: A surgical procedure (Mustard or Senning) that redirects venous return to correct TGA, resulting in the right ventricle serving the systemic circulation.
Ejection fraction: The percentage of ventricular blood volume ejected per heartbeat; a key measure of systolic function.
Global longitudinal strain (GLS): A speckle tracking-derived index of myocardial deformation; reduced GLS indicates impaired longitudinal contractility.
Speckle tracking echocardiography (STE): An imaging technique that tracks natural acoustic markers to quantify myocardial strain and deformation.
References
- Overview of Long-Term Outcome in Adults with Systemic Right Ventricle and Transposition of the Great Arteries: A Review. Diagnostics (2023).
- Multi‐modality imaging of the systemic right ventricle in congenital heart disease. Echocardiography (2024).
- Heart failure in systemic right ventricle: Mechanisms and therapeutic options. Frontiers in Cardiovascular Medicine (2023).
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