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

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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
PubMed

Insights

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.

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.

Keywords:
Hypertrophic cardiomyopathy (HCM)septal myectomy

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