title: Outcomes of heart transplantation for cardiac amyloidosis: subanalysis of the spanish registry for heart transplantation.
abstract: Amyloidosis (Am), a systemic disease, has poor prognosis because of organ damage produced by protein deposition in the extracellular space. Although heart transplantation (HTx) is possible, donor availability concerns and high mortality make this approach controversial. The Spanish Registry for Heart Transplantation includes 25 Am patients (54 +/- 9 years): 13 with AL type, 2 with AA and 10 with TTR mutation. Fifteen patients (60%) died during follow-up (4.9 +/- 1.3 years): 9 AL-Am patients, both AA-Am patients and 4 with TTR-Am. HTx survival for Am patients was similar to patients without Am at 1 month but significantly worse at 5 years: 46% versus 78% (p < 0.02). Of 10 AL-Am patients undergoing successful HTx, 4 died of systemic Am. Stem cell transplantation was performed in 3 (1 died of acute rejection). Five of 10 patients with TTR-Am underwent liver transplant; 4 remained alive at the last follow-up. Findings include poor outcome for AL-Am patients despite HTx and better survival for TTR-Am patients if HTx is associated with liver transplantation. Given the shortage of donors and poor outcome for Am patients, we would recommend that HTx be reserved for patients without or with mild systemic Am and be supplemented by additional therapies as indicated.
authors.affiliation: Hospital Clínic, Barcelona, Spain. eroig@clinic.ub.es
authors.forename: E
authors.initials: E
authors.lastname: Roig
authors.forename: L
authors.initials: L
authors.lastname: Almenar
authors.forename: F
authors.initials: F
authors.lastname: González-Vílchez
authors.forename: G
authors.initials: G
authors.lastname: Rábago
authors.forename: J
authors.initials: J
authors.lastname: Delgado
authors.forename: M
authors.initials: M
authors.lastname: Gómez-Bueno
authors.forename: M G
authors.initials: MG
authors.lastname: Crespo-Leiro
authors.forename: J M
authors.initials: JM
authors.lastname: Arizón
authors.forename: L
authors.initials: L
authors.lastname: de la Fuente
authors.forename: N
authors.initials: N
authors.lastname: Manito
mesh_terms: D000328:Adult; D000368:Aged; D000682:Amyloid / Q000235:genetics; D000686:Amyloidosis / Q000401:mortality / Q000601:surgery*; D009202:Cardiomyopathies / Q000401:mortality / Q000601:surgery*; D005260:Female; D016027:Heart Transplantation / Q000401:mortality*; D006801:Humans; D008297:Male; D008875:Middle Aged; D011228:Prealbumin / Q000235:genetics; D012042:Registries; D012189:Retrospective Studies; D013030:Spain; D016019:Survival Analysis; D014019:Tissue Donors / Q000600:supply & distribution; D016896:Treatment Outcome
chemical_list: D000682:Amyloid; D011228:Prealbumin; C076571:transthyretin-related amyloid fibril protein, human