Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a disease that most often manifests in young, apparently healthy adults. It is the most common cause of sudden death in athletes in Europe. Researchers at the National Institute of Cardiology are looking for a way to prevent the progression of the disease.
ABM to fund study on arrhythmogenic right ventricular cardiomyopathy
Published April 17, 2023 09:06
From April 17 to 24, we are celebrating Heart Week. Cardiovascular diseases pose the greatest challenge to modern medicine, especially since young people are increasingly affected.
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare heart disease characterized by progressive replacement of the heart muscle with fatty or fibrous tissue. This results in cardiac arrhythmias that can be life-threatening especially in young people. ARVC is genetically determined. The disease can have a variety of symptoms, most commonly manifested by palpitations and loss of consciousness during exertion, often in a person unaware of his disease. In later stages of the disease, there are symptoms of heart failure: edema, enlargement of the abdominal girth, and reduced exercise tolerance. Additional examinations reveal an abnormal electrocardiogram, right ventricular dilatation and abnormal contractility, which is best visualized by cardiac ECHO and MRI.
- People with ARVC, and even asymptomatic carriers of the pathogenic mutation, are forbidden to participate in sports, as intense exercise promotes disease progression and worsens prognosis. No drugs are known to slow down or retard the progression of the disease, explains Prof. Dr. Elżbieta Katarzyna Biernacka, the principal investigator.
Prevent disease progression
Researchers at the National Institute of Cardiology will conduct a study to test whether sacubitril/valsartan prevents disease progression in patients with ARVC. The study is funded by the Medical Research Agency.
-We hope that the drug (sacubitril/valsartan) we will offer our patients will halt the progression of the severe disease of arrhythmogenic right ventricular cardiomyopathy. The drug is expected to have an inhibitory effect on myocardial fibrosis, and it is fibrosis that is the cause of progressive contractile and arrhythmogenic cardiomyopathy in these patients," explains Prof. Elżbieta Katarzyna Biernacka, MD, principal investigator.
The primary objective of the ARNI-ARVC multicenter, randomized trial is to evaluate the anti-remodeling and anti-fibrotic effects of sacubitril/valsartan in ARVC, which would be expected to prevent disease progression and translate into an improved clinical course: delay or prevent the onset of heart failure symptoms and result in a reduction in arrhythmias. The cause of structural changes and dangerous arrhythmias in ARVC is fibro-fatty remodeling of the myocardium. The use of the new drug, thanks to its multidirectional neurohumoral activity, including modification of myocyte arrhythmic potential and inhibition of myocardial fibrosis processes, can stop the progression of structural changes leading to heart failure and the formation of new foci of ventricular arrhythmias.
The ARNI-ARVC study will enroll 100 patients with ARVC diagnosed on the basis of current diagnostic criteria and normal or indirectly reduced left ventricular ejection fraction (LVEF). Recruitment will be conducted by 12 cardiac centers in Poland. Patients will be block randomized in a 3:2 ratio to treatment with sacubitril/valsartan or to a control group. Observation of each patient will be conducted for 4 years.
Source: ABM
