EP receptor activation of AC2 leads to cAMP production in non-raft and nuclear compartments of human ASMs, while β adrenoceptor signalling is broadly detected across microdomains. The activity of PDE4 appears to play a role in maintaining the integrity of compartmentalized EP receptor responses in these cells.

No abstract

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is a relatively under-recognized hereditary cardiomyopathy. It is characterized pathologically by fibro-fatty infiltration of right ventricular (RV) myocardium and clinically by consequences of RV electrical instability. Timely intervention with device therapy and pharmacotherapy may help reduce the risk of arrhythmic events or sudden cardiac death. Here, we describe a classic case of a young adult with ARVC and a brief literature review. The patient presented with exertional palpitations and ARVC was suspected after his routine electrocardiogram (EKG) revealed symmetric T wave inversions and possible epsilon waves in right precordial leads. Subsequent work up showed fatty infiltration of RV myocardium on cardiac magnetic resonance imaging and inducible ventricular tachycardia from the right ventricle during electrophysiologic study. Those findings confirmed the diagnosis of ARVC and warranted treatment with implantable cardioverter defibrillator. It is always exciting to encounter rare pathological entities with classic clinical findings, especially when they present as a diagnostic challenge.We were able to provide correct diagnosis and management, thereby preventing the potentially lethal consequences. Therefore, it is important to recognize the possible EKG findings of ARVC and to know when to pursue further investigations and to implement therapies.

Carotid web is a radiological description of a shelf-like intraluminal filling defect in the carotid bulb. It is histologically defined as atypical fibromuscular dysplasia (FMD), with abnormal fibrosis and smooth muscle cell hyperplasia in the tunica intima. The spur-like intraluminal protrusion can serve as a nidus for thrombus formation, which could cause systemic embolism and ischemic strokes. We report a case of a 20-year-old female patient presenting with acute ischemic stroke in the ipsilateral middle cerebral artery (MCA) territory. We also discuss the incidence, the prevalence, the pathophysiology, the treatment, and the recurrence of carotid web based on the currently available literature.

Patient: Female, 83Final Diagnosis: Right-sided direct carotid cavernous fistulaSymptoms: Chemosis • proptosis and eye painMedication: Topical α2-adrenergic agonistClinical Procedure: Endovascular embolizationSpecialty: Internal Medicine • Interventional Radiology • OphthalmologyObjective:Rare disease/diagnostic challengeBackground:Carotid cavernous fistulas (CCFs) are rare potentially sight-threatening abnormal connections between carotid artery and cavernous sinus.Case Report:We report a case of CCF in an 83-year-old female, who presented with swollen and painful right eye. The patient was initially treated with empiric antibiotics for suspected peri-orbital cellulitis, as noted clinically and in computed tomography (CT) orbits. However, lack of clinical improvement, physical finding of orbital bruit/thrill, and enlarged superior ophthalmic vein in magnetic resonance (MR) orbits suggest alternate diagnoses. Eventually, CT angiogram (CTA) and carotid-arteriography confirmed the diagnosis of right-sided direct CCF, which was subsequently treated with endovascular embolization. Not only does this case highlight the importance of CCF, which could be a differential diagnosis of swollen red eye, it also addresses the vital importance of physical examination in modern medicine despite the seemingly promising technologies.Conclusions:Internists should have a low threshold of clinical suspicion for CCF in a patient with swollen red eyes in order to provide timely and proper management.

Patient: Female, 74Final Diagnosis: Acute coronary syndromeSymptoms: Throat painMedication: —Clinical Procedure: Percutaneous coronary interventionSpecialty: CardiologyObjective:Challenging differential diagnosisBackground:Acute coronary syndrome (ACS) is a common and potentially life-threatening condition encountered in emergency departments. Despite its dreaded nature, nearly one-third of ACS present without chest pain and may mislead clinicians. Additionally, Wellens’ syndrome is a pre-infarction stage of significant proximal left anterior descending (LAD) artery stenosis, which can lead to extensive anterior wall myocardial infarction without timely intervention.Case Report:We report the case of a 74-year-old woman presenting with isolated throat pain and Wellens’ pattern in the initial EKG, which prompted the proper workup and management. Subsequently, coronary angiogram revealed more than 90% occlusion of the proximal LAD artery, and a drug-eluting stent was deployed. The patient did well after the procedure and the follow-up at 2 weeks after discharge was uneventful.Conclusions:This case highlights the importance of awareness of atypical presentation of ACS and importance of Wellens’ syndrome. We also discuss the incidence of craniofacial symptoms of ACS, and the epidemiology, pathophysiology, management, and prognosis of Wellens’ syndrome.

Coronary interventions are the mainstay of treatment for stenotic coronary vascular lesions. New stent designs are constantly being evaluated to improve stent performances and clinical outcomes. Coronary stent fracture is uncommon; however, it is associated with potential major consequences including acute coronary syndrome and the need for repeated target vessel revascularization due to in-stent restenosis or stent thrombosis. We report a case of a 66-year-old man with an extensive cardiac disease history, who presented with intractable angina and was found to have a fracture of a current generation, platinum chromium everolimus-eluting stent (Synergy, Boston Scientific Inc.).

Cardiac calcified amorphous tumor (CAT) is an extremely rare benign intracavitary tumor of the heart. It may mimic other cardiac tumors and can present with signs or symptoms of systemic embolization. There are limited data regarding CAT in the literature. We report a case of a 68-year-old woman with a cardiac CAT and mitral annular calcification (MAC), who presented with acute ST-elevation myocardial infarction (STEMI) and occipital stroke. After extensive review of the literature, we believe that this case is possibly the first description of a cardiac CAT presenting with STEMI. The CAT was surgically removed, and the diagnosis was confirmed by histology. The patient tolerated the surgery and reported no events at 6-month follow-up.