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

Shiraz A. Maskatia, Jamie A. Decker, Joseph A. Spinner, Jeffrey J. Kim, Jack F. Price, John L. Jefferies, William J. Dreyer, E. O.Brian Smith, Joseph W. Rossano, Susan W. Denfield

Research output: Contribution to journalReview articlepeer-review

Abstract

Pediatric patients with hypertrophic cardiomyopathy (HCM) and restrictive physiology (RP) with poor outcomes have been identified, but data on their course are limited. Our goal was to delineate the clinical features and course of children with HCM and RP. An institutional review of 119 patients identified between 1985 and 2010 with the diagnosis of HCM was performed. The diagnosis of RP was based on >1 echocardiogram along with at least one of the following: left atrial enlargement without evidence of left ventricle dilation, E/E0 ratio ≥ 10, and E/A ratio ≥ 3. Outcomes analysis was performed using Cox or Poisson regression when appropriate. RP was present in 50 (42%) patients. In patients without RP, 10-year freedomfrom- death or aborted sudden cardiac death (aSCD), and death or heart transplant (HT), were 93.6 and 98.5%, respectively. In patients with RP, 10-year freedom-fromdeath or aSCD, and death or HT, were 59.0 and 71.2%, respectively. RP conferred a 3.5-fold increase in incidence rate of hospitalization (P = 0.01), a 3.8-fold increase in hazard of death or aSCD (P = 0.02), and a 5.7-fold increase in hazard of death or HT (P = 0.04). Assessment for RP is of paramount importance in children with HCM because those without RP have a good prognosis, and those with RP account for the majority of poor outcomes.

Original languageEnglish (US)
Pages (from-to)141-149
Number of pages9
JournalPediatric Cardiology
Volume33
Issue number1
DOIs
StatePublished - Jan 2012
Externally publishedYes

Keywords

  • Diastology
  • Hypertrophic cardiomyopathy
  • Restrictive physiology

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Cardiology and Cardiovascular Medicine

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