A Rare Case of Cavernous Hemangioma of the Mitral Valve Presenting As Multifocal Embolic Brain Infarcts.

Primary cardiac tumors (PCTs) are rare and represent a heterogeneous group of tumors, potentially arising from various parts of the heart. The majority of these tumors are benign (90%), with myxoma being the most common subtype. Cardiac hemangiomas are rare vascular tumors that constitute 1-2% of all benign heart neoplasms. We present a rare case of a 79-year-old woman presented with multifocal embolic brain infarcts secondary to cavernous hemangioma of the mitral valve (MV). Cavernous hemangioma was successfully resected with follow-up imaging at six months, demonstrating no regrowth. There are no pathognomonic signs or findings to suggest cavernous hemangioma of the MV on clinical examination or imaging studies. Surgical resection and histopathologic analysis remain the gold standard for diagnosis and treatment, respectively. Following complete resection, the prognosis is generally favorable with a low recurrence rate, but periodic echocardiography is recommended to detect any potential recurrence.

Early Thrombosis of Splenic Artery Stent Graft.

Splenic artery aneurysms (SAAs) are among the most common visceral aneurysms behind aortic and iliac arteries. Certain factors like aneurysm size (especially giant SAAs), hypertension (HTN), symptomatology, pregnancy, portal hypertension (pHTN), and liver transplantation increase the risk of rupture. Most often found incidentally, but when symptomatic, can present with nonspecific symptoms like nausea, vomiting, anorexia, and epigastric/left upper quadrant pain. Diagnosis can be accomplished with different modalities of CT or MRI and digital subtraction angiography (DSA) being the gold standard for diagnosis. Treatment is usually preferred for aneurysms >2 cm, symptomatic cases, and pregnant women. Various surgical/interventional procedures can be performed and selected based on the patient's sex, age, location of the aneurysm, size of the aneurysm, and presenting complaints/complications. Endovascular techniques with or without stent-graft placement are being used more, given the minimally invasive nature of these procedures. No clear guidelines exist on initiation of dual antiplatelet therapy (DAPT), but based on guidelines from visceral arterial stenting (especially iliac arteries and renal arteries), multiple case reports/series on SAAs, we highly recommend the usage of DAPT pre- and post-stent-graft placement to improve patency.

Is the Renal Resistive Index a Marker for Revascularization in Atherosclerotic Renal Artery Stenosis?

Renal artery stenosis (RAS) is one of the major causes of resistant/malignant hypertension. It can be described as atherosclerotic or non-atherosclerotic. Atherosclerotic RAS comprises almost 90% of all RAS cases and is a prevalent disease of the elderly. Multiple risk factors contribute to atherosclerosis development, which leads to the release of renin and aldosterone, causing resistant/malignant hypertension. Early recognition is prudent but challenging as there are no early clinical signs. We believe that renal resistive index with supportive clinical, laboratory, and imaging modalities can help select revascularization patients.

Metastatic renal cell carcinoma complicated by right atrial thrombus.

Metastasis of renal cell carcinoma to the heart is a rare event. Herein we present a case of renal cell carcinoma presenting with progressive fatigue, abdominal pain, and weight loss. Imaging studies revealed complex renal mass with extension to right atrium and histopathology confirmed the metastatic renal cell carcinoma.

Leptomeningeal Carcinomatosis in Epithelial Ovarian Cancer: A Diagnostic Challenge.

Leptomeningeal carcinomatosis (LCM), also known as neoplastic meningitis, is a rare entity. It is generally seen in solid tumors. Ovarian cancers can infrequently cause LCM. The clinical presentation is variable. Diagnosis is made by a lumbar puncture that shows malignant cells in the cerebrospinal fluid (CSF) and usually correlates with imaging findings. Given the low individual sensitivities of lumbar puncture (55%) and magnetic resonance imaging (70%), it is recommended to combine both modalities for optimal diagnostic results. Treatment options vary depending on the type of primary carcinoma, however, the prognosis is guarded. We report a case of LCM in a patient with stage IV epithelial ovarian cancer in remission, which became a diagnostic challenge due to a lack of imaging findings.

Lung Adenocarcinoma Presenting as Malignant Pericardial Effusion/Tamponade.

Lung cancers are the most common primary tumors that involve the pericardium with a prevalence of up to 50%. Usually, pericardial involvement goes undetected with almost 10%-12% found among all cancer related autopsies. Rarely pericardial effusions can be the initial site of metastasis and initial manifestation of a primary tumor. In our case, we report a 57-year-old female presenting with cardiac tamponade and subsequent testing was done which revealed lung adenocarcinoma. Malignant pericardial effusions are often silent, but certain times can present with symptoms of shortness of breath, chest pain, cough, arrhythmias, and rarely as pericardial tamponade. A high index of suspicion is required when a patient presents with tamponade to diagnose malignancy. Emergent pericardiocentesis may be warranted depending on the clinical presentation but quite often, patients tend to have a poor prognosis despite therapy given the extent of disease.

A Rare Case of RYR2 Mutation Causing Sudden Cardiac Arrest Due to Catecholaminergic Polymorphic Ventricular Tachycardia.

