Fatal cyclophosphamide cardiomyopathy: its clinical course and treatment.
Fatal cyclophosphamide cardiomyopathy: its clinical course and treatment.
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致命的环磷酰胺心肌病：其临床过程和治疗。
DOI：
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发表时间：
1996
影响因子：
4.8
通讯作者：
R. Gingrich
中科院分区：
文献类型：
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作者：
C. K. Lee;G. Harman;R. Hohl;R. Gingrich
环磷酰胺诱发的急性失代偿性心肌病通常是不可逆的。为了研究临床过程和治疗结果，分析了13例（1.7%）在1980年1月至1995年9月期间接受消融性移植方案治疗的III级急性心肌病和低血压患者。9例患者中有8例死于急性致死性限制性心肌病伴无反应性低血压（COPD），而4例首次发作后存活的患者中有3例死于亚急性充血性心力衰竭（SCHF）。急性致死性限制性心肌病的特点是对容量超负荷极度敏感，心肌水肿和快速致死性病程。其与进行性、无反应性低血压、左心室每搏作功指数降低（LVSWI：29.29 +/- 9.74 g-m/beat/m2）以及全身和肺血管阻力指数显著降低（SVRI：429.72 +/- 168.84，PVRI：58.63 +/- 45.08 dyne.sec/cm5.m2）相关。亚急性CHF通过心肌水肿、扩张的心腔和双心室泵衰竭（代表缩短分数降低）（FS：19.5 +/- 4.9%）确定。在接受常规治疗的10例患者中，9例死亡，1例持续慢性CHF。三名接受抗坏血酸和茶碱抗氧化治疗的患者中有一名幸存下来。数据表明外周血管塌陷也可能是致命性ARCH的原因。
Acute decompensating cardiomyopathy induced by cyclophosphamide is usually irreversible. To investigate the clinical course and the outcome of therapy, 13 patients (1.7%) with grade III acute cardiomyopathy and hypotension who were treated with ablative transplant regimens between January 1980 and September 1995 were analyzed. Eight of nine patients died of acute fatal restrictive cardiomyopathy with unresponsive hypotension (ARCH), whereas three of four patients who survived the initial episode died of subacute congestive heart failure (SCHF). Acute fatal restrictive cardiomyopathy was characterized with extreme sensitivity to volume overload, myocardial edema and a rapidly fatal course. It was associated with progressive, unresponsive hypotension, reduced left ventricular stroke work index (LVSWI: 29.29 +/- 9.74 g-m/beat/m2) and markedly reduced systemic and pulmonary vascular resistance indices (SVRI: 429.72 +/- 168.84, PVRI: 58.63 +/- 45.08 dyne.sec/cm5.m2). Subacute CHF was identified by myocardial edema, dilated chambers and biventricular pump failure represented by decreases in fractional shortening (FS: 19.5 +/- 4.9%). Of 10 patients who received conventional therapy, nine died and one sustained chronic CHF. One of three patients with ARCH on antioxidant therapy of ascorbic acid and theophylline survived the episode. The data suggests peripheral vascular collapse may also be responsible for fatal ARCH.