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Management of Systemic Right Ventricular Failure in Patients With Congenitally Corrected Transposition of the Great
Aleksei A Filippov1, Pedro J Del Nido1, Nikolay V Vasilyev2
1From Department of Cardiac Surgery, Boston Children's Hospital, Harvard Medical School, Boston, MA.
Circulation
|October 26, 2016
Summary
Congenitally corrected transposition of the great arteries (ccTGA) management focuses on preventing systemic right ventricle (RV) failure. Current strategies include surgical repair in youth and palliative or transplant options for adults with RV dysfunction.
Area of Science:
- Cardiology
- Congenital Heart Disease
- Cardiac Surgery
Background:
- Congenitally corrected transposition of the great arteries (ccTGA) presents long-term challenges, primarily systemic right ventricle (RV) dysfunction and failure.
- While anatomic repair offers benefits in pediatric populations, it is not suitable for all patients, especially adolescents and adults.
- Systemic RV failure is a significant cause of mortality and morbidity in individuals with ccTGA.
Purpose of the Study:
- To review current surgical, medical, and interventional strategies for managing systemic RV failure in ccTGA patients.
- To highlight challenges in preventing and treating RV dysfunction beyond adolescence.
- To discuss palliative options and heart transplantation for advanced RV failure in adults with ccTGA.
Main Methods:
- This is a review article synthesizing current knowledge on ccTGA management.
- It analyzes surgical, palliative, and transplant-related interventions.
- Focus is on strategies applicable to both pediatric and adult ccTGA populations.
Main Results:
- Anatomic repair is effective in younger patients but has limitations for older individuals.
- Palliative options like cardiac resynchronization therapy and ventricular assist devices can improve function and delay progression in non-surgical candidates.
- Heart transplantation remains the sole long-term solution for severe RV failure in adults, limited by organ availability.
Conclusions:
- Managing systemic RV failure in ccTGA requires tailored strategies based on age and disease severity.
- Palliative interventions play a crucial role in improving quality of life and delaying advanced therapies.
- Further research and improved donor organ availability are needed for long-term management of adult ccTGA patients with RV failure.
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