Atypical form of arrhythmogenic cardiomyopathy

A case of a family suffering from arrhythmogenic cardiomyopathy affecting dominantly the left ventricle of the heart was diagnosed in our hospital. A forty-six-year-old man was admitted to the emergency room because of a collapse with both respiratory and circulatory arrest during a sport activity. Fibrillation of the ventricles was initially registered upon electrocardiography, and there had been several sudden deaths at a young age in the patient's family. There was no significant stenosis of his coronary arteries and because of the electrocardiographic and echocardiographic findings, cardiac magnetic resonance was indicated. The cardiac magnetic resonance finding on the patient's heart probably corresponds to a less frequent type of arrhythmogenic cardiomyopathy with dominant left ventricular involvement. The patient's first-degree relatives underwent comprehensive examinations at our cardiology department, and a similar cardiac magnetic resonance finding concerning the heart was made in the younger brother of the proband. Both brothers had an implantable cardioverter-defibrillator implanted and the whole family remains subject to regular follow-ups at our department.