title: Severe congestive heart failure with cardiac liver cirrhosis 10 years after orthotopic liver transplantation for familial amyloidotic polyneuropathy.
abstract: Reported herein is an autopsy case of familial amyloidotic polyneuropathy (FAP) with cardiac liver cirrhosis associated with amyloid cardiomyopathy after liver transplantation. At 47 years of age a Japanese woman with a transthyretin Val30Met mutation and sensorimotor polyneuropathy underwent liver transplantation; no postoperative deterioration related to the graft or polyneuropathy occurred. However, cardiovascular dysfunction associated with amyloid deposition gradually worsened. Pacemaker implantation and diuretics were ineffective against the heart failure; 10 years after transplantation the patient died. Autopsy revealed massive pleural and pericardial effusions and amyloid cardiomyopathy, especially in the right atrium and cardiac conduction system. Amyloid deposition was slight in all organs except the heart, but liver cirrhosis with reversed lobulation and centrilobular hemorrhagic necrosis was prominent. There was no histological evidence for chronic liver graft rejection. These findings suggest that liver transplantation effectively stopped amyloid deposition and ameliorated clinical FAP symptoms but that amyloid cardiomyopathy after liver transplantation in advanced clinical stages may lead to severe congestive heart failure and cardiac liver cirrhosis.
authors.affiliation: Department of Cell Pathology, Graduate School of Medical and Pharmaceutical Sciences, Kumamoto University, Kumamoto, Japan. nanaomi@gpo.kumamoto-u.ac.jp
authors.forename: Naomi
authors.initials: N
authors.lastname: Sakashita
authors.forename: Yukio
authors.initials: Y
authors.lastname: Ando
authors.forename: Katsuki
authors.initials: K
authors.lastname: Haraoka
authors.forename: Hisayasu
authors.initials: H
authors.lastname: Terazaki
authors.forename: Taro
authors.initials: T
authors.lastname: Yamashita
authors.forename: Masaaki
authors.initials: M
authors.lastname: Nakamura
authors.forename: Motohiro
authors.initials: M
authors.lastname: Takeya
mesh_terms: D000328:Adult; D019943:Amino Acid Substitution / Q000235:genetics; D000682:Amyloid / Q000378:metabolism; D028227:Amyloid Neuropathies, Familial / Q000235:genetics / Q000601:surgery*; D017809:Fatal Outcome; D005260:Female; D006333:Heart Failure / Q000209:etiology* / Q000473:pathology; D006801:Humans; D008103:Liver Cirrhosis / Q000209:etiology* / Q000473:pathology; D016031:Liver Transplantation / Q000009:adverse effects*; D011228:Prealbumin / Q000235:genetics
chemical_list: D000682:Amyloid; D011228:Prealbumin