Brugada Syndrome in a Patient with Vascular Ehlers-Danlos Syndrome: Sudden Death Risk Amplified.

The vast majority of sudden cardiac arrests occur in patients with structural heart disease and in approximately 10% of the cases, it can occur in those with structurally normal hearts. Brugada syndrome is an autosomal dominant sodium channelopathy that has been implicated in sudden deaths. Given their low prevalence, our knowledge about Brugada syndrome is still evolving. Apart from schizophrenia, there have been no reports of associated medical conditions. We recently encountered a patient with vascular Ehlers-Danlos syndrome who was also found to have Brugada syndrome. Both these conditions share some common clinical presentations including a propensity for sudden death.

DPP4 inhibitors and cardiovascular outcomes: safety on heart failure.

Diabetes is an important risk factor for cardiovascular disease. However, clinical data suggests intensive glycemic control significantly increase rather than decrease cardiovascular mortality, which is largely due to the fact that a majority of oral anti-diabetic drugs have adverse cardiovascular effect. There are several large-scale clinical trials evaluating the cardiovascular safety of DPP4 inhibitors, a novel class of oral anti-diabetic medications, which have been recently completed. They were proven to be safe with regard to cardiovascular outcomes. However, concerns on the safety of heart failure have been raised as the SAVOR-TIMI 53 trial reported a 27% increase in the risk for heart failure hospitalization in diabetic patients treated with DPP4 inhibitor saxagliptin. In this review, we will discuss recent advances in the heart failure effects of DPP4 inhibition and GLP-1 agonism.

Invasive Cardiac Lipoma: a case report and review of literature.

BACKGROUND: Cardiac lipomas are rare benign tumors of the heart. They are usually asymptomatic and are thus most often diagnosed on autopsies. Symptoms, when present, depend upon the location within the heart. Typical locations are the endocardium of the right atrium and the left ventricle. Diagnostic modality of choice is cardiac MRI. Treatment guidelines have not yet been established due to the very low prevalence of these tumors and are thus guided by the patient's symptomatology. CASE PRESENTATION: We describe a case of an invasive cardiac lipoma, wherein the initial symptom of the patient was shortness of breath. Although the echocardiogram visualized the tumor in the right atrium, a cardiac MRI was performed for better tissue characterization. The MRI revealed a large, fat containing, septated mass in the right atrium with invasion into the inter-atrial septum and inferior cavoatrial junction. There was also invasion of the coronary sinus along the inferior and left lateral aspect of the posterior atrioventricular groove. Although the mass appeared to represent a lipoma by imaging characteristics, the unusual extension into the coronary sinus led to consideration of a low-grade liposarcoma in the differential. Thus a pre-operative biopsy was performed along with MDM2 gene amplification to rule out a liposarcoma preceding surgical excision. CONCLUSION: Cardiac lipomas are well-characterized on cardiac MRI, which is the diagnostic modality of choice. Typical locations are the right atrium and the left ventricle. However, in those with atypical features such as invasion of the coronary sinus, pre-operative biopsy for histopathologic confirmation is imperative to exclude well-differentiated liposarcoma. Our patient with a simple lipoma underwent partial resection to relieve symptoms. We discuss prognosis and treatment of symptomatic cardiac lipomas.

Role of electromagnetic navigational bronchoscopy in pulmonary nodule management.

The incidence of pulmonary nodules and lung cancer is rising. Some of this increase in incidence is due to improved pick up by newer imaging modalities. However, the goal is to diagnose these lesion, many of which are located in the periphery, by safe and relatively non-invasive methods. This has led to the emergence of numerous techniques such as electromagnetic navigational bronchoscopy (ENB). Current evidence supports a role for these techniques in the diagnostic pathway. However, numerous factor influence the diagnostic accuracy. Thus despite significant advances, more research needs to be undertaken to further improve the currently available diagnostic technologies.

Lipid Management Guidelines from the Departments of Veteran Affairs and Defense: A Critique.

In December 2014, the US Department of Veterans Affairs and Department of Defense (VA/DoD) published an independent clinical practice guideline for the management of dyslipidemia and cardiovascular disease risk, adding to the myriad of recently published guidelines on this topic. The VA/DoD guidelines differ from major US guidelines published by the American College of Cardiology/American Heart Association in 2013 in the following ways: recommending moderate-intensity statins for the majority of patients with statin indications regardless of atherosclerotic cardiovascular disease risk; advocating for limited on-treatment lipid monitoring; and deemphasizing ancillary data, such as coronary artery calcium testing, to improve atherosclerotic cardiovascular disease risk estimation. In the context of manifold treatment recommendations from numerous guideline committees, the VA/DoD recommendations may generate further confusion and mixed messages among healthcare providers about the optimal treatment of dyslipidemia. In this review, we critically appraise the VA/DoD recommendations with a focus on the evidence base for each area where the VA/DoD guidelines differ from the American College of Cardiology/American Heart Association guidelines. We also call for harmonization of lipid treatment guidelines to ensure high-quality and consistent care for patients with, and at risk for, atherosclerotic cardiovascular disease.

Hypercalcemia and diffuse osteolytic lesions in a 45-year-old patient with myeloid sarcoma with megakaryocytic differentiation.

