Related Experiment Video
Updated: Jan 12, 2026
Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model
Published on: August 8, 2022
Hypertrophic Cardiomyopathy in Iran: General Characteristics, Clinical Presentations, Imaging Features, and Major
Saman Rostambeigi1, Negin Mahmoudi Hamidabad2, Sarah Nadri1
1Rajaie Cardiovascular, Medical, and Research Institute, Iran University of Medical Sciences, Tehran, Iran.
Insights
Hypertrophic cardiomyopathy (HCM) patients in Iran exhibit diverse clinical features and outcomes. Obstructive HCM and positive late gadolinium enhancement (LGE) are associated with increased Major Adverse Cardiovascular Events (MACE).
Area of Science:
- Cardiology
- Genetics
- Internal Medicine
Background:
- Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiomyopathy.
- Early diagnosis and management are crucial for improving quality of life and preventing sudden cardiac death.
Purpose of the Study:
- To determine the general characteristics of HCM patients in Iran.
- To identify gaps in diagnosis and management of HCM in the region.
Main Methods:
- Cross-sectional study of 322 HCM patients from 2016-2020.
- Data reviewed included clinical, imaging, and therapeutic information.
- Follow-up for Major Adverse Cardiovascular Events (MACE) up to 48 months.
Main Results:
- Mean age was 49.7 years; 40% were women.
- Dyspnea on exertion was the most common symptom (49%).
- MACE occurred in 34.16% of patients, higher in obstructive HCM and with positive LGE.
Conclusions:
- Obstructive HCM and LGE ≥ 10% are linked to increased MACE.
- Disopyramide and ICD implantation are viable treatments, followed by myectomy.
- Genetic testing and alcohol septal ablation are rarely used.
Introduction:
Hypertrophic cardiomyopathy (HCM), the most common inherited cardiomyopathy, has significant clinical presentation and outcome diversity. Early diagnosis and proper management are essential to improve the patient's quality of life and prevent sudden cardiac death. Collecting patient information in each country is the first step in finding the gaps in the proper diagnosis and management of these patients. We aimed to determine the general characteristics of HCM in the largest referral tertiary heart center in Iran.
Methods:
In this cross-sectional study, between 2016 and 2020, clinical, imaging, and therapeutic data of patients diagnosed with HCM at Rajaie Cardiovascular Medical Research Center were reviewed and followed up to 48 months for Major adverse cardiovascular events (MACE), defined as composite endpoint of death, stroke (nonfatal), hospitalization due to decompensated heart failure (HF) and malignant ventricular arrhythmia defined as sustained ventricular tachycardia or ventricular fibrillation.
Results:
A total of 322 patients, with a mean age of 49.7 ± 1 years, including 129 women (40%), were studied. Women were significantly older than men (53.1 ± 9.1 vs. 47.2 ± 8.9, P < 0.0001). The most prevalent symptom was dyspnea on exertion, affecting 158 patients (49%). Nonobstructive HCM was present in 180 patients (55.9%), and atrial fibrillation was observed in 82 patients (25.46%). Major Adverse Cardiovascular Events were more frequent in patients with obstructive forms of the disease. Beta-receptor-blocking agents were the most used medication (85%). In addition, 29 patients (9% overall and 20.4% of those with obstructive forms) were treated with disopyramide, and 19 patients (5.9%) underwent surgical myectomy. Late gadolinium enhancement (LGE) was detected in 56 out of 122 patients (45.9%) who underwent cardiac magnetic resonance imaging. MACE incidence was notably higher in patients with positive LGE. Overall, MACE occurred in 110 patients (34.16%). Malignant ventricular arrhythmias were reported in 64 patients (19.87%), HF in 36 patients (11.18%), and HCM-related death in 4 patients (1.24%).
Conclusion:
This study showed the general characteristics, clinical outcomes, and therapeutic approach of HCM patients in Iran. We found more MACE in obstructive versus nonobstructive forms and LGE ≥ 10%. Disopyramide and implantable cardioverter-defibrillator implantation are well-adapted therapeutic options, followed by surgical myectomy. However, genetic testing and alcohol septal ablation are uncommon procedures.
Related Concept Videos
01:29Cardiomyopathy III: Hypertrophic Cardiomyopathy
01:25Cardiomyopathy I: Introduction and Classification
01:29Cardiomyopathy V: Interprofessional Care
01:30Cardiomyopathy II: Dilated Cardiomyopathy
01:29Cardiomyopathy IV: Restrictive Cardiomyopathy
01:23Mitral Stenosis II: Clinical features and Diagnostic Tests