title: Spontaneous pericardial hematoma with familial amyloid polyneuropathy.
abstract: There are more than a few risks of hemorrhage complication in patients with amyloidosis. Although most cases with amyloidosis exhibit minor bleeding manifestations, they can be occasionally associated with life-threatening problems. To our knowledge, there are only a few cases of spontaneous pericardial hematoma associated with amyloidosis. We here report a patient who suddenly died of cardiac tamponade with massive pericardial hematoma 7 years after the diagnosis of familial amyloid polyneuropathy (FAP). A 69-year-old female with FAP with cardiogenic shock was admitted to our emergency room. Although she previously underwent permanent pacemaker implantation for atrial fibrillation with slow ventricular response, electrocardiogram showed a critical pacing failure. Emergent telemetry check revealed a sudden extreme increase of pacing capture threshold in the right ventricle. Maximum pacing voltage could not improve the critical condition, and she died 7 h after arrival. Autopsy showed a massive pericardial hematoma in the right ventricular free wall, and microscopic examination revealed typical amyloid deposition in the arterial wall of the pericardium. In this case, it is assumed that a sudden rupture of fragile pericardial vessels with amyloid deposition led to the lethal pericardial hematoma.
authors.affiliation: Department of Cardiovascular Medicine, Kyoto Prefectural University of Medicine, Kyoto, Japan. naka-nao@koto.kpu-m.ac.jp
authors.forename: Naohiko
authors.initials: N
authors.lastname: Nakanishi
authors.forename: Takahisa
authors.initials: T
authors.lastname: Sawada
authors.forename: Rena
authors.initials: R
authors.lastname: Sato
authors.forename: Kenji
authors.initials: K
authors.lastname: Yanishi
authors.forename: Yoshiki
authors.initials: Y
authors.lastname: Akakabe
authors.forename: Shinya
authors.initials: S
authors.lastname: Nishizawa
authors.forename: Akira
authors.initials: A
authors.lastname: Kuroyanagi
authors.forename: Yoshinori
authors.initials: Y
authors.lastname: Tsubakimoto
authors.forename: Akihiro
authors.initials: A
authors.lastname: Matsui
authors.forename: Takeshi
authors.initials: T
authors.lastname: Nakamura
authors.forename: Hirokazu
authors.initials: H
authors.lastname: Shiraishi
authors.forename: Akiyoshi
authors.initials: A
authors.lastname: Matsumuro
authors.forename: Takeshi
authors.initials: T
authors.lastname: Shirayama
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Matsubara
mesh_terms: D000368:Aged; D028227:Amyloid Neuropathies, Familial / Q000150:complications* / Q000503:physiopathology*; D017809:Fatal Outcome; D005260:Female; D006406:Hematoma / Q000175:diagnosis / Q000209:etiology*; D006801:Humans; D010496:Pericardium / Q000473:pathology*