Acute megakaryocytic leukemia is a rare form of acute myeloid leukemia that carries a poor prognosis. As most cases of osteolytic lesions are due to plasma cell and myeloid malignancies, maintaining a broad differential directly influences clinical course. We document a 45-year-old patient with progressive constitutional symptoms, osteolytic bone lesions in the setting of hypercalcemia, who developed acutely worsening pancytopenia. The diagnosis of myeloid sarcoma with megakaryocytic differentiation was made after obtaining tissue from osteolytic bone that stained strong for CD34. Immunohistochemical testing underscores the importance of how serologic and urine testing remains limited and can delay early diagnosis in this disease.

PCSK9 inhibitors and their role in high-risk patients in reducing LDL cholesterol levels: evolocumab.

Patients with familial hypercholesterolemia or statin intolerance are especially challenging to manage since LDL cholesterol levels often remain considerably elevated despite clinicians' best efforts. With statins regarded as first-line pharmacologic therapy by the current American College of Cardiology/American Heart Association guidelines to reduce LDL cholesterol and cardiovascular risk, there is now a critical need to determine when other agents will play a role beyond maximally tolerated statin therapy and lifestyle changes. In this review, we take a closer look at evolocumab (Repatha(®)), one of the new injectable human monoclonal antibodies to PCSK9 and its efficacy and safety properties from the results of various trials.

PCSK9 inhibitors and their role in high-risk patients in reducing LDL cholesterol levels: alirocumab.

In this review, we examine alirocumab (Praluent(®)), a monoclonal antibody to PCSK9 and its role in reducing LDL-C levels. By comparing the results of various studies and trials we discuss the efficacy and safety of alirocumab. We aim to guide clinicians of the role of alirocumab in clinical practice. Overall, PCSK9 inhibitors are promising new agents in further reducing LDL-C levels in addition to diet and maximally tolerated statin therapy. Long-term outcome studies are currently ongoing and will further delineate the role of PCSK9 inhibitors.

When a pulmonary embolism is not a pulmonary embolism: a rare case of primary pulmonary leiomyosarcoma.

Arterial leiomyosarcomas account for up to 21% of vascular leiomyosarcomas, with 56% of arterial leiomyosarcomas occurring in the pulmonary artery. While isolated cases of primary pulmonary artery leiomyosarcoma document survival up to 36 months after treatment, these uncommon, aggressive tumors are highly lethal, with 1-year survival estimated at 20% from the onset of symptoms. We discuss a rare case of a pulmonary artery leiomyosarcoma that was originally diagnosed as a pulmonary embolism (PE). A 72-year-old Caucasian female was initially diagnosed with 'saddle pulmonary embolism' based on computerized tomographic angiography of the chest 2 months prior to admission and placed on anticoagulation. Dyspnea escalated, and serial computed tomography scans showed cardiomegaly with pulmonary emboli involving the right and left main pulmonary arteries with extension into the right and left upper and lower lobe branches. An echocardiogram on admission showed severe pulmonary hypertension with a pulmonary artery pressure of 82.9 mm Hg, and a severely enlarged right ventricle. Respiratory distress and multiorgan failure developed and, unfortunately, the patient expired. Autopsy showed a lobulated, yellow mass throughout the main pulmonary arteries measuring 13 cm in diameter. The mass extended into the parenchyma of the right upper lobe. On microscopy, the mass was consistent with a high-grade primary pulmonary artery leiomyosarcoma. Median survival of patients with primary pulmonary artery leiomyosarcoma without surgery is one and a half months, and mortality is usually due to right-sided heart failure. Pulmonary artery leiomyosarcoma is a rare but highly lethal disease commonly mistaken for PE. Thus, we recommend clinicians to suspect this malignancy when anticoagulation fails to relieve initial symptoms. In conclusion, early detection and suspicion of pulmonary artery leiomyosarcoma should be considered in patients refractory to anticoagulation, prompting initiation of early intervention.

Isolated pulmonic valve endocarditis presenting as neck pain.

We discuss a unique case of a 52-year-old man with no history of intravenous drug use or dental procedures who presented with neck pain, 2 weeks of fevers, chills, night sweats, cough, and dyspnea found to have isolated pulmonic valve (PV) endocarditis. The patient did not have an associated murmur, which is commonly seen in right-sided infectious endocarditis. A transthoracic echocardiogram showed a thickened PV leaflet, with subsequent transesophageal echocardiogram showing a PV mass. Speciation of blood cultures revealed Streptococcus oralis. In right-sided infective endocarditis, usually the tricuspid valve is involved; however, in our case the tricuspid valve was free of any mass or vegetation. The patient did meet Duke criteria and was thus started on long-term intravenous antibiotics for infectious endocarditis. The patient's symptoms quickly improved with antibiotics. A careful history and evaluating the patient's risk factors are key in earlier detection of infective endocarditis (IE). Because of early detection and a high index of suspicion, the patient had no further complications and did not require any surgery. In conclusion, clinical suspicion of right-sided IE should be high in patients who present with persistent fevers and pulmonary symptoms in order to reduce the risk of complications, and to improve outcomes.