Abstract 3339: Treatment of Choice 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
Objectives: Cardiac amyloidosis (CA) is associated with a poor prognosis and a survival rate of less than 30 % after two years of clinical manifestation. Considered as a semi-malignant disease, CA is often a contraindication for HTx. However, based on the type of CA, there are excellent treatment options in combination with HTx. For AL-amyloidosis, chemotherapy and stem cell transplantation, for TTR-amyloidosis, in which the liver is the source of the pathologic protein, liver transplantation is therapy of choice after Htx. Methods: Until 2005 more than 56 pts with ALL and more than 20 pts. with TTR amyloidosis have been investigated in our center. 14 patients showed signs of end stage heart failure. Results: Four patients died while less than one month on the heart waiting list. 5 patients with AL (mean age 41.8 years) and 6 patients with TTR-amyloidosis (mean age 42.6 years) were successfully transplanted with a one year survival of 89 %. One patient died 8 months after htx due to infection. 3 AL-pts. received chemotherapy and PBSCT and 2 TTR-pts. were liver transplanted. One AL patient showed complete remission of amyloidosis after one year. Conclusions: Cardiac amyloidosis is a potentially curative disease in combination of Htx with either chemotherapy and PBSCT or LTx depending of the type of the amyloidosis. Due to the natural course of the disease, urgent Htx after cardiac manifestation is mandatory. With this approach, excellent survival rates and even remission of the underlying disease is possible.