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Published on: September 13, 2014
Orthotopic Heart and Combined Heart Liver Transplantation: the Ultimate Treatment Option for Failing Fontan
Leigh C Reardon1,2, Jeannette P Lin1, Glen S VanArsdell1,2,3
1Ahmanson/UCLA Adult Congenital Heart Disease Center, Division of Adult Cardiology, 100 UCLA Medical Plaza Suite 630E, Los Angeles, CA 90095 USA.
Insights
Failing Fontan physiology presents long-term challenges due to central venous hypertension. Heart and liver transplantation offer potential solutions for these complex congenital heart cases.
Area of Science:
- Cardiology
- Congenital Heart Disease
- Transplantation
Background:
- Single ventricle physiology involves rare congenital cardiac abnormalities requiring surgical palliation.
- The Fontan operation, a key intervention, leads to chronic central venous hypertension and multi-organ issues.
- Atrial arrhythmias can further complicate single ventricle physiology.
Purpose of the Study:
- To provide a comprehensive update on failing Fontan physiology.
- To review the role of heart and combined heart and liver transplantation in managing Fontan patients.
- To detail the challenges and opportunities of transplantation in this population.
Main Methods:
- Review of current literature and clinical experience.
- Analysis of outcomes for heart and liver transplantation in Fontan patients.
- Expert insights from the Ahmanson/UCLA Adult Congenital Heart Center.
Main Results:
- Fontan-associated long-term complications include central venous hypertension and multi-organ dysfunction.
- Patients with single ventricle physiology are among the most challenging transplant candidates.
- Transplantation strategies are evolving to address these complex cases.
Conclusions:
- Heart and liver transplantation represent critical therapeutic options for failing Fontan physiology.
- Careful patient selection and management are essential for successful transplantation outcomes.
- The review highlights the expertise required for managing these complex congenital cardiac cases.
Purpose Of The Review:
This is a comprehensive update on failing Fontan physiology and the role of heart and combined heart and liver transplantation in the current era.
Recent Findings:
Single ventricle physiology encompasses a series of rare congenital cardiac abnormalities that are characterized by absence of or hypoplasia of one ventricle. This effectively results in a single ventricular pumping chamber. These abnormalities are rarely compatible with long-term survival if left without surgical palliation in the first few years of life. Surgical treatment of single ventricle physiology has evolved over the past 60 years and is characterized by numerous creative innovations. These include the development of arteriopulmonary shunts, the evolution of partial cavopulmonary connections, and the eventual development of the "Fontan" operation. Regardless of the type of Fontan modification, the long-term consequences of the Fontan operation are predominantly related to chronic central venous hypertension and the multi-organ consequences thereof. Atrial arrhythmias can further compromise this circulation.Patients with single ventricle physiology represent a special sub-segment of congenital cardiac transplants and are arguably the most challenging patients considered for transplantation.
Summary:
This review describes in detail the challenges and opportunities of heart and liver transplantation in Fontan patients, as viewed and managed by the experienced team at the Ahmanson/UCLA Adult Congenital Heart Center.
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