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Right ventricular metastasis of leiomyosarcoma.

ABSTRACT: Metastatic presentation of leiomyosarcoma in the heart is very rare. We present transthoracic echocardiography and combined PET/CT images of a case with a large right ventricular metastasis of leiomyosarcoma. The patient was placed on cytostatic drugs for palliative purposes, but passed away one month later because of an untreatable ventricular tackycardia.

SUBMITTER: Dencker M

PROVIDER: S-EPMC2685770 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Right ventricular outflow tract obstruction due to a leiomyosarcoma.

Project description:A 65-year-old female presenting with worsening dyspnea and notable weight loss were found to have a systolic murmur on physical examination. On workup with computed tomography (CT) angiogram, a solid mass was found extending from the inferior vena cava into the right ventricle. Transesophageal echocardiography demonstrated this mass extension causing right ventricular outflow tract obstruction. After surgical removal, the pathology of the mass was endometrial leiomyosarcoma.

| S-EPMC7559950 | biostudies-literature

Isolated Right Ventricular Metastasis in a Woman with Advanced Hepatocellular Carcinoma after Palliative Therapy.

Project description:Hepatocellular carcinoma (HCC) with cardiac metastasis represents a group of rarity and poor prognosis. Few case reports have described this patient entity, and it remains unclear regarding the optimal treatment and predicted outcome for these patients. In our case, we represented a 67-year-old female patient with presentations of dyspnea and heart murmur, which conducted to the diagnosis of advanced HCC with isolated right ventricular metastasis. Because of multiple comorbidities regarding cardiac and pulmonary systems, she received best supportive care and survived 2 months after initial diagnosis. A systemic literature review of 80 published cases (including our patient) since the 1950s was also performed from PubMed, and the data were gathered from the medical record and literature reviews. In our review, patients with advanced HCC and cardiac metastasis involved the right heart mainly with a predominance of right atrium (53%). Meanwhile the overall 3-month survival rate in our review was 70.7%. In subgroup analysis, the overall 3-month survival was highest (97%) in patients treated with surgery and other therapies, and lowest (27%) in patients with best supportive care. To our knowledge, this is the first comprehensive literature review addressing the epidemiology, optimal treatment, and prognosis for advanced-stage HCC with cardiac metastasis. We suggