Author + information
- Received January 25, 1988
- Revision received November 3, 1988
- Accepted November 11, 1988
- Published online May 1, 1989.
- ↵†Address for reprints: Barry Maron, MD, Building 10. Room 7B-15. National Institutes of Health, Bethesda, Maryland 20892.
This study describes the long-term outcome of 33 patients with hypertrophic cardiomyopathy who experienced a cardiac arrest but were successfully resuscitated. Cardiac arrest occurred at ages 9 to 62 years (mean 32); five patients survived multiple (two or three) arrests. A variety of treatments were administered; 18 patients with left ventricular outflow tract obstruction underwent ventricular septal myotomy-myectomy or mitral valve replacement and also received drug therapy; 15 patients received medical therapy alone.
To date, 22 (67%) of the 33 patients have survived after the initial cardiac arrest for periods of 17 months to 22 years (mean 7 years); 12 patients have survived for ≥5 and 6 for ≤ 10 years. Of the 22 survivors, 16 have remained asymptomatic or only mildly symptomatic over the period of follow-up; 6 others have become severely symptomatic with heart failure, including 3 with evidence of left ventricular wall thinning and cavity enlargement. Eight patients ultimately died of natural cardiac causes (suddenly or of progressive heart failure) 7 months to 8.4 years (mean 4 years) after their initial cardiac arrest. Actuarial patient survival was 97 ± 3%, 74 ± 9% and 61 ± 11% after 1, 5 and 10 years, respectively. Event-free rate (defined as actuarial survival without recurrent cardiac arrest or death) was 83 ± 7%, 65 ± 9% and 53 ± 11%, respectively.
For this group of patients with hypertrophic cardiomyopathy who were treated in a nonsystematic fashion with a variety of therapeutic strategies, the long-term outcome after surviving a cardiac arrest was variable. Recurrent cardiac arrest or premature cardiac death (sudden or due to congestive failure) occurred in one-third of the patients, most commonly within the first 5 years after the initial arrest. Conversely, most patients have survived and remained free of subsequent cardiac arrest usually without experiencing marked symptoms; hence, aborted episodes of sudden death do not necessarily convey a uniformly ominous prognosis in hypertrophic cardiomyopathy and in some patients appear to be isolated and unexpected events in the natural history of their disease.
- Received January 25, 1988.
- Revision received November 3, 1988.
- Accepted November 11, 1988.