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Late deaths after Fontan procedure: the next frontier
Shuhua Luo1,2, Osami Honjo1,2
1Division of Cardiovascular Surgery, Labatt Family Heart Centre, The Hospital for Sick Children.
Current Opinion in Cardiology
|December 22, 2018
Summary
The Fontan operation offers excellent medium-term survival for single ventricle physiology. However, long-term Fontan circulation faces challenges due to inherent limitations, impacting adult patient outcomes.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Adult Congenital Heart Disease
Background:
- The Fontan operation has been a cornerstone in treating single ventricle physiology for over 40 years.
- Contemporary surgical strategies have led to excellent medium-term survival rates.
- However, the Fontan circulation presents fundamental physiologic limitations leading to late failure and death.
Purpose of the Study:
- To review the challenges and potential opportunities in managing failing Fontan circulation.
- To discuss current and emerging therapeutic strategies for long-term Fontan patient care.
- To highlight the growing population of adolescents and adults living with Fontan circulation.
Main Methods:
- Literature review focusing on medical therapy, imaging, and interventions for lymphatic abnormalities.
- Exploration of transplantation and mechanical support options for Fontan failure.
- Summary of quality of life outcomes in Fontan survivors.
Main Results:
- The review identifies key challenges in managing late Fontan failure.
- Potential opportunities include advancements in medical therapy, diagnostic imaging, and novel interventions.
- Quality of life remains a critical consideration for this growing population.
Conclusions:
- Improving long-term outcomes for Fontan circulation requires advancements in surgical techniques and therapeutic tools.
- A deeper understanding of failing Fontan physiology is crucial.
- Addressing the unique needs of the aging Fontan population is paramount.
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