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Myocarditis in hypertrophic cardiomyopathy patients presenting acute clinical deterioration
Andrea Frustaci1, Romina Verardo, Marina Caldarulo
1Heart and Great Vessels Attilio Reale Department, La Sapienza University, Viale del Policlinico 155, Rome, Italy. biocard@inmi.it
Insights
Myocarditis, an inflammation of the heart muscle often caused by viruses, frequently leads to acute clinical deterioration in patients with hypertrophic cardiomyopathy (HCM). Identifying myocarditis in HCM patients can improve prognosis and treatment strategies.
Area of Science:
- Cardiology
- Pathology
- Genetics
Background:
- Hypertrophic cardiomyopathy (HCM) is a genetic heart muscle disease.
- Acute clinical deterioration in HCM patients can be severe and life-threatening.
- The role of myocarditis in HCM exacerbations is not well understood.
Purpose of the Study:
- To investigate the prevalence and impact of myocarditis in patients with hypertrophic cardiomyopathy.
- To determine if myocarditis is a significant factor in acute electrical instability and clinical decline in HCM.
Main Methods:
- 119 HCM patients (42 with acute deterioration, 77 stable) underwent cardiac catheterization and endomyocardial biopsy.
- Biopsies were analyzed for histology, immunohistochemistry, and viral genomes.
- Gene analysis of sarcomeric proteins was performed; controls had mitral stenosis.
Main Results:
- Histological findings consistent with HCM were present in all patients.
- Active myocarditis (CD45RO+ lymphocytes and myocyte necrosis) was found in 28 of 42 unstable HCM patients, but none of the stable patients.
- Viral genomes were detected in 14 of the 28 myocarditis patients, but not in stable HCM patients or controls.
Conclusions:
- Viral myocarditis is a common cause of acute clinical deterioration in hypertrophic cardiomyopathy.
- Recognizing myocarditis in HCM patients is crucial for potentially improving disease prognosis and guiding treatment decisions.
Aim:
We sought to determine whether myocarditis can be a major cause of acute electrical instability or clinical deterioration in HCM patients.
Methods And Results:
A total of 119 HCM patients (69 M/50F, mean age 41 +/- 8), 42 with acute clinical deterioration and 77 clinically stable, underwent cardiac catheterization with left ventricular endomyocardial biopsy and gene analysis of major sarcomeric proteins. Endomyocardial tissue was processed for histology, immunohistochemistry, and polymerase chain reaction for the most common cardiotropic viruses. Controls were surgical samples from 50 patients with mitral stenosis. All 119 patients showed histological findings suggestive of HCM. In addition, CD45RO+ lymphocytes (> or =14/mm(2)) with focal necrosis of the adjacent severely hypertrophied and often disorganized myocytes, consistent with an overlapping active myocarditis, were observed in 28 of 42 unstable and none of 77 stable HCM patients. A viral genome was detected in 14 of 28 patients with myocarditis and in none of HCM patients without and in none of controls. No correlation between sarcomeric protein gene mutations and HCM clinical profile was observed.
Conclusion:
Myocarditis, often viral, represents a common cause of acute clinical deterioration in HCM. Its recognition can potentially affect disease prognosis and treatment.
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