Myxoma Involving the Pulmonary Valve - A Case Report of an Atypical Cardiac Tumor, Treated Using an Unusual Approach.

Background: Atypical myxoma has been reported in various locations in the heart, however, myxoma involving the pulmonary valve is rare. Here we present a case of pulmonic valve myxoma which was resected via a percutaneous approach. Case Report: A 66-year-old female with known metastatic adenocarcinoma of the lung, and chronic obstructive pulmonary disease presented with acute onset shortness of breath for two days. The patient experienced respiratory arrest en-route to the hospital and required intubation. Computed tomography angiography (CTA) of the chest revealed a new 1.4 × 1.6 cm intracardiac mass along the pulmonary valve. Further evaluation with cardiac magnetic resonance imaging revealed it to be a large vascular tumor on the ventricular side of the pulmonary valve, attached with a narrow stalk. Due to high surgical risk, the patient underwent transesophageal echocardiographic guided percutaneous removal of the mass. Pathology confirmed the mass to be a myxoma. Conclusion: Atypical myxoma should be considered in the differential diagnosis of valvular masses. Percutaneous resection of valvular masses may be feasible in high-risk surgical patients. LEARNING POINTS: Pulmonary valve myxoma is a rare condition and the literature on the characteristics and treatment options for pulmonary valve myxoma is limited.Our patient was treated with a minimally invasive treatment approach: removal of a tumor with intra operative transesophageal echocardiographic guidance using AngioVac and Flow Triever catheters.Percutaneous resection of valvular masses may be feasible in high surgical risk patients.

Outcomes in High-Risk Pulmonary Embolism Patients Undergoing FlowTriever Mechanical Thrombectomy or Other Contemporary Therapies: Results From the FLAME Study.

BACKGROUND: Hemodynamically unstable high-risk, or massive, pulmonary embolism (PE) has a reported in-hospital mortality of over 25%. Systemic thrombolysis is the guideline-recommended treatment despite limited evidence. The FLAME study (FlowTriever for Acute Massive PE) was designed to generate evidence for interventional treatments in high-risk PE. METHODS: The FLAME study was a prospective, multicenter, nonrandomized, parallel group, observational study of high-risk PE. Eligible patients were treated with FlowTriever mechanical thrombectomy (FlowTriever Arm) or with other contemporary therapies (Context Arm). The primary end point was an in-hospital composite of all-cause mortality, bailout to an alternate thrombus removal strategy, clinical deterioration, and major bleeding. This was compared in the FlowTriever Arm to a prespecified performance goal derived from a contemporary systematic review and meta-analysis. RESULTS: A total of 53 patients were enrolled in the FlowTriever Arm and 61 in the Context Arm. Context Arm patients were primarily treated with systemic thrombolysis (68.9%) or anticoagulation alone (23.0%). The primary end point was reached in 9/53 (17.0%) FlowTriever Arm patients, significantly lower than the 32.0% performance goal (P<0.01). The primary end point was reached in 39/61 (63.9%) Context Arm patients. In-hospital mortality occurred in 1/53 (1.9%) patients in the FlowTriever Arm and in 18/61 (29.5%) patients in the Context Arm. CONCLUSIONS: Among patients selected for mechanical thrombectomy with the FlowTriever System, a significantly lower associated rate of in-hospital adverse clinical outcomes was observed compared with a prespecified performance goal, primarily driven by low all-cause mortality of 1.9%. REGISTRATION: URL: https://www. CLINICALTRIALS: gov; Unique identifier: NCT04795167.

Surgical and Device Interventions in the Treatment of Chronic Thromboembolic Disease.

Chronic thromboembolic pulmonary disease (CTEPD) is characterized by unresolved clot burden in large pulmonary arteries, obstructive disease in smaller arteries, and increased downstream clot burden. This occurs in the setting of abnormal fibrinolysis or hematological disorders. Up to 50% of patients in some studies are unaware of a self-history of a deep venous thrombosis or pulmonary embolism. Ultimately, they present with symptoms of pulmonary hypertension (PH), which can result in right heart failure (RHF). Pulmonary endarterectomy (PEA) is curative, though many patients have prohibitive surgical risk or surgically inaccessible disease, warranting other interventions such as balloon pulmonary angioplasty (BPA) and medical therapy. Rarely, other treatment options may be implemented. We focus this review on PEA and BPA, with an overview of the history of CTEPD and the evolution of these procedures. We will briefly discuss other treatment modalities.

A Targeted Management Approach to Cardiogenic Shock.

Cardiogenic shock is a clinical syndrome characterized by low cardiac output and sustained tissue hypoperfusion resulting in end-organ dysfunction and death. In-hospital mortality rates range from 50% to 60%. Urgent diagnosis, timely transfer to a tertiary or quaternary medical facility with critical care management capabilities and multidisciplinary shock teams is a must to increase survival. Aggressive, hemodynamically guided medical management with careful monitoring of clinical and hemodynamic parameters with timely use of appropriate mechanical circulatory support devices is often necessary. As treatment options evolve, prospective randomized controlled trials are needed to define best practices that define superior clinical outcomes.

Type I Kounis syndrome variant: A case report and literature review.

