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Related Experiment Video

Updated: Feb 22, 2026

Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model

Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model

Published on: August 8, 2022

Hypertrophic cardiomyopathy in children.

Arman Arghami¹, Joseph A Dearani¹, Sameh M Said¹

¹Department of Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota, USA.

Annals of Cardiothoracic Surgery

|September 26, 2017

Summary

Septal myectomy is a safe and effective treatment for children with obstructive hypertrophic cardiomyopathy (HCM), improving survival rates. Careful patient selection is crucial, especially for prophylactic procedures in asymptomatic children.

Keywords:

Hypertrophic cardiomyopathy (HCM)septal myectomy

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Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model

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Published on: August 8, 2022

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Scanning Electron Microscopy of Macerated Tissue to Visualize the Extracellular Matrix

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Area of Science:

Cardiology
Pediatric Cardiology
Cardiovascular Surgery

Background:

Hypertrophic cardiomyopathy (HCM) affects 1 in 500 adults and is a leading cause of death in individuals under 35.
Pediatric HCM often presents asymptomatically, with a 1% annual mortality rate after the first year of life.

Purpose of the Study:

To evaluate the safety and efficacy of septal myectomy in children with obstructive HCM.
To assess the impact of septal myectomy on long-term survival in pediatric patients with HCM.
To highlight the importance of patient selection for septal myectomy, including prophylactic procedures.

Main Methods:

Review of published data on septal myectomy outcomes in pediatric obstructive HCM.
Comparison of survival rates between treated and untreated HCM patients.
Analysis of factors influencing successful surgical outcomes.

Main Results:

Septal myectomy is demonstrated to be safe and effective in pediatric obstructive HCM.
Published data indicates improved late survival in children undergoing septal myectomy compared to those untreated.
Successful outcomes are strongly linked to meticulous patient selection and surgical expertise.

Conclusions:

Septal myectomy offers a safe and effective therapeutic option for children with obstructive HCM.
Prophylactic septal myectomy in asymptomatic pediatric patients requires careful consideration regarding patient selection and surgical skill.
Improved long-term survival is a key benefit of timely and appropriate surgical intervention for HCM in children.