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Treatment options for severe cardiac amyloidosis: heart transplantation combined with chemotherapy and stem cell transplantation for patients with al-amyloidosis and heart and liver transplantation for patients with attr-amyloidosis
European Journal of Cardio-Thoracic Surgery, 02/08/08

Sack F-U et al. - Cardiac amyloidosis (CA) is a potentially curative disease after HTx when combined with either chemotherapy and stem cell transplantation (SCT) or LiverTx depending on the type of the amyloidosis. With this approach, excellent survival rates and even remission of the underlying disease is possible.

• Study was undertaken to investigate the outcomes of chemotherapy and stem cell transplantation in AL-amyloidosis, and liver transplantation in TTR-amyloidosis
• Pts (n>60) with AL-amyloidosis and pts (n>25) with ATTR-amyloidosis were investigated

• 18 pts showed signs of end-stage heart failure
• 4 pts died within 1 month after listing for HTx
• 7 pts with AL (mean age 41.8 y) and 5 pts with ATTR-amyloidosis (mean age 42.6 y) were successfully transplanted with an actual survival rate of 91.6%
• 1 patient died 8 mos after HTx due to infection
• 5 AL pts received chemotherapy and SCT and one ATTR patient was liver transplanted
• 3 AL pts showed complete remission of amyloidosis

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