[Clinical characteristics and hypertrophic segments distribution of patients with hypertrophic cardiomyopathy]
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Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model
Published on: August 8, 2022
Chao-wu YAN1, Shi-hua ZHAO, Hua LI
1Department of Radiology, Cardiovascular Institute and Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100037, China.
Insights
Hypertrophic cardiomyopathy (HCM) in Chinese men often presents later in life. Comprehensive diagnostic approaches combining clinical, ECG, echocardiography, and cardiac MRI are crucial for accurate HCM diagnosis.
Area of Science:
- Cardiology
- Medical Imaging
- Genetics
Context:
- Hypertrophic cardiomyopathy (HCM) is a significant cardiovascular condition.
- Understanding the clinical characteristics and distribution of hypertrophy is key for diagnosis and management.
- Cardiac Magnetic Resonance imaging (CMR) provides detailed anatomical information.
Purpose:
- To analyze the clinical data and hypertrophic segments distribution in patients with hypertrophic cardiomyopathy (HCM).
- To correlate clinical findings with imaging data in a cohort of Chinese HCM patients.
Summary:
- A study of 225 Chinese patients with CMR-defined HCM revealed a high prevalence in men, with a mean age of 50.4 years.
- Asymmetrical hypertrophy was common (222 patients), with significant septal thickness in obstructive HCM.
- Electrocardiogram abnormalities were frequent (216 patients), and 95 had left ventricular outflow obstruction.
Impact:
- This study highlights the male predominance and late-onset presentation of HCM in the Chinese population.
- Integrating clinical, ECG, echocardiography, and CMR data is vital for accurate HCM diagnosis in clinical practice.
- Findings contribute to a better understanding of HCM's phenotypic diversity and diagnostic strategies.
Objective:
to analyze the clinical data and hypertrophic segments distribution of patients with hypertrophic cardiomyopathy (HCM).
Methods:
clinical data including signs and symptoms, electrocardiogram and echocardiography were collected. All patients were imaged with cardiac magnetic resonance imaging (CMR).
Results:
from March 2004 to March 2007, 225 consecutive patients [163 males, mean age (50.4 ± 14.5) years] with CMR defined HCM were included in this study, positive familial history was obtained in 73 patients, 50 patients were associated with hypertension, 14 patients with coronary artery disease and 5 patients with diabetes mellitus, 28 patients were asymptomatic, 197 patients were symptomatic, and 11 patients with syncope. Electrocardiogram abnormalities occurred in 216 patients. Systolic murmurs were present in 126 patients. Echocardiography examination evidenced left ventricular outflow obstruction in 95 patients, mitral insufficiency in 32 patients, 32.1% segments were hypertrophied, asymmetrical hypertrophy presented in 222 patients and symmetrical hypertrophy in 3 patients. The left atrial dimension was (39.4 ± 8.3) mm, and left ventricular diastolic dimension was (47.8 ± 5.5) mm in this cohort. Apical hypertrophy occurred in 67 patients. The thickness of ventricular septum was (24.3 ± 5.3) mm in obstructive HCM and (21.6 ± 4.6) mm in non-obstructive HCM (P < 0.05). The thickness of hypertrophy apical segment was (15.6 ± 3.4) mm.
Conclusions:
HCM in Chinese patients is characterized by the high prevalence among men and late onset of presentation. Combining clinical, electrocardiogram, echocardiographic and CMR results are of importance for correctly diagnosing HCM in daily practice.
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