Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is a rare genetic heart condition characterized by the progressive replacement of normal heart muscle tissue with fibrous and fatty tissue in the right ventricle (the chamber responsible for pumping blood to the lungs), leading to abnormal heart rhythms.
ARVC typically develops during adolescence or early adulthood, although it can occur at any age. The condition is primarily inherited, with mutations in various genes associated with the disease. However, certain environmental factors may contribute to its manifestation.
The main clinical manifestation of ARVC is the occurrence of arrhythmias, which are irregular heart rhythms. These arrhythmias can be potentially life-threatening, as they can lead to sudden cardiac arrest or even sudden death. Symptoms may include palpitations, lightheadedness, fainting, and shortness of breath. In some cases, heart failure can also occur as a result of the weakened heart muscle's reduced ability to pump blood efficiently.
Diagnosis of ARVC involves a combination of medical history analysis, physical examination, electrocardiogram (ECG), echocardiogram, cardiac magnetic resonance imaging (MRI), and genetic testing. Treatment options typically involve managing symptoms and reducing the risk of complications through medications to control heart rhythm, implantation of defibrillators to monitor and treat life-threatening arrhythmias, and lifestyle modifications, such as avoiding intense physical activity and certain stimulant medications.
Long-term management of ARVC involves regular follow-up with healthcare professionals, proper medication adherence, and close monitoring for any changes in heart function or symptoms. In some cases, heart transplantation may be necessary if the disease progresses severely and life-threatening complications cannot be controlled through other therapeutic approaches.
