Document Detail

Spontaneous pericardial hematoma with familial amyloid polyneuropathy.
MedLine Citation:
PMID:  19922334     Owner:  NLM     Status:  MEDLINE    
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.
Naohiko Nakanishi; Takahisa Sawada; Rena Sato; Kenji Yanishi; Yoshiki Akakabe; Shinya Nishizawa; Akira Kuroyanagi; Yoshinori Tsubakimoto; Akihiro Matsui; Takeshi Nakamura; Hirokazu Shiraishi; Akiyoshi Matsumuro; Takeshi Shirayama; Hiroaki Matsubara
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis     Volume:  16     ISSN:  1744-2818     ISO Abbreviation:  Amyloid     Publication Date:  2009 Dec 
Date Detail:
Created Date:  2009-11-20     Completed Date:  2010-12-08     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9433802     Medline TA:  Amyloid     Country:  England    
Other Details:
Languages:  eng     Pagination:  221-5     Citation Subset:  IM    
Department of Cardiovascular Medicine, Kyoto Prefectural University of Medicine, Kyoto, Japan.
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MeSH Terms
Amyloid Neuropathies, Familial / complications*,  physiopathology*
Fatal Outcome
Hematoma / diagnosis,  etiology*
Pericardium / pathology*

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