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Restrictive physiology is associated with poor outcomes in children with hypertrophic cardiomyopathy

Shiraz A Maskatia1, Jamie A Decker, Joseph A Spinner

  • 1Lillie Frank Abercrombie Section of Pediatric Cardiology, Texas Children's Hospital, Houston, TX, USA. samaskat@texaschildrenshospital.org

Pediatric Cardiology
|September 6, 2011
PubMed

Insights

Pediatric hypertrophic cardiomyopathy (HCM) patients with restrictive physiology (RP) face significantly worse outcomes. Identifying RP is crucial for risk stratification and improving the prognosis of children with HCM.

Area of Science:

  • Pediatric Cardiology
  • Cardiovascular Research
  • Clinical Outcomes

Background:

  • Hypertrophic cardiomyopathy (HCM) is a significant cause of pediatric heart disease.
  • Restrictive physiology (RP) in pediatric HCM is associated with poor outcomes, but data are limited.
  • Understanding the clinical course of pediatric HCM with RP is essential for management.

Purpose of the Study:

  • To delineate the clinical features and course of pediatric patients diagnosed with HCM.
  • To specifically investigate the impact of restrictive physiology (RP) on the outcomes of these patients.
  • To assess the prognostic significance of RP in children with HCM.

Main Methods:

  • Retrospective institutional review of 119 pediatric patients with HCM diagnosed between 1985 and 2010.
  • Diagnosis of RP based on echocardiographic criteria including left atrial enlargement, E/E' ratio ≥ 10, and E/A ratio ≥ 3.
  • Outcomes analysis using Cox or Poisson regression, focusing on death, aborted sudden cardiac death (aSCD), and heart transplant (HT).

Main Results:

  • Restrictive physiology (RP) was identified in 50 (42%) of the pediatric HCM patients.
  • Patients with RP had significantly lower 10-year freedom from death or aSCD (59.0%) and death or HT (71.2%) compared to those without RP (93.6% and 98.5%, respectively).
  • RP was associated with a 3.5-fold increased hospitalization rate, a 3.8-fold increased hazard of death or aSCD, and a 5.7-fold increased hazard of death or HT.

Conclusions:

  • Restrictive physiology (RP) is a critical determinant of poor outcomes in pediatric patients with hypertrophic cardiomyopathy (HCM).
  • Children with HCM and RP experience substantially worse prognoses, including increased risk of hospitalization, death, and heart transplantation.
  • Routine assessment for RP is paramount in pediatric HCM patients to identify those at high risk and guide clinical management effectively.

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