Kounis syndrome defined as the occurrence of acute coronary syndrome in the setting of allergic reaction due to mast cells activation and inflammatory mediators release that induces coronary vasospasm, plaque erosion, or even stent thrombosis. A 25-year-old postpartum female with asthma and recurrent episodes of chest pain was admitted with ST-segment elevation myocardial infarction in the setting of coronary artery spasms. The patient was started on calcium channel blockers and nitrite-based medication with no improvement. She was noted to have eosinophilia and initiation of corticosteroid-based regimen lead to resolution of chest pain episodes and normalization of eosinophilia. Kounis syndrome should be considered in young patients with chest pain. Coronary vasodilators are considered as the first-line of treatment. The use of corticosteroids has been described in the literature in severe or refractory cases.

A rare case of Silver-Russell syndrome associated with growth hormone deficiency and urogenital abnormalities.

INTRODUCTION: Silver-Russell syndrome (SRS) is a very rare genetic disorder. This is usually characterized by asymmetry in the size of the two halves or other parts of the body. BACKGROUND: We are presenting a case of SRS with growth hormone (GH) deficiency and urogenital abnormalities. CASE REPORT: A 15-year-old boy a product of non-consanguineous marriage brought with a history of short stature and poor development of secondary sexual characters. There were no adverse perinatal events, but weighed 1500 g (<3(rd)centile) at birth. He had delayed developmental milestones. He has had a poor appetite and feeding difficulties since childhood. On physical examination, he had a broad forehead, triangular facies, and low-set prominent ears. Asymmetry of the face, upper and lower extremities was noted, with hemihypertrophy on the right side. His height was 119 cm (<3(rd)centile) and weight was 18 kg which were low (<3(rd)centile) as per his age. He was biochemically euthyroid and GH stimulation testing with clonidine (0.15 mg/m(2)) showed low GH levels at 30', 60', and 90' were 1.7, 1.6, and 1.1ng/ml, respectively. On micturatingcystourethrogram, grade V complex was noted on the right side. Dimercaptosuccinic acid (DMSA) showed normal functioning kidneys. He was started on recombinant GH with a height velocity of 10 cm/year. CONCLUSION: Urogenital abnormalities are rare but well described anomalies associated with SRS, and all cases have to be screened for them. GH deficiency is not uncommon in SRS, and GH treatment proves to be beneficial.

Recurrent stroke as a presenting feature of acquired partial lipodystrophy.

Acquired partial lipodystrophy (PL) (Barraquer-Simons syndrome) is a rare condition with onset in childhood, and it is characterized by progressive loss of subcutaneous fat in a cephalocaudal fashion. This report describes a case of acquired PL in a 16-year-old girl, who had progressive loss of facial fat since 3 years. Systemic lupus erythematosus (SLE), anticardiolipin antibody, primary hypothyroidism, diabetes, and dyslipidemia may antedate the development of complications such as cerebrovascular stroke and cardiovascular disease. The girl had developed recurrent left hemiparesis, and withdrawn from school due to poor performance.

Delayed manifestation of a mid-esophageal tear with profuse hemorrhage after transesophageal echocardiogram.

Esophageal injury or perforation is a rare but life threatening complication of transesophageal echocardiogram (TEE). Most of the patients manifest symptoms within 24 hours of the procedure and upper esophagus is the most common site of injury. We report a patient who underwent TEE for the evaluation of severe valvular insufficiency and 4 days after the procedure developed an esophageal tear in its mid portion associated with profuse upper gastrointestinal bleeding. Though delayed esophageal perforation in the lower segment has been previously reported after a perioperative TEE, no such report exists to our knowledge, in the setting of a nonsurgical TEE.

Nuclear localization of KLF4 is associated with an aggressive phenotype in early-stage breast cancer.

PURPOSE: The Krüppel-like transcription factor KLF4/GKLF induces both malignant transformation and a slow-growth phenotype in vitro. Although KLF4 expression is increased in most cases of breast cancer, it was unknown whether these cases represent a distinct subtype with a different clinical outcome. EXPERIMENTAL DESIGN: We examined expression of KLF4 by immunostaining 146 cases of human primary infiltrating ductal carcinoma of the breast. Staining patterns were correlated with clinical outcome and with established prognostic factors. RESULTS: Subcellular localization exhibited case-to-case variation. Tumors with high nuclear staining and low cytoplasmic staining were termed type 1. For patients with early-stage disease (i.e., stage I or IIA), type 1 staining was associated with eventual death because of breast cancer (hazard ratio, 2.8; 95% confidence interval, 1.23-6.58; P = 0.011). The association was stronger in patients with early-stage cancer and small primary tumors (i.e., < or =2.0 cm in diameter; hazard ratio, 4.3; 95% confidence interval, 1.75-10.62; P < 0.001). For patients with early-stage disease, multivariate analysis indicated that type 1 staining was independently associated with outcome (adjusted hazard ratio 2.6; 95% confidence interval, 1.10-6.05; P = 0.029). Type 1 staining was also associated with high histological grade (P = 0.032), increased expression of Ki67 (P = 0.016), and reduced expression of BCL2 (P = 0.032). In vitro, KLF4 was localized within the nucleus of transformed RK3E epithelial cells, consistent with a nuclear function of this transcription factor during induction of malignant transformation. CONCLUSIONS: The results suggest that localization of KLF4 in the nucleus of breast cancer cells is a prognostic factor and identify KLF4 as a marker of an aggressive phenotype in early-stage infiltrating ductal carcinoma.