title: Heart transplantation in a 68-year-old patient with senile systemic amyloidosis.
abstract: Senile systemic amyloidosis (SSA) results from deposition, predominantly in the heart, of amyloid fibrils derived from wild-type transthyretin (T TR) molecules. Cardiac autopsies indicate that SSA progressively increases in subjects 80 years of age and older. However, only a few cases of patients with SSA and cardiac failure have been recognized by cardiac biopsies during life. Here, we report a case of heart transplantation in a 68-year-old male patient with SSA. After cardiopulmonary resuscitation in October 1998, he underwent complete evaluation. Myocardial biopsies revealed the presence of amyloid deposition. Immunohistochemical staining of the amyloid indicated T TR. Genomic DNA analysis of the T TR exons did not result in any identification of a mutation. In 2001, heart transplantation was performed because progressive heart failure occurred. At the 1-year follow-up, no amyloid deposits were found in the donor heart. At the 2-year follow-up, the patient's physical and mental health was excellent. We conclude that heart transplantation can be an effective treatment in progressive heart failure due to SSA.
authors.affiliation: Heart Center North-Rhine Westfalia, Department of Cardio-Thoracic Surgery, Ruhr University of Bochum, Bad Oeynhausen, Germany. ufuchs@hdz-nrw.de
authors.forename: Uwe
authors.initials: U
authors.lastname: Fuchs
authors.forename: Armin
authors.initials: A
authors.lastname: Zittermann
authors.forename: Ole
authors.initials: O
authors.lastname: Suhr
authors.forename: Gösta
authors.initials: G
authors.lastname: Holmgren
authors.forename: Gero
authors.initials: G
authors.lastname: Tenderich
authors.forename: Kazutomo
authors.initials: K
authors.lastname: Minami
authors.forename: Reiner
authors.initials: R
authors.lastname: Koerfer
mesh_terms: D000368:Aged; D000686:Amyloidosis / Q000628:therapy*; D001706:Biopsy; D004452:Echocardiography; D005091:Exons; D006085:Graft Survival; D006331:Heart Diseases / Q000628:therapy*; D016027:Heart Transplantation / Q000379:methods*; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D009206:Myocardium / Q000473:pathology; D011228:Prealbumin / Q000235:genetics* / Q000378:metabolism; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D011228:Prealbumin