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Cardiomyopathy, Pediatric

Kimberly M. Molina, MD and Lindsay J. May, MD, FRCPC Reviewed 10/2018
 


BASICS

DESCRIPTION

Cardiomyopathy (CM) is a disease of the heart muscle, which impairs function (systolic, diastolic, or both). It is classified based on structural and functional abnormalities: 
  • Dilate...

DIAGNOSIS

In the early stages of all three forms of CM symptoms are nonspecific and can mimic other disease processes. The cardiac examination can be completely normal. 

HISTORY

DCM: 
  • Irritability, failu...

TREATMENT

GENERAL MEASURES

The initial assessment evaluates their degree of compensation in heart failure and whether they are congested/dry, warm/cold. This will guide initial treatment and should be ...

ONGOING CARE

PATIENT EDUCATION

The Children’s Cardiomyopathy Foundation includes helpful resources for families of those affected by CM. 

PROGNOSIS

  • DCM: The rate of death or transplant is ~30% at 1-year ...

ADDITIONAL READING

  • Alvarez JA, Orav EJ, Wilkinson JD, et al. Competing risks for death and cardiac transplantation in children with dilated cardiomyopathy: results from the pediatric cardiomyopathy re...

CODES

ICD9

  • 425.4 Other primary cardiomyopathies

  • 425.11 Hypertrophic obstructive cardiomyopathy

  • 425.9 Secondary cardiomyopathy, unspecified

  • 425.18 Other hypertrophic cardiomyopathy

  • 425.8 Cardiomyopathy in o...

FAQ

  • Q: Should family members be evaluated once CM is diagnosed in a 1st-degree relative?

  • A: Yes. In some forms of CM, there is a strong genetic component, and family members should be evaluated. If the...

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