Sarcoidosis: Hiding in Plain Sight

Introduction: Sarcoidosis is a systemic disease that can masquerade as many conditions. Due to its often patchy distribution, myocardial biopsy is only ∼30% sensitive. Herein we present a case of sarcoidosis diagnosed from an extra-cardiac biopsy years after the onset of cardiomyopathy. Case Report: A 54 year old man initially presented with dyspnea and fatigue due to new onset HFrEF of 20%. He had a significant family history of SCD. His workup revealed non-obstructive CAD, a myxomatous mitral valve with bileaflet prolapse and moderate regurgitation, and a cystic structure attached to the tricuspid valve. Given his frequent ventricular ectopy, he was discharged on guideline therapy for HFrEF and a wearable cardioverter defibrillator. Genetic testing for dilated cardiomyopathies revealed EYA4 and TTN variants of unknown significance. A cardiac MRI a few months later demonstrated no abnormal late gadolinium enhancement and persistent systolic dysfunction, so he underwent AICD implant. His course was further complicated by recurrent admissions for supraventricular and ventricular tachyarrhythmias. A cardiopulmonary exercise stress test demonstrated low-risk results. Further evaluation noted prolonged AV conduction. A RHC revealed mild post-capillary pulmonary hypertension and a mildly decreased cardiac index at 2.01 L/min/m2. Months later, he underwent CRT-D upgrade. He started improving functionally until he developed left eyelid swelling, initially thought to be lymphoma. An orbital biopsy revealed non-caseating granulomas. A PET/CT demonstrated FDG uptake within subcarinal lymph nodes but no cardiac uptake. Initially he declined immunomodulatory therapy, but as his symptoms worsened, he started corticosteroids and transitioned to mycophenolate mofetil due to intolerance of methotrexate. Due to disease progression on repeat FDG-PET, he was switched to infliximab. The diagnosis of sarcoidosis can be difficult due to its variable presentation, including heart block, heart failure, ventricular arrhythmias and sudden cardiac death. Cardiac involvement occurs in 25% of cases. Our case was complicated by a cMRI that was negative for cardiac involvement, which emphasizes the importance of complimentary inflammatory imaging with FDG-PET. A multidisciplinary approach is needed that engages sarcoid specialists for earlier diagnosis to ensure rapid initiation of therapy to reduce end-organ dysfunction

Compression wraps as adjuvant therapy in the management of acute systolic heart failure

BACKGROUND: Guidelines recommend targeting decongestion in management of decompensated HF, with lower extremity edema often serving as the clinical target. LECW are seldom used in the acute setting, with a paucity of data on efficacy in HF, despite serving as a cornerstone of chronic lymphedema management. PRIMARY OBJECTIVE: Study the efficacy and safety of LECW in acute decompensated HF. METHODS: Open-label, randomized, parallel-group clinical trial. PRIMARY OUTCOMES: Days on intravenous (IV) diuretic therapy, total hospital length of stay (LOS), and 30-day all-cause readmission. RESULTS: 32 patients were enrolled, with 29 patients completing the study. Enrollment was suspended due to the COVID-19 pandemic. Overall LOS was shorter in the intervention group (3.5 vs 6 days, p = 0.05), with no significant difference in total days on IV diuresis or 30-day readmission rate with use of LECW. Fewer patients required continuous diuretic infusion after treatment with LECW (0 vs 7 patients, p = 0.027). The intervention group scored significantly better on the MLWHF (55.5 vs 65, p = 0.021), including both the physical and emotional dimension scores. No adverse events were reported with use of LECW, including a significantly lower incidence of AKI (1 vs 13 patients, p = 0.005). CONCLUSION: The use of LECW resulted in reduced hospital LOS compared to standard therapy, with no difference in days of IV diuresis administration or 30-day readmission. Treatment with LECW also resulted in less continuous IV diuretic therapy, fewer incidence of AKI, and improved quality of life. Trends toward less escalation of diuresis, and greater reduction in edema were also observed

Cardio-Rheumatology: Prevention of Cardiovascular Disease in Inflammatory Disorders

