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Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model
Published on: August 8, 2022
Hypertrophic cardiomyopathy secondary to hepatitis C virus-related vasculitis
Giulio Cavalli1, Alvise Berti, Gabriele Fragasso
1aDepartment of Internal Medicine and Clinical Immunology bDepartment of Cardiology cDepartment of Radiology, IRCCS San Raffaele Scientific Institute, Milan, Italy.
Insights
Cryoglobulinemic vasculitis, linked to HCV infection, can rarely cause severe cardiac issues like hypertrophic cardiomyopathy. Treatment with rituximab led to significant recovery of heart function and structure.
Area of Science:
- Cardiology
- Rheumatology
- Nephrology
Background:
- Cryoglobulinemic vasculitis is a small-vessel vasculitis typically linked to chronic Hepatitis C Virus (HCV) infection.
- It commonly affects the skin, kidneys, and peripheral nervous system.
- Cardiac involvement is an exceptionally rare but severe complication.
Observation:
- A case of hypertrophic cardiomyopathy secondary to cryoglobulinemic vasculitis is presented.
- Cardiac imaging revealed severe left ventricular hypertrophy, diffuse hypokinesia, reduced ejection fraction, and subtle late gadolinium enhancement.
- These findings suggest cardiac microcirculation involvement in cryoglobulinemic vasculitis.
Findings:
- The patient was treated with the anti-CD20 monoclonal antibody rituximab.
- Following treatment, significant clinical improvement was observed.
- Cardiac ultrasound and MRI demonstrated a marked reduction in left ventricular hypertrophy and improved cardiac function.
Implications:
- This case highlights rituximab as an effective treatment for cardiac manifestations of cryoglobulinemic vasculitis.
- It underscores the importance of considering cardiac involvement in patients with cryoglobulinemic vasculitis.
- Further research may elucidate the mechanisms of cardiac microcirculation involvement in this condition.
Abstract:
: Almost invariably associated with chronic HCV infection, cryoglobulinemic vasculitis is a small-vessel vasculitis commonly affecting the skin, kidneys, and peripheral nervous system. Cardiac involvement, possibly due to cardiac microcirculation involvement, is an utterly rare and severe complication. We describe a case of hypertrophic cardiomyopathy secondary to cryoglobulinemic vasculitis. Evaluation with transthoracic cardiac ultrasound and cardiac MRI evidenced severe left ventricular hypertrophy and diffuse hypokinesia, a marked decrease in left ventricular ejection fraction, and a subtle late enhancement of inferior and lateral left ventricular walls. Upon clinical stabilization, the patient received treatment with anti-CD20 monoclonal antibody rituximab. Clinical and radiological follow-up with cardiac ultrasound and cardiac MRI documented a dramatic and sustained clinical improvement, with marked reduction of left ventricular hypertrophy, resolution of late enhancement, recovery of left ventricular contractility and function.
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