Buchanan W P, Schreiter S W, Kahl F R, Lantz P E, Cheek H B, Risley J, Nomeir A M
Department of Medicine, Bowman Gray School of Medicine, Wake Forest University, Winston-Salem, NC 27157.
J Am Soc Echocardiogr. 1993 Jan-Feb;6(1):87-90. doi: 10.1016/s0894-7317(14)80261-7.
A combination of hypertrophic obstructive cardiomyopathy (HOCM) and cardiac amyloidosis in the same patient is very rare. Clinical diagnosis could be extremely difficult and may require myocardial biopsy. We are reporting a patient with this combination who was referred to our institution because of features of HOCM based on clinical, echocardiographic and Doppler criteria. Cardiac amyloidosis was only recognized after myocardial biopsy that failed to reveal evidence of HOCM. Only after the patient expired from severe, intractable heart failure did the autopsy findings confirm the association of HOCM. We believe that the combination of the two cardiomyopathic processes is very rare and makes treatment extremely difficult.
肥厚型梗阻性心肌病(HOCM)与心脏淀粉样变性在同一患者中同时存在的情况极为罕见。临床诊断可能极具难度,可能需要进行心肌活检。我们报告了一位患有这两种病症的患者,该患者因基于临床、超声心动图和多普勒标准的HOCM特征而被转诊至我院。心脏淀粉样变性仅在心肌活检后才被识别,而活检未能发现HOCM的证据。直到患者因严重的顽固性心力衰竭死亡后,尸检结果才证实了HOCM的存在。我们认为这两种心肌病过程同时存在的情况非常罕见,使得治疗极其困难。