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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.
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.
Abstract:
Our aim was (1) to detect the presence of fibrosis by Cardiac magnetic resonance imaging (CMR) in the pediatric age group. (2) Correlate CMR findings with demographic data, LV function, and other echocardiographic parameters. We studied 40 pediatric patients diagnosed as HCM by echocardiography. All patients were subjected to clinical examination (in which the NYHA classification was determined for each patient), echocardiography, and CMR. CMR was done on a 1.5T Philips Achieva scanner in SSFP with delayed myocardial enhancement (DE-MRI). All demographic and functional parameters as well as pressure gradient across left ventricular outflow tract (LVOT) were correlated with the percentage of myocardial enhancement. We studied 13 female and 27 male patients from 45 days up to 18 years. The mean percentage of DE-MRI was 9.7 ± 9%. We found significant correlation between the NYHA classification and the pressure gradient across the LVOT (P = < 0.001) as well as the percentage of DE-MRI (P = 0.004). The percentage of DE-MRI showed positive correlation with LV myocardial mass index (P = 0.042). It didn't correlate with any other demographic or LV functional cardiac parameters. A good positive correlation was detected between the percentage of DE-MRI and the severity of pressure gradient across LVOT measured by echocardiography (r = 0.69 and P = <0.001). We found a significant correlation between the percentage of DE-MRI in children with HCM and the pressure gradient across LVOT, NYHA classification, and LV myocardial mass. This may help in the further management of those patients, planning for follow-up, and prognosis of the disease.