Arrhythmogenic Cardiomyopathy (ACM) is a rare, mostly hereditary, heart disease. It is characterized by the progressive replacement of cardiomyocyte tissue (the tissue that makes up the muscular wall of the heart) with fat and fibrous tissue, affecting the ventricles of the heart, especially the right ventricle. The replacement of cardiomyocyte tissue disrupts the heart’s electrical system and makes it difficult for the heart to function normally. It leads to arrhythmias, often severe, which can cause fainting or even sudden death, especially in young people or athletes.
Symptoms of arrhythmogenic cardiomyopathy are:
- ventricular arrhythmias, which can cause palpitations, tachycardia or fainting.
- shortness of breath and fatigue due to reduced cardiorespiratory capacity.
Diagnosis and Treatment
Diagnosis includes a medical history, a clinical examination with auscultation of the heart, an ultrasound of the heart, an electrocardiogram, and genetic blood tests to identify inherited mutations associated with the condition, especially if there is a family history. Magnetic resonance imaging of the heart is required to provide detailed imaging of the heart walls and detect fatty and fibrous replacement.
Treatment of arrhythmic cardiomyopathy focuses mainly on managing arrhythmias and improving cardiac function with specific medication.
Instructions are given to avoid intense physical exercise, especially for athletes, due to the increased likelihood of arrhythmias in extreme physical situations.
If arrhythmias – mainly ventricular – are dangerous for sudden death, implantation of a cardioverter-defibrillator is recommended.
The prognosis of arrhythmic cardiomyopathy depends on the stage of the disease, the age of diagnosis and the effectiveness of the treatment. With proper therapeutic approach and monitoring, patients can live a good quality of life. However, the disease remains dangerous for arrhythmias and sudden death, so continuous monitoring of the patient is critical for his survival.
