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Updated: Mar 6, 2026
Implantation of Total Artificial Heart in Congenital Heart Disease
Published on: July 18, 2014
Total Artificial Heart Implantation as a Bridge to Heart Transplantation in an Active Duty Service Member With
Michael S Scully1, Dylan E Wessman1, James M McKee2
1Department of Cardiology, Naval Medical Center San Diego, 34730 Bob Wilson Drive, Suite 303, San Diego, CA 92134-3303.
Insights
This case report highlights a successful total artificial heart transplant as a bridge to heart and stem cell transplant for a patient with advanced cardiac amyloidosis. This approach offers a potential lifeline for patients with this severe heart condition.
Area of Science:
- Cardiology
- Hematology
- Transplant Surgery
Background:
- Cardiac amyloidosis, a complication of light-chain (AL) amyloidosis, affects up to 50% of patients with primary AL amyloidosis.
- Amyloid cardiomyopathy (CM) historically has a poor prognosis, with 1-year survival rates of 35-40% and heart transplantation considered controversial.
- The systemic nature of AL amyloidosis and poor survival rates have made cardiac transplantation a challenging option.
Observation:
- A 47-year-old active duty male presented with atypical chest pain, diagnosed with advanced cardiac amyloidosis.
- The patient experienced rapidly progressive left ventricular dysfunction, heart failure, and cardiac arrest.
- He underwent a total artificial heart as a bridge to orthotopic heart and kidney transplantation, followed by autologous stem cell transplant.
Findings:
- The patient received a total artificial heart, followed by heart and kidney transplantation, and autologous stem cell transplant.
- He is currently in remission with good functional capacity, able to perform daily activities and moderate exercise.
- This case demonstrates the feasibility of advanced heart failure management in severe cardiac amyloidosis.
Implications:
- Modern advancements in chemotherapy and heart failure management are improving outcomes for select AL amyloid CM patients undergoing transplantation.
- Total artificial heart as a bridge therapy can be a viable option for critically ill patients with advanced cardiac amyloidosis.
- Ongoing research aims to further improve treatment strategies and outcomes for this life-threatening disease.
Introduction:
Cardiac involvement by light-chain (AL) amyloid occurs in up to 50% of patients with primary AL amyloidosis. The prognosis of amyloid heart disease is poor with 1-year survival rates of 35 to 40%. Historically, heart transplantation was considered controversial for patients with AL amyloid cardiomyopathy (CM) given the systemic nature of the disease and poor survival. We present a case report of an active duty service member diagnosed with advanced cardiac amyloid who underwent total artificial heart transplant as a bridge to heart transplant and eventual autologous stem cell transplant.
Case Report:
A 47-year-old active duty male initially evaluated for atypical chest pain was found to have severe concentric left ventricular hypertrophy on echocardiogram but normal voltage on electrocardiogram. Cardiac magnetic resonance imaging, laboratory studies, and bone marrow biopsy established the diagnosis of cardiac amyloidosis. At the time of diagnosis, the patient's prognosis was very poor with a median survival of 5 months on the basis of the Mayo Clinic revised prognostic staging system for amyloidosis. The patient developed rapidly progressive left ventricular dysfunction and heart failure leading to cardiac arrest. The patient received a total artificial heart as a bridge to orthotopic heart and kidney transplantation and eventual stem cell transplant. He continues to be in remission and has a fair functional capacity without restriction in activities of daily living or moderate exercise.
Discussion:
Amyloid CM is a rare and devastating disease. The natural course of the disease has made heart transplant in these patients controversial. Modern advancements in chemotherapies and advanced heart failure treatments have improved outcomes for select patients with AL amyloid CM undergoing heart transplantation. There is ongoing research seeking improvement in treatment options and outcomes for patients with this deadly disease.
