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Delayed Myocardial Enhancement in Pediatric Hypertrophic Cardiomyopathy: Correlation with LV Function,

Sonia El Saiedi1, Noha Hossam Behairy2, Ahmed Kharabish1

  • 1Cairo University, Kasr El Aini Hospital, Giza, Egypt.

Pediatric Cardiology
|April 13, 2017
PubMed

Insights

Cardiac magnetic resonance imaging (CMR) detects fibrosis in pediatric hypertrophic cardiomyopathy (HCM). Fibrosis correlates with disease severity, aiding in patient management and prognosis.

Area of Science:

  • Cardiology
  • Pediatric Imaging
  • Cardiovascular Disease

Background:

  • Hypertrophic cardiomyopathy (HCM) is a significant cause of sudden cardiac death in children.
  • Assessing myocardial fibrosis is crucial for risk stratification and management in pediatric HCM.
  • Current methods for fibrosis detection in pediatric HCM require further validation.

Purpose of the Study:

  • To detect myocardial fibrosis using cardiac magnetic resonance imaging (CMR) in pediatric HCM patients.
  • To correlate CMR findings, specifically delayed myocardial enhancement (DE-MRI), with clinical, demographic, and echocardiographic parameters.
  • To evaluate the utility of DE-MRI in assessing disease severity and guiding management in pediatric HCM.

Main Methods:

  • A cohort of 40 pediatric patients diagnosed with HCM underwent clinical examination, echocardiography, and CMR.
  • CMR was performed using a 1.5T scanner with cine and DE-MRI sequences.
  • DE-MRI quantified the percentage of myocardial enhancement, which was correlated with demographic data, LV function, NYHA classification, LVOT pressure gradient, and LV myocardial mass index.

Main Results:

  • The mean percentage of DE-MRI in pediatric HCM patients was 9.7 ± 9%.
  • Significant correlations were found between DE-MRI percentage and NYHA classification (P=0.004), LVOT pressure gradient (P<0.001), and LV myocardial mass index (P=0.042).
  • DE-MRI showed a strong positive correlation with the severity of LVOT pressure gradient (r=0.69, P<0.001).

Conclusions:

  • DE-MRI is a valuable tool for detecting and quantifying myocardial fibrosis in pediatric HCM.
  • CMR findings, particularly DE-MRI, correlate significantly with clinical indicators of disease severity, including NYHA class and LVOT obstruction.
  • These findings support the use of CMR in the management, follow-up, and prognosis assessment of pediatric HCM patients.

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