Spontaneous pericardial hematoma with familial amyloid polyneuropathy.
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.
Nakanishi
Naohiko
N
Department of Cardiovascular Medicine, Kyoto Prefectural University of Medicine, Kyoto, Japan. naka-nao@koto.kpu-m.ac.jp
Sawada
Takahisa
T
Sato
Rena
R
Yanishi
Kenji
K
Akakabe
Yoshiki
Y
Nishizawa
Shinya
S
Kuroyanagi
Akira
A
Tsubakimoto
Yoshinori
Y
Matsui
Akihiro
A
Nakamura
Takeshi
T
Shiraishi
Hirokazu
H
Matsumuro
Akiyoshi
A
Shirayama
Takeshi
T
Matsubara
Hiroaki
H
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*