Single or combined cardiac transplantation for Cardiac Amyloidosis. A report from the French National Referral Center for Cardiac Amyloidosis

Abstract

Cardiac Amyloidosis (CA) is caused by the extracellular accumulation of insoluble fibrillar proteins. The most common types of CA are AL amyloidosis (light-chain), transthyretin amyloidosis: Hereditary (hTTR) or wild-type (wTTR). Without treatment and adequate care management, the prognosis for CA is poor. Heart transplantation is remains the final cardiac therapeutic option. This is a retrospective study that included all patients who had a heart transplant for CA from 2005 to 2018 ( n = 18). We report the baseline and post-transplant data follow-up. Eighteen patients had a heart transplant: 13 (72%) had AL amyloidosis and 5 had hTTR (28%). The average age was 56 years (43 to 66 years), there were 8 women (44%). Four patients had combined heart and liver transplant (all of them were hTTR). Four patients had combined heart and kidney transplant (all of them were AL). All AL patients received at least one cycle of chemotherapy before the surgery and 8 (62%) were in partial remission. Six patients (33%) were urgently transplanted. The 30-day survival is 100%, the one-year survival was 75%. The deaths occurred only in AL: 3 died from infection and 1 from severe hemorrhage (scarpa rupture), of which one with heart and kidney. No deaths at 1 year were associated with a recurrence of amyloidosis. In most AL with partial remission before the surgery, a slight decrease of light-chains under immunosuppressive treatments was observed. Heart transplant is a therapeutic option for selected CA in AL and hTTR and can be combined with kidney or liver transplant. In severe AL CA cardiac transplant need to perform before expecting the complete remission as immunosuppressive therapy may stabilize or decrease the light-chain after the transplant. The survival of transplant CA patients is comparable to that of non-AC patients, and may have better survival than those with a single heart transplant.