With a minimal incidence of amyloid recurrence in the cardiac allograft, cardiac transplantation is well tolerated, increases functional ability, and lengthens survival in patients with ATTR-CM, according to a 30-year retrospective study. ATTR-CM should be included in the list of transplantation indications.
Cardiomyopathy caused by transthyretin amyloidosis cardiomyopathy (ATTR-CM) is progressive and fatal. Patients with severe heart failure have only cardiac transplantation (CT) as a treatment option. Despite a few modest case studies showing similar CT results for patients with ATTR-CM and non-amyloid cardiomyopathies, several centers still view ATTR-CM as a complete contraindication to CT. A presumed risk of amyloid recurrence in the cardiac allograft is one reason for this. This retrospective study was published in the European Heart Journal and discussed the outcomes of patients with ATTR-CM evaluated over the previous 30 years who underwent CT at the UK National Amyloidosis Centre.
All ATTR-CM patients who received CT and were checked between 1990 and 2020 were retrospectively evaluated. A sizable cohort of ATTR-CM patients who were not transplanted was used to compare outcomes and identify pre-transplantation illness and patient characteristics.
The study observed 11 ATTR-CM patients. Nine were males and eight had wild-type ATTR-CM. The median follow-up after a CT was 65.7 months, and the median age at the CT was 60.3 years. Pre-transplant, the median (range) NT-proBNP concentration was 4478 ng/L (1057–8778 ng/L), the median (range) LVEF was 39% (27–56%), and the mean (IQR) interventricular septal diameter was 18 mm (15.9–20.1 mm). Eight individuals had NYHA functional class III status; the other three had class II status.
The longest surviving patient was >19 years after the CT, and the one, three, and five-year survival rates were 100%, 89%, and 86%, respectively. For all non-amyloid CT patients, survival is at least equivalent to UK and US CT outcome registry data. Endomyocardial biopsies and/or Tc-DPD scintigraphy were used to determine whether any patients had experienced a recurrence of amyloid in the cardiac allograft. Two patients were started on Patisiran for amyloid polyneuropathy at the 5th and 211th month post-CT. Two patients experienced graft rejection that required treatment; both patients were successfully treated with intravenous steroids. Six patients had chronic kidney disease.
Three individuals passed away, including one with ATTRv-CM, who died from leptomeningeal amyloidosis-related complications. Regardless of the stage of NAC ATTR illness, including those diagnosed younger than 65 years of age (P = 0.028), survival within the cohort of patients who received CT was considerably longer than that of UK individuals with ATTR-CM, generally (P = 0.006).
This study concluded that cardiac transplantation is well tolerated, improves functional ability, and lengthens survival in ATTR-CM with a low chance of amyloid recurrence in the cardiac allograft. Moreover, it is also suggested that ATTR-CM should be routinely included in the cardiac transplant indications list.
Razvi, Y., Patel, R., Ioannou, A., Rauf, M. U., Masi, A., Porcari, A., . . . Gillmore, J. D. (2022). Cardiac transplantation in transthyretin amyloid cardiomyopathy: outcomes from three decades of tertiary centre experience. European Heart Journal, 43(Supplement_2). doi:10.1093/eurheartj/ehac544.1784