Hypertrophic Cardiomyopathy in Children, Adolescents and Young Adults Associated With Low Cardiovascular Mortality With Contemporary Management Strategies
Background—Youthful age has been considered the time of greatest risk for patients with hypertrophic cardiomyopathy (HCM), largely the possibility of sudden death (SD). The last 2 decades have witnessed more reliable identification of at-risk patients and utilization of implantable cardioverter-defibrillators (ICD) for prevention of SD, and other contemporary treatment options. Whether such management advances have significantly altered the considerable mortality rate for young HCM patients remain unresolved.
Methods and Results—We studied long-term outcome in 474 consecutive HCM patients presenting at 2 referral institutions between 7 and 29 years of age. Over 7.1 ± 5.1 years follow-up (6.0 [3.0, 10.0] ), 452 patients (95%) survived, with 95% experiencing no or mild symptoms. HCM-related death occurred in 18 patients (3%; 0.54%/year): arrhythmic SD (n=12), progressive heart failure and heart transplant complications (n=5), or postoperatively (n=1). In contrast, aborted life-threatening events occurred in 63 other high-risk patients (13%) with ICD interventions for ventricular tachyarrhythmias [n=31], resuscitated out-of-hospital cardiac arrest [n=20], or heart transplant for advanced heart failure [n=12], 1.8%/year, 3-fold higher than HCM mortality. Five- and 10-year survival (considering only HCM deaths) was high (97% and 94%, respectively), virtually identical to that reported in middle-aged adult HCM patients (98% and 94%, p=0.23).
Conclusions—In a large hospital-based cohort of young HCM patients, representing an age group considered at greatest risk, low mortality rates can be achieved with application of contemporary cardiovascular treatment strategies, due largely to reliable identification of high-risk patients who benefited from ICDs for SD prevention, thereby creating the opportunity for extended longevity and good quality of life.
- cardiac surgery
- hypertrophic cardiomyopathy
- sudden cardiac death
- implanted cardioverter defibrillator
- heart failure
- Received May 21, 2015.
- Revision received September 23, 2015.
- Accepted October 16, 2015.