Inflammation plays a well-established role in the development and progression of atherosclerosis. Individuals exposed to chronic inflammation are at an increased risk of developing cardiovascular disease, including coronary artery disease and heart failure, independent of associated traditional risk factors. Traditional risk assessment tools and calculators underestimate the true cardiac risk in this population. In addition to this, there is a lack of awareness on the association between inflammation and cardiovascular disease. These factors lead to undertreatment in terms of preventive cardiac care in patients with chronic inflammatory disease

Unity, diversity and the challenge of sustainable development in the Mediterranean

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Unity, Diversity and the Challenge of Sustainable Development: an Introduction to the Mediterranean

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Eosinophilic myocarditis in a patient with biopsy proven systemic sarcoidosis who was referred for bradycardia

Background Eosinophilic myocarditis (EM) is an understudied and often missed diagnosis whose constellation of symptoms are frequently attributed to other morbidities. Case A 57-year-old woman with mediastinal lymphadenopathy (Figure 1A), uveitis, celiac lymph node biopsy in 2013 demonstrating noncaseating granulomas and a questionable prior diagnosis of hypereosinophilic syndrome presented to establish care for dizziness and bradycardia (Figure 1B). She had a stroke in 2004 with an echocardiographic diagnosis of left ventricular (LV) thrombus (Figure 1C-D) for which she was prescribed coumadin. She exhibited a pruritic maculopapular skin rash on several areas of her body (Figure 1E-F) and laboratory tests showed a history of peripheral eosinophilia. Decision-making There was concern for cardiac sarcoidosis or undiagnosed EM. A cardiac MRI demonstrated thickening of the LV apex (Figure G-H) with corresponding sub endocardial perfusion defect and late gadolinium enhancement, without LV thrombus. These findings were consistent with late stage myocardial fibrosis from EM. Reliance on the single imaging modality of echocardiogram resulted in past misdiagnosis of thrombus and delayed therapy of myocardial fibrosis in this patient. Conclusion This case highlights the need for awareness of EM and the importance of considering alternative or additional diagnoses in patients with complex past medical histories. An association of sarcoidosis and EM has not yet been reported in the literature

A CASE OF MARANTIC ENDOCARDITIS OF THE AORTIC VALVE IN A PATIENT WITH METASTATIC COLON CANCER

Background: A 74-year-old male with history of coronary artery disease and complex peripheral arterial disease presented to our emergency department with shortness of breath and melena. Case: The patient presented with a two-week history of shortness of breath and melena. Physical exam was significant for a 4/6 systolic ejection murmur at the left sternal border and cold lower extremities with diminished pedal pulses. Laboratory evaluation revealed hemoglobin of 5.8 g/dL and CEA level \u3e 950 ng/mL. CT scan of the abdomen demonstrated findings concerning for metastatic disease to the liver and concern for peritoneal carcinomatosis. Subsequently, colonoscopy demonstrated a large ascending colonic mass with biopsy revealing invasive, poorly-differentiated carcinoma. A transesophageal echocardiogram (TEE) revealed large (1.35 cm x 0.29 cm) highly mobile sub-valvular and valvular-proper aortic vegetations. Serial blood cultures were negative, including HACEK organisms. Patient\u27s course was complicated with development of scattered, punctate erythematous macules of the right foot consistent with emboli. Decision-making: Given TEE findings and diagnosis of colonic adenocarcinoma, the initial leading diagnosis was aortic valve endocarditis. After infectious etiologies were excluded, the presumptive diagnosis of marantic endocarditis with thromboemboli was made. Antibiotics were stopped and the patient was started on anticoagulation. Conclusion: Marantic endocarditis is a rare, noninfectious endocarditis. It predominantly affects the mitral and aortic valves, with deposition of platelet thrombi. Advanced malignancies of the lung and gastrointestinal tract comprise a majority of cases, yet most cases are diagnosed at autopsy. Antemortem cases most commonly present with thromboembolic phenomena. We describe an antemortem case of aortic valve marantic endocarditis in a patient with advanced colon adenocarcinoma who developed thromboembolic sequela