HRCR Rare Diseases Article Collection
Exercise-induced arrhythmogenic right ventricular cardiomyopathy: Reverse remodeling with detraining
Long-term exercise training leads to structural cardiac adaptations, collectively referred to as the “athlete’s heart.” While the ventricles both undergo dilation and eccentric hypertrophy, it has been shown that the right ventricle (RV) experiences disproportionate remodeling under intense sports activity.1 Occasionally, the remodeling that occurs in the athlete’s heart may resemble arrhythmogenic right ventricular cardiomyopathy (ARVC), a pathologic cardiomyopathy associated with sudden death.Exercise-induced Brugada syndrome type 1 pattern
Brugada syndrome (BrS) is an inherited disorder usually afflicting patients with otherwise structurally normal hearts. Many triggers have been identified that can result in the phenotypic expression of the higher-risk BrS type 1 pattern. These triggers include but are not limited to fever, heightened vagal tone, and sodium (Na) channel blocking agents. Conversion to the type 1 pattern during exercise is unusual and in fact isoproterenol is a treatment for ventricular tachycardia (VT) storm in Brugada syndrome.Unmasked type 1 Brugada ECG pattern without a fever after a COVID-19 vaccination
Brugada syndrome (BrS) patients are regarded as a high-risk population under the COVID-19 pandemic because infection-induced fevers may unmask a type 1 Brugada electrocardiogram (ECG) pattern potentially leading to lethal ventricular arrhythmias.1 Vaccinations seem to be crucial in BrS patients; however, careful observation and antipyretic drugs are necessary for vaccine-induced fevers.2 Among the enormous number of COVID-19 vaccinations around the world, although the incidence is rare, death events, including sudden cardiac death, after a vaccination have been reported.A case of cardiac sarcoidosis with concurrent myocardial ischemia
Sarcoidosis is a rare multisystem disease of unknown etiology affecting 10–40 in 100,000 population, characterized by granulomatous inflammation.1 It has a diverse set of presentations ranging from diffuse to localized disease and can have either acute or chronic clinical course with multiple organ involvement.1 Classically, lungs are the most commonly affected organ, but systemic, dermatologic, and cardiac involvement also occur. Specifically, 20%–30% of sarcoidosis patients have been observed to have cardiac involvement in an autopsy1; however, only about 5% have a symptomatic presentation with cardiac disease.Parahisian pacing to unmask Brugada pattern with concomitant left bundle branch block and to document epicardial ablation endpoint in Brugada syndrome
Brugada syndrome, traditionally described as a channelopathy affecting transmembrane sodium current, has been recognized as a structural disease affecting the epicardial right ventricular outflow tract (RVOT).1–3 This observation has been the basis for successful arrhythmic substrate ablation in these patients, with resolution of the Brugada pattern in the anterior precordial leads reported as a reliable procedural endpoint.4,5 However, the typical precordial electrocardiogram (ECG) pattern is obscured in patients with concomitant left bundle branch block (LBBB),6 making this a challenging observation in such patients.
