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Heart transplantation in patients with amyloidosis: single-center experience
A Alloni1, C Pellegrini, T Ragni
1Cardiac Surgery Department, IRCCS Policlinico S Matteo, Pavia, Italy. alealloni@hotmail.com
Insights
Heart transplantation for amyloidosis shows encouraging midterm survival, potentially halting organ damage in select cases. Careful follow-up is crucial due to cardiac events being a primary cause of death.
Area of Science:
- Cardiology
- Transplantation Medicine
- Amyloidosis Research
Background:
- Amyloidosis is a systemic disease often considered a contraindication for heart transplantation.
- This study evaluates outcomes for heart failure patients with amyloidosis undergoing evaluation for cardiac transplantation since 1991.
Purpose of the Study:
- To analyze the outcomes of patients with amyloidosis awaiting heart transplantation.
- To assess the effectiveness of heart transplantation as a therapy for cardiac amyloidosis.
Main Methods:
- Eight patients with amyloidosis (AL lambda, APO A1, TTR types) were evaluated and listed for heart transplant since 1991.
- Four patients received heart transplants, three died while awaiting donors, and one was recently transplanted.
Main Results:
- Five amyloidosis patients (0.7%) underwent heart transplantation out of 713 total transplants since 1985.
- Two transplanted patients survived long-term (60 and 41 months) with no cardiac infiltration; one had a combined heart-liver transplant.
- Two patients died post-transplant: one from sudden death with graft infiltration, another from multiple organ failure due to systemic disease progression.
Conclusions:
- Heart transplantation may halt organ damage in isolated cardiac amyloidosis, though definitive conclusions require larger studies.
- Cardiac events remain the leading cause of mortality.
- Long-term follow-up is essential to monitor systemic disease progression; midterm survival is promising.
Introduction:
Amyloidosis is a systemic disease. Heart transplantation in this subset of patients is contraindicated by the majority of authors. In our center, patients with heart failure due to amyloidosis have been evaluated for cardiac transplantation since 1991. The aim of this study was to analyze the outcome of these patients waiting for transplant and the effectiveness of this therapy.
Materials And Methods:
Since 1991, eight patients affected by amyloidosis have been evaluated and enrolled on the waiting list for transplant: five affected by AL lambda type; two by APO A1; and one by TTR. Four were transplanted, three died waiting for a donor (two from cardiac failure, one from sudden death), and one has been recently transplanted after 17 months on waiting list.
Results:
Since 1985, 713 patients underwent heart transplantation in our center, five of whom were affected by amyloidosis (0.7%). Two are still alive (60 and 41 months) without evidence of cardiac amyloidotic infiltration. One patient recently underwent a combined heart-liver transplantation. Two patients died after the intervention: one sudden death after 23 months with amyloidotic infiltration of transplanted heart, and one multiple organ failure (MOF) due to progression of the systemic disease.
Conclusions:
Despite the small size of the group preventing us from drawing definitive conclusion, heart transplantation may prevent therapy to arrest organ damage in patients with isolated cardiac involvement. Cardiac events are the main cause of death. Patients must be followed-up for evolution of systemic disease. The midterm survival is encouraging.
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