Cardiovascular manifestations of Erdheim-Chester disease: A narrative review with two cases.

Erdheim-Chester disease (ECD) is a rare 'L' (Langerhans) group histiocytic neoplasm that affects a multitude of organ systems, causing osteosclerotic bone lesions, periaortic encasement ('coated' aorta), retroperitoneal fibrosis involving kidneys and ureters ('hairy kidney'), and infiltration of the central nervous system. Cardiovascular involvement can occur in up to 70% of patients and is usually found during computed tomography/magnetic resonance imaging evaluation. When present, cardiovascular symptoms can have wide variability in presentation from asymptomatic to pericarditis, fatal cardiac tamponade, myocardial infarction, conduction abnormalities, heart failure, renal artery stenosis, and claudication. Cardiac involvement found on imaging includes right atrial pseudotumor, right atrioventricular groove infiltration, and pericardial effusions. ECD can involve the large- and medium-sized arteries, often seen as periarterial thickening (commonly coating the aorta) with stenosis/occlusion. Although more cardiovascular ECD cases have begun to be published in the literature, more data are needed on the outcomes of these patients, as well as how cardiovascular manifestations respond to treatment of ECD.

Vasculitis: When to Consider this Diagnosis?

Vasculitis is a diverse group of disorders involving inflammation of the blood vessels. Approaching the diagnosis of vasculitis can be challenging, given the differing clinical presentation and organ manifestations. Often vasculitis is a diagnosis that is considered too late, given the heterogeneous presentation and various mimics. This article aims to provide physicians with a diagnostic approach to vasculitis.

Inferior Vena Cava Thrombus Secondary to Ureteropelvic Junction Obstruction with Severe Hydronephrosis.

BACKGROUND: Benign external compression of the Inferior Vena Cava (IVC) with distal thrombus formation is seldomly described in the medical literature. CASE PRESENTATIONS: We report a case of external IVC compression by a dilated right renal pelvis and hydronephrotic kidney secondary to longstanding Ureteropelvic Junction (UPJ) obstruction found in a 68-year-old male. Management included therapeutic anticoagulation, IVC filter placement, attempted thrombectomy by interventional radiology, and interval repeats imaging. This patient demonstrated complete resolution of the caval thrombus on repeat imaging 2 months following discharge. This case highlights the importance of interdisciplinary team coordination, a crucial component of patient's management and eventual treatment plan. It is reasonable to manage patients with IVC thrombus with anticoagulation alone. CONCLUSION: In the modern era, angiointerventional techniques provide minimally invasive approaches to the management of vascular disorders and minimize morbidity.

A Review of the Past, Present and Future of Cancer-associated Thrombosis Management.

Venous thromboembolism (VTE) can have a significant impact on the management, quality of life and mortality of patients with cancer. VTE occurs in 5-20% of patients with cancer, and malignancy is associated with up to 25% of all VTE. It is the second leading cause of death in ambulatory patients with cancer who are receiving chemotherapy. Increased rates of cancer-associated thrombosis are attributed to improved patient survival, increased awareness, surgery, antineoplastic treatments and the use of central venous access devices. Many factors influence cancer-associated thrombosis risk and are broadly categorized into patient-related, cancer-related and treatment-related risks. Direct-acting oral anticoagulants have shown themselves to be at least as effective in preventing recurrent VTE in patients with cancer with symptomatic and incidental VTE. This has led to a change in treatment paradigms so that direct-acting oral anticoagulants are now considered first-line agents in appropriately selected patients. In this article, we review the prior and recent landmark studies that have directed the treatment of cancer-associated thrombosis, and discuss specific factors that affect management as well as future treatment considerations.

Fibromuscular dysplasia: A comprehensive review on evaluation and management and role for multidisciplinary comprehensive care and patient input model.

Fibromuscular dysplasia is a nonatherosclerotic, under-recognized disorder primarily seen in middle-aged women. It can lead to several complications, such as hypertension, headaches, dissections, aneurysms, myocardial infarctions, and cerebrovascular accidents, to name a few. This article provides a comprehensive review of current literature on epidemiology, etiology, diagnosis, treatment, and long-term surveillance and fibromuscular dysplasia management. In addition, it renders the role of education and prevention for patients living with this condition and family screening. Lastly, it emphasizes the importance of a comprehensive multidisciplinary care model and patient input, given the complexity of this disease and its systemic presence and protean manifestations.