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Right Ventricular Remodeling and Function in Hypoplastic Left Heart Syndrome.
Thomas Salaets1,2, Marc Gewillig1, Alexander Van De Bruaene3
1Division of Pediatric Cardiology, University Hospitals Leuven, Leuven, Belgium.
JACC. Advances
|December 5, 2024
Summary
The right ventricle (RV) in hypoplastic left heart syndrome (HLHS) adapts to systemic pumping, facing remodeling that can cause dysfunction. Understanding RV changes is key for preserving heart health after Fontan completion.
Area of Science:
- Cardiology
- Pediatric Cardiac Surgery
- Congenital Heart Disease Research
Background:
- The right ventricle (RV) in hypoplastic left heart syndrome (HLHS) assumes systemic pumping function, adapting to altered volume loads.
- RV remodeling in HLHS is influenced by anatomical subtype, surgical strategy, tricuspid regurgitation, recoarctation, and genetics.
- Maladaptive remodeling can lead to systolic and diastolic dysfunction, impacting patient outcomes.
Purpose of the Study:
- To review recent empirical observations on RV remodeling and function in HLHS.
- To enhance understanding of the RV's physiology in patients with HLHS.
- To identify potential areas for future research in myocardial health preservation.
Main Methods:
- Comprehensive literature review of recent empirical observations.
- Synthesis of data on RV size, function, and remodeling in HLHS.
- Analysis of factors influencing RV adaptation and dysfunction.
Main Results:
- RV systolic dysfunction predicts pre-Fontan mortality.
- Progressive diastolic dysfunction post-Fontan is increasingly recognized as significant.
- RV remodeling is a complex process influenced by multiple clinical and genetic factors.
Conclusions:
- Understanding RV remodeling and function is crucial for managing HLHS patients.
- Diastolic dysfunction plays a critical role in long-term outcomes after Fontan completion.
- Further research is needed to optimize assessment and preservation of RV myocardial health.
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