Childhood Hypertrophic Obstructive Cardiomyopathy and Its Relevant Surgical Outcome.

BACKGROUND

Contemporary experiences regarding childhood hypertrophic obstructive cardiomyopathy are limited. This study aimed to describe the clinical presentation of childhood hypertrophic obstructive cardiomyopathy and its relevant surgical outcome.

METHODS

In all, 117 consecutive children with hypertrophic obstructive cardiomyopathy aged 0.6 to 17.5 years who underwent septal myectomy at our institution between February 2009 and December 2018 were included. Medical records and other patient-related data were reviewed.

RESULTS

In the present study, the anatomic and physiologic characteristics of childhood hypertrophic obstructive cardiomyopathy were highly heterogeneous, with simultaneous right ventricular outflow tract obstruction in 22 patients (18.8%), coronary myocardial bridging in 25 patients (21.4%), and intraventricular anatomic abnormalities in 61 patients (52.1%). The mean peak left or right ventricular outflow tract gradient, interventricular septal thickness, and degree of mitral regurgitation significantly decreased after surgery. One early death was noted in the study. During follow-up, three sudden cardiac deaths were noted. The overall survival rates at follow-up were 100% at 1 year and 96.5% at 3 years. The overall survival rates free from reoperation were 99.1% at 1 year and 98.0% at 3 years.

CONCLUSIONS

In our cohort of children with hypertrophic obstructive cardiomyopathy undergoing septal myectomy, biventricular obstruction, myocardial bridging, and intraventricular anatomic abnormalities are frequent phenotypic components. Despite the complexity of childhood hypertrophic obstructive cardiomyopathy, surgical treatment results in a favorable outcome in carefully selected patients.