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Background

Restrictive Physiology is a Major Predictor of Poor Outcomes in Children with Hypertrophic Cardiomyopathy.

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  1. Restrictive Physiology is a Major Predictor of Poor Outcomes in Children with Hypertrophic Cardiomyopathy Shiraz A Maskatia MD, Jamie A Decker MD, Joseph A Spinner BA, Jeffrey J Kim MD, Jack F Price MD, John L Jefferies MD, William J Dreyer MD, E O’Brian Smith PhD, Joseph W Rossano MD, Susan W Denfield MD

  2. Background • Hypertrophic cardiomyopathy (HCM) is associated with an increased risk of sudden and heart failure deaths in children 1,2 • Previously identified risk factors for death or heart transplant (HT) in children include abnormal BP response to exercise and left ventricular hypertrophy 2 • A subgroup of patients with restrictive physiology (RP) with worse outcomes has been described, but data in children is limited 3,4 1. Nugent et al Circulation 2005 2. Decker et al J Am Coll Cardiol 2009 3. Colan et al Circulation 2007 4. Webber et al Circulation (S2) 2008

  3. Hypothesis • The presence of restrictive physiology is a major predictor of hospitalization, death or transplant in children with HCM

  4. Methods • Retrospective review of patients followed for HCM at Texas Children’s Hospital • Inclusion criteria: • Age < 18 years at the time of diagnosis • Follow up time ≥ 1 year • Echocardiogram (echo) evidence of HCM without secondary cause • Outcomes analysis included Poisson and Cox regression

  5. Methods • RP on echo defined as > 1 echo with one of the following: • Left atrial enlargement without left ventricular dilation • Mitral inflow E/A ratio ≥ 3 • Mitral E/E’ ratio ≥ 10 • RP on catheterization (cath) defined as: • Systolic pulmonary artery pressure ≥ 35 mmHg • Left or right ventricular end diastolic pressure ≥ 15 mmHg • Pulmonary vascular resistance index ≥ 4 woods units

  6. Results • 444 patients with HCM treated at our institution from 1/1/1985 to 1/1/2010 • 119 patients met inclusion criteria • RP by echo was present in 49 (41%) patients • Left atrial enlargement was present in 43 (88%) patients • E/E’ ratio ≥ 10 present in 27 (55%) patients • E/A ratio ≥ 3 present in 9 (18%) patients

  7. Results • 17 (14%) patients underwent cath • RP by cath present in 11 (65%) patients • Of the 11 patients with RP by cath, 10 (91%) had RP by echo • No evidence of RP present in 69 (58%) patients • One patient had RP by cath, and not by echo

  8. Patient Characteristics

  9. Results • Patients with RP by echo • higher ventricular septal z-scores (p=0.03) • more likely to have ventricular arrhythmias (p=0.02 OR=5.6 CI=1.1-28.2) • more likely to have an abnormal blood pressure response to exercise (p=0.01 OR=3.9 CI=1.4-11.4) • Patients with RP by cath had higher left ventricular free wall z-scores (p=0.03)

  10. Results • 7 patients died; 6 (86%) had RP • 3 pts underwent HT; all 3 had RP • Aborted sudden cardiac death (aSCD) occurred in 9 pts; 6 (67%) had RP • RP by echo or cath had a positive predictive value of 17% and a negative predictive value of 98% for death or HT

  11. Limitations • Retrospective analysis • Determination of left atrial volume was subjective • Only 14% of patients in the study underwent cath – may result in selection bias.

  12. Conclusions • Children with hypertrophic cardiomyopathy without restrictive physiology have a good prognosis • Children with hypertrophic cardiomyopathy and restrictive physiology accounted for the majority of poor outcomes • Patients with evidence of restrictive physiology on echocardiogram and symptoms should undergo further hemodynamic assessment by cardiac catheterization

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