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Childhood Hypertrophic Obstructive Cardiomyopathy and Its Relevant Surgical Outcome
Changsheng Zhu1, Shuiyun Wang1, Yi Ma1
1Department of Cardiovascular Surgery and Beijing Key Laboratory for Molecular Diagnostics of Cardiovascular Diseases, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Insights
Childhood hypertrophic obstructive cardiomyopathy (HOCM) presents with diverse anatomical issues. Septal myectomy surgery offers favorable outcomes for carefully selected pediatric HOCM patients, with high survival and reoperation-free rates.
Area of Science:
- Cardiology
- Pediatric Surgery
- Cardiovascular Research
Background:
- Limited contemporary data exists on childhood hypertrophic obstructive cardiomyopathy (HOCM).
- Understanding HOCM's clinical presentation and surgical outcomes in children is crucial.
Purpose of the Study:
- To describe the clinical presentation of childhood HOCM.
- To evaluate the surgical outcomes of septal myectomy in pediatric patients with HOCM.
Main Methods:
- Retrospective review of 117 pediatric patients (0.6-17.5 years) undergoing septal myectomy for HOCM.
- Data collected included patient demographics, clinical characteristics, and surgical outcomes between February 2009 and December 2018.
Main Results:
- Significant reductions in outflow tract gradients, septal thickness, and mitral regurgitation post-surgery.
- High survival rates (100% at 1 year, 96.5% at 3 years) and reoperation-free rates (99.1% at 1 year, 98.0% at 3 years).
- Frequent co-occurrence of biventricular obstruction, myocardial bridging, and intraventricular abnormalities.
Conclusions:
- Childhood HOCM exhibits complex phenotypes including biventricular obstruction and intraventricular abnormalities.
- Septal myectomy is a safe and effective treatment for carefully selected pediatric HOCM patients, yielding favorable long-term results.
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.
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