Multiple Intramyocardial Masses in an Otherwise Healthy 35-Year-Old Woman.

Sarcoidosis with manifest cardiac involvement typically presents with heart failure, conduction abnormalities, or ventricular arrhythmias. Here, we present a case of a young woman whose presentation raised suspicion for metastatic cardiac disease of unknown primary origin. Further investigation revealed cardiac sarcoidosis with multiple intramyocardial granulomatous masses in the absence of significant enlargement of hilar or mediastinal nodes. This case highlights the following: (i) sarcoidosis can mimic metastatic cardiac tumours; and (ii) hilar and mediastinal lymph nodes can be metabolically active in cardiac sarcoidosis in the absence of significant enlargement.

La sarcoïdose avec atteinte cardiaque patente se manifeste typiquement par une insuffisance cardiaque, des troubles de la conduction ou des arythmies ventriculaires. Nous présentons ici le cas d'une jeune femme dont le tableau clinique évoquait une pathologie cardiaque métastatique d'origine primitive inconnue. Des examens supplémentaires ont révélé une sarcoïdose cardiaque associée à de multiples granulomes intramyocardiques, en l'absence d'hypertrophie importante des ganglions hilaires ou médiastinaux. Ce cas illustre les points suivants : (i) les symptômes de la sarcoïdose peuvent imiter ceux de tumeurs cardiaques métastatiques; et (ii) les ganglions lymphatiques hilaires et médiastinaux associés à une sarcoïdose cardiaque peuvent présenter une activité métabolique en l'absence d'hypertrophie importante.

Exercise and arrhythmic risk in TMEM43 p.S358L arrhythmogenic right ventricular cardiomyopathy.

BACKGROUND: High-level exercise has been associated with a malignant phenotype in desmosomal and genotype-negative forms of arrhythmogenic right ventricular cardiomyopathy (ARVC). This is the first study to examine this issue with ARVC secondary to the TMEM43 p.S358L mutation. OBJECTIVE: The purpose of this study was to evaluate the impact of exercise on arrhythmic risk and cardiac death in TMEM43 p.S358L ARVC. METHODS: Individuals with the TMEM43 p.S358L mutation enrolled in a prospective registry who had received a primary prevention implantable cardioverter-defibrillator (ICD) were invited to complete the modified Paffenbarger Physical Activity Questionnaire to assess their physical activity in the year before their ICD implantation. Time-to-event analyses using unadjusted and adjusted Cox proportional hazards models evaluated associations between physical activity and first appropriate ICD discharge secondary to malignant ventricular arrhythmia or cardiac death. RESULTS: In 80 subjects with the TMEM43 p.S358L mutation, exercise ≥9.0 metabolic equivalent of task (MET)-hours/day (high level) in the year before ICD implantation was associated with an adjusted 9.1-fold increased hazard of first appropriate ICD discharge (there were no deaths) relative to physical activity <9.0 MET-hours/day (moderate level) (95% confidence interval [CI] 3.3-24.6 MET-hours/day; P < .001). The median age from birth to first appropriate ICD discharge was 58.5 years (95% CI 56.5-60.5 years) vs 35.8 years (95% CI 28.2-43.4 years) (P < .001) in subjects in moderate- and high-level exercise groups, respectively. CONCLUSION: Exercise ≥9.0 MET-hours/day is associated with an increased risk of malignant ventricular arrhythmias in the TMEM43 p.S358L subtype of ARVC. Extrapolating these data, we suggest molecular testing be offered in early childhood to inform exercise choices reflective of the genotype.

QT prolongation and monomorphic VT caused by pheochromocytoma.

INTRODUCTION: Pheochromocytoma may present with ECG abnormalities as one of the few clues to the diagnosis. METHODS AND RESULTS: A 30-year-old woman presented with chest pain and a QTc of 525 ms. Four weeks later following a syncopal episode, her QTc was 660 ms. Short runs of monomorphic ventricular tachycardia were recorded. Investigations revealed an adrenal pheochromocytoma. Her QTc normalized after excision of the tumor. Comprehensive QT gene screening of KCNQ1, KCNH2, SCN5A, KCNE1, and KCNE2 was negative. CONCLUSION: Pheochromocytoma may induce monomorphic VT and QT prolongation. The interaction of different catecholamines may have a compounding effect on cardiac repolarization.

Management of infections involving implanted cardiac electrophysiologic devices.

Indications for cardiac electrophysiologic device implantation have expanded, and the target demographic has widened. Unfortunately, these changes have been accompanied by an increase in cardiac device-associated infections out of proportion to the increase in implantation rate. Diagnosing a cardiac device infection may be challenging because of the spectrum of clinical manifestations, ranging from isolated generator pocket pain to frank sepsis with clear evidence of endocarditis. Any component of the device may be involved, but the cornerstone of therapy remains extraction of the device and its leads along with appropriate antibiotic treatment. Given the inherent risks of lead extraction, making the correct diagnosis is paramount. This review outlines the epidemiology, pathophysiology, clinical manifestations, diagnosis, and management of cardiac device infections.