**5.3 Heart transplantation as ultimate surgical option in amyloid cardiomyopathy**

Cardiac transplantation is logically the only substantial surgical treatment option for the heart with profound structural alteration due to the vast deposits of amyloid. From methodological point of view, however, principal concerns involve the operative risk of transplant surgery in patients with multiorgan amyloid disease, and the subsequent risk of recurrent amyloidosis in the transplanted graft. The shortage of donor organs is also difficult to ignore.

The first reports of successful heart transplantation for cardiac amyloidosis are dated back to early 80-ties (Conner, Hosenpud et al. 1988; Hall and Hawkins 1994). Mc Gregor reported in 1998 the Mayo Clinic experience (McGregor, Rodeheffer et al. 1998) of 8 patients but the largest cohort was published by Dubrey in 2004 (Dubrey, Burke et al. 2001; Dubrey, Burke et al. 2004) comprising 24 patients transplanted over the period of 18 years. In AL amyloidosis patients, the survival was 50%, 50%, and 20% at 1, 2, and 5 years in those without subsequent chemotherapy contrary to 71%, 71%, and 36% respectively in 7 AL transplanted patients with additional chemotherapy. Without chemotherapy the median of amyloid recurrence in the graft was 11 months. Survival of the 7 non-AL amyloidosis patients was 86%, 86%, and 64% at 1, 2, and 5 years. Overall 5-year survival of all amyloid patients was 38% in contrast to 67% in patients undergoing heart transplantation for other indications (Dubrey, Burke et al. 2004).

Regardless of the relatively small patient cohorts it can be assumed that the outcome of heart transplantation for AL amyloidosis is significantly worse than in general heart transplant population, namely due to progression of the systemic disease (Shah, Inoue et al. 2006; Luo, Chou et al. 2010).

In some clinical settings, heart transplantation may need to be combined with liver or kidney transplantation (Gillmore, Stangou et al. 2001; Schwartz, Kuiper et al. 2007; Audard, Matignon et al. 2009; Baumgratz, Vila et al. 2009). Liver transplantation should be instrumental or potentially curative in familial amyloidosis for abolishment of the aberrant transthyretin production. Scarcity of reported cases preclude consistent conclusion but it seems that precise knowledge of specific transthyretin mutation subtype may help to differentiate the outcome of transplantation strategy (Sharma, Perri et al. 2003).
