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Heart Transplantation in Cardiac Amyloidosis
Quick Takes
- The outcomes in carefully selected patients with cardiac amyloid are not dissimilar to those with nonamyloid cardiomyopathy.
- Patients with smoldering myeloma or AL amyloidosis with limited gastrointestinal, kidney, or bone marrow involvement need NOT be automatically disqualified from transplantation if noncardiac organ dysfunction is not thought to be life-limiting.
- Studies with larger cohorts are the next step to confirm these findings.
Study Questions:
What are the outcomes after heart transplantation in patients with cardiac amyloidosis?
Methods:
The study authors evaluated all patients treated between 2004 and 2017 at a major academic center for amyloid cardiomyopathy, and who ultimately underwent heart transplantation. Data were collected retrospectively via chart review. They examined pre-transplantation characteristics and post-transplantation outcomes in this group compared with the overall transplantation population at their center. Patients were evaluated for postoperative complications, including bleeding, renal failure, rejection, infection, malignancy, graft failure, and mortality. The authors divided patients with amyloid cardiomyopathy into two groups: those with light chain (AL) amyloidosis and those with transthyretin (ATTR) amyloidosis. Survival curves for patients who underwent cardiac transplantation for amyloid and nonamyloid cardiomyopathy were then derived using the Kaplan-Meier method. They compared unadjusted survival rates by log-rank analysis.
Results:
During the study period, 31 patients (13 with AL amyloidosis and 18 with ATTR amyloidosis) underwent heart transplantation. Of the patients with AL amyloidosis, two had Mayo stage II disease, five had Mayo stage III disease, and six had Mayo stage IV disease. Patients with ATTR amyloidosis were older, were more likely to be male, had worse baseline renal function, and had longer waitlist times compared with both patients with AL amyloidosis and the overall transplantation population. Median waitlist times did not differ between patients who underwent heart transplantation for cardiac amyloidosis (42 days) and noncardiac amyloidosis indications (42 days). Post-transplantation, there were no differences in postoperative bleeding, renal failure, infection, rejection, or malignancy. There were four deaths after transplantation, but no deaths were attributable to patients’ amyloidosis or amyloidosis therapy complications. There was no significant difference in mortality between patients who underwent heart transplantation for amyloid cardiomyopathy and patients who underwent heart transplantation for all other indications. Less than one-half of AL patients who underwent heart transplantation for cardiac AL amyloidosis underwent subsequent stem cell transplantation (SCT), all of whom required proteasome-based therapy after SCT for ongoing pathologic light chain control.
Conclusions:
The study authors concluded that in carefully selected patients with cardiac amyloidosis, heart transplantation can be an effective therapeutic option with outcomes similar to those transplanted for other causes of heart failure.
Perspective:
Although this is a single-center study, the findings are important because it suggests that in carefully selected patients with amyloidosis, heart transplantation is beneficial. An important take-away is that patients with smoldering myeloma or AL amyloidosis with limited gastrointestinal, kidney, or bone marrow involvement need not be disqualified from transplantation if noncardiac organ dysfunction is not thought to be life-limiting. As the study authors point out, cardiac amyloidosis is now designated as status IV under the new (in 2018) listing criteria, and therefore, it remains to be seen how these new criteria will affect transplantation wait times and cardiac outcomes for patients with cardiac amyloidosis.
Clinical Topics: Cardiac Surgery, Cardio-Oncology, Heart Failure and Cardiomyopathies, Invasive Cardiovascular Angiography and Intervention, Cardiac Surgery and Heart Failure, Acute Heart Failure, Heart Transplant
Keywords: Amyloidosis, Bone Marrow, Cardiomyopathies, Cardiotoxicity, Graft Rejection, Heart Failure, Heart Transplantation, Kidney Diseases, Multiple Organ Failure, Postoperative Complications, Stem Cell Transplantation, Renal Insufficiency
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