Heart Transplantation in Cardiac Amyloidosis: A Ten Year Experience

Purpose Cardiac amyloidosis (CA) is an infiltrative cardiomyopathy associated with poor prognosis. We sought to summarize the outcomes for patients with light chain (AL) or transthyretin (ATTR) CA receiving heart transplantation at our institution and a collaborating institution. Methods This is a retrospective examination of patients with end-stage CA who underwent orthotopic heart transplantation (OHT) at the Cleveland Clinic in Cleveland, Ohio, and with collaboration at Baylor Scott & White Advanced Heart Failure Clinic in Dallas, Texas, from May 2008 to September 2018. Results OHT was performed in 14 cases of CA, twelve at Cleveland Clinic and two at Baylor Scott & White Advanced Heart Failure Clinic. Twelve patients had ATTR (3 wild type, 9 hereditary) and 2 patients AL. Twelve patients have completed one-year follow-up and 100% of patients are alive with a median post-transplant survival of 3 years (range 0.1-10.5 years). Median time from amyloidosis diagnosis to OHT was 16 months (IQR, 9-29 months). Median time awaiting transplant was 5 months (IQR, 1-8 months). Four patients received a heart-multiorgan transplantation: one patient with AL received a kidney and three patients with hereditary ATTR (hATTR) due to the T60A mutation received a liver. One patient with AL underwent autologous stem cell transplantation 6 months after OHT and is stable at 4.2 years follow-up. Six patients with hereditary ATTR due to the V122I mutation underwent isolated OHT; two with long-term follow-up of 7.2 and 10.3 years respectively. Three patients displayed presumed signs of extra-cardiac amyloid involvement post-transplantation: an hATTR V122I patient required spinal stenosis surgery 2 years after transplant, a wild-type ATTR patient required spinal stenosis surgery 10 years after transplant, and an hATTR T60A patient developed chronic diarrhea 9 years after combined heart-liver transplant. No patient had evidence of recurrent amyloid deposition in the cardiac allograft. Conclusion Our experience confirmed that heart transplantation is a viable long-term treatment option for select patients with CA. Post OHT, presumed extra-cardiac involvement of amyloidosis occurred in 3 ATTR patients, but no patients showed re-emergence of CA in their recipient organ.