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 pericar...

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Bibliographic Details
Published in:Amyloid 2009-12, Vol.16 (4), p.221-225
Main Authors: Nakanishi, Naohiko, Sawada, Takahisa, Sato, Rena, Yanishi, Kenji, Akakabe, Yoshiki, Nishizawa, Shinya, Kuroyanagi, Akira, Tsubakimoto, Yoshinori, Matsui, Akihiro, Nakamura, Takeshi, Shiraishi, Hirokazu, Matsumuro, Akiyoshi, Shirayama, Takeshi, Matsubara, Hiroaki
Format: Article
Language:English
Subjects:
Aged
amyloid angiopathy
Amyloid Neuropathies, Familial - complications
Amyloid Neuropathies, Familial - physiopathology
familial amyloid polyneuropathy
Fatal Outcome
Female
Hematoma - diagnosis
Hematoma - etiology
Humans
Pericardial hematoma
Pericardium - pathology
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Summary: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.
ISSN:1350-6129
1744-2818
DOI:10.3109/13506120903421751