Characteristics and Longer-Term Outcomes of Contemporary Patients <18 Years of Age With Hypertrophic Cardiomyopathy.

Abstract We describe characteristics and outcomes of contemporary pediatric hypertrophic cardiomyopathy (PHC) patients. We studied 398 consecutive pediatric HC patients ( 6) and 8% had LVOTG >30 mm Hg (range 0-139 mm Hg). At a median of 5.9 years [interquartile range 2.4, 9], there were 23 (6%) ICD's placed and 47 (12%) primary composite events [9 (2%) deaths, 3 (1%) appropriate ICD discharge, 29 (7%) myectomy and 8 (2%) OHT]. There were no in hospital deaths following myectomy/OHT. Presence of symptoms (Hazard ratio or HR 2.45), ventricular tachycardia (HR 1.52) and higher basal septal LV z-score (HR 1.10) were independently associated with primary composite outcomes. LV septal z-score >4 was independently associated with events on spline analysis. Rate of SCD/appropriate ICD discharge was 0.5%/year. In conclusion, contemporary PHC patients seen at an experienced center have excellent outcomes with presence of symptoms and higher LV septal thickness associated with primary composite events.