Arrhythmogenic right ventricular cardiomyopathy is an inherited disease characterized by fibrofatty infiltration of the myocardium. Patients suffer from palpitations, presyncope, or syncope, which typically present during or after physical exercise. However, in a minority of cases, sudden cardiac death (SCD) may be the first disease manifestation. SCD in patients with arrhythmogenic right ventricular cardiomyopathy is difficult to predict and there are often no alarming signs or symptoms. Patients with a history of an aborted SCD due to ventricular fibrillation and those who have sustained ventricular tachycardia, or severe dysfunction of the right or left ventricle, or both, are considered to be at high risk for future arrhythmic events. The occurrence of unexplained syncope, non-sustained ventricular tachycardia, and moderate dysfunction of the right or left ventricle, or both, puts the patient at an intermediate risk. Other independent risk factors for adverse events include inducibility at programmed ventricular stimulation, young age at the time of diagnosis, male sex, and compound/digenic heterozygosity of desmosomal gene mutations. Electrocardiographic risk factors for adverse arrhythmic events include T-wave inversion across precordial and inferior leads, low QRS amplitude, and QRS fragmentation. Healthy gene carriers are considered to be at low risk for future arrhythmic events.
Incremental value of electroanatomical mapping for the diagnosis of arrhythmogenic right ventricular cardiomyopathy in a patient with sustained ventricular tachycardia