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a complex disorder that can induce lethal ventricular arrhythmias, secondary to activation of the sympathetic nervous system. This disease is often diagnosed in childhood but can also manifest in adulthood (the early 40s). Gene mutations such as CALM1, RYR2 (ryanodine receptor-2), CASQ2, and TRDN have been identified as common causes of CPVT. Those affected can present with episodes of syncope, sudden cardiac arrest, or sudden cardiac death due to either fast polymorphic ventricular tachycardia (VT) or bidirectional VT. Diagnosing and managing CPVT can often be challenging as patients are often asymptomatic and may present after a sudden cardiac arrest. Exercise stress testing and genetic testing play a pivotal role in the workup of CPVT. Avoidance of strenuous activities and pharmacological therapy with beta-blockers are the mainstays of treatment. Here, we report a case of CPVT in a patient with RYR2 gene mutation, causing sudden cardiac arrest.

Left Ventricular Assist Device Therapy and Acute Pancreatitis: Higher Incidence and Worse Clinical Outcomes.

OBJECTIVE: The aim of the study was to compare incidence and outcomes of acute pancreatitis among advanced heart failure therapies. METHODS: Two retrospective cohorts are as follows: A, patients with heart failure presenting to our hospitals and B, the US National Inpatient Sample. Three groups were compared: left ventricular assist device (LVAD) recipients, transplant recipients, and controls who did not qualify for advanced therapies. Primary outcomes were pancreatitis incidence and mortality. Secondary outcomes included kidney failure, multiorgan failure, shock, and health care utilization. RESULTS: Cohort A included 1344 heart failure patients, and cohort B included 677,905 patients with acute pancreatitis. In cohort A, annual pancreatitis incidence was 6.7 cases per 1000 LVAD recipients, 4.1 per 1000 LVAD bridge-to-transplant, 2.3 per 1000 transplant recipients, and 3.2 per 1000 heart failure controls (P = 0.03). Combined, the incidence was 5.6 per 1000 LVAD users and 2.7 in 1000 non-LVAD users (relative risk, 2.1; P = 0.009). In cohort B, increased mortality was seen in LVAD users, but not in transplant recipients. Left ventricular assist device patients had higher odds of kidney failure, multiorgan failure, shock, and intensive care. CONCLUSIONS: Patients with LVAD have double risk of pancreatitis, worse clinical outcomes, and increased healthcare utilization. Studies elucidating the mechanisms behind pancreatic injury in advanced heart failure are suggested.

Aberrant lipid metabolism as a therapeutic target in liver cancer.

INTRODUCTION: Hepatocellular carcinoma (HCC) is one of the most common and lethal cancers. Progress has been made in treatment of HCC; however, improved outcomes are much needed. The increased metabolic needs of cancer cells underscore the importance of metabolic pathways in cancer cell survival. Lipid metabolism has a role in HCC development; aberrant overexpression of several key enzymes is seen in many solid human tumors. Areas covered: We discuss aberrant lipid metabolism and the promise of multiple targets, in particular related to HCC treatment. We searched PubMed and clinicaltrials.gov for published and unpublished studies from 2000 to 2019. These terms were used: lipids, fatty acid metabolism, lipid metabolism, liver cancer, HCC, de novo fatty acid synthesis, ATP citrate lyase, stearoyl CoA denaturase, fatty acid synthase, acetyl coenzyme A carboxylase, CD147, KLF4, monoglyceride lipase, AMP activated protein kinase. Expert opinion: The importance of dysregulation of fatty acid synthesis in cancer is a growing area of research. HCC demonstrates significant alteration in lipid metabolism, representing great potential as a target for novel therapeutics. Various agents have demonstrated promising anti-neoplastic activity. This strategy deserves further development for improved outcomes.

A phase I study of the safety and tolerability of VLX600, an Iron Chelator, in patients with refractory advanced solid tumors.

Introduction VLX600 is a novel iron chelator designed to interfere with intracellular iron metabolism, leading to inhibition of mitochondrial respiration and bioenergetic catastrophe and resultant tumor cell death. Methods We conducted a multicenter, phase 1, dose escalation study to determine the safety and adverse event profile and the maximum tolerated dose and recommended phase 2 dose of VLX600. Other endpoints included pharmacokinetics, and preliminary evidence of anti-cancer efficacy as assessed according to RECIST 1.1 criteria. VLX600 was administered intravenously on days 1, 8, and 15 of each 28-day treatment cycle. Results Nineteen patients were enrolled, and seventeen received at least one dose of VLX600. Dose increments were reduced to 50% after dose level 3 (40 mg) due to the occurrence of a grade 3 pulmonary embolism. The study was then closed early due to slow recruitment. No maximum tolerated dose (MTD) nor RP2D had been identified at the time of study closure. Overall, the drug was well tolerated and no DLTs were observed. Fourteen patients experienced drug-related adverse events of any grade. The most frequently reported drug-related AEs were fatigue, nausea, constipation, vomiting, increased alkaline phosphatase, anemia, and decreased appetite. No formal efficacy or survival analyses were performed. No objective responses were observed, though six patients (32%) had stable disease as best response. Conclusion VLX600 was reasonably well tolerated and, together with preclinical data, there is support for further efforts to explore its activity as single agent and in combination with drugs or radiation.