Tiberti G, Bana G, Selva A, Bossi I, Mancini R, Bossi M
Divisione di Cardiologia, Ospedale di Lecco.
Minerva Cardioangiol. 1997 Mar;45(3):115-9.
A 48-year old patient affected by congestive heart failure came to our observation for cardiac arrest due to ventricular fibrillation. After cardiopulmonary resuscitation and defibrillation he underwent complete evaluation. Echo Doppler findings were consistent with restrictive cardiomyopathy. Laboratory findings revealed monoclonal gammopathy and plasma cells dyscrasia. Diagnosis of amyloidosis was then suspected and biopsies of different organs and tissues were performed. Presence of amyloid deposits was found only in myocardial specimens from the right ventricle. Medical treatment with drugs of various classes, administered during hemodynamic invasive monitoring, was uneffective in improving the hemodynamic and clinical status of the patient and he entered in a heart transplantation list. He died six months later, while awaiting for transplantation. Although isolated cardiac amyloidosis is quite rare, we believe that this condition has ever to be kept in mind during differential diagnosis of restrictive cardiomyopathies and we remark that endomyocardial biopsy was mandatory and necessary for certain diagnosis in this case; in addition, the unefficacy of drugs nowadays available for treatment of congestive heart failure in amyloid cardiomyopathy is confirmed.
一名48岁的充血性心力衰竭患者因室颤导致心脏骤停前来我院就诊。经过心肺复苏和除颤后,他接受了全面评估。超声心动图检查结果与限制性心肌病相符。实验室检查结果显示单克隆丙种球蛋白病和浆细胞发育异常。随后怀疑为淀粉样变性,并对不同器官和组织进行了活检。仅在右心室心肌标本中发现了淀粉样沉积物。在血流动力学侵入性监测期间给予各种药物进行药物治疗,未能有效改善患者的血流动力学和临床状况,于是他被列入心脏移植名单。六个月后,他在等待移植期间死亡。尽管孤立性心脏淀粉样变性相当罕见,但我们认为在限制性心肌病的鉴别诊断过程中必须始终考虑到这种情况,并且我们强调,对于该病例的明确诊断,心内膜心肌活检是必不可少且必要的;此外,目前用于治疗淀粉样心肌病所致充血性心力衰竭的药物无效这一点也得到了证实。