FATAL CYCLOPHOSPHAMIDE CARDIOMYOPATHY - ITS CLINICAL COURSE AND TREATMENT

Acute decompensating cardiomyopathy induced by cyclophosphamide is usu ally 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 betwee n January 1980 and September 1995 were analyzed, Eight of nine patient s died of acute fatal restrictive cardiomyopathy with unresponsive hyp otension (ARCH), whereas three of four patients who survived the initi al episode died of subacute congestive heart failure (SCHF), Acute fat al restrictive cardiomyopathy was characterized with extreme sensitivi ty to volume overload, myocardial edema and a rapidly fatal course, It was associated with progressive, unresponsive hypotension, reduced le ft ventricular stroke work index (LVSWI: 29.29 +/- 9.74 g-m/beat/m(2)) and markedly reduced systemic and pulmonary vascular resistance indic es (SVRI: 429.72 +/- 168.84, PVRI: 58.63 +/- 45.08 dyne.sec/cm(5).m(2) ). Subacute CHF was identified by myocardial edema, dilated chambers a nd biventricular pump failure represented by decreases in fractional s hortening (FS: 19.5 +/- 4.9%), Of 10 patients who received conventiona l therapy, nine died and one sustained chronic CHF, One of three patie nts 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.