[Clinical profile and therapeutic strategies in patients with arrhythmogenic cardiomyopathy treated in a national reference institute].

Objective: To determine the epidemiological, clinical, electrocardiographic, imaging characteristics and main therapeutic strategies performed in patients with arrhythmogenic cardiomyopathy treated in a national reference cardiovascular institute. Materials and methods: Observational, descriptive and retrospective study that attempts to identify the clinical characteristics, complementary tests and therapeutic strategies performed in patients with arrhythmogenic cardiomyopathy treated at the Instituto Nacional Cardiovascular - INCOR EsSalud in Lima, Peru. Results: Thirteen patients were found with arrhythmogenic cardiomyopathy. The median age at which the diagnosis was made was 38.2 years and 69.3% were male. The most frequent clinical manifestations were tachycardic palpitations (92.3%), presyncope (84.6%) and heart failure (69.2%). 23% of the patients suffered a cardiac arrest. All the patients presented at least one episode of ventricular tachycardia, 92.3% with complete left bundle branch block morphology and upper axis. 76.9% received an implantable cardioverter defibrillator (ICD), 15.3% underwent ablation and 15.3% received a heart transplant. 84.6% of the patients live to this day. Conclusions: Arrhythmogenic cardiomyopathy predominantly affected the young and male population. All the patients had a potentially fatal ventricular arrhythmia. Biventricular disease by echocardiography and cardiac magnetic resonance occurred in 69.2% and 100% of the cases, respectively. The therapeutic strategies used were antiarrhythmic medical treatment, placement of an ICD as secondary prevention, ablation, and heart transplantation. To date, 84.6% of patients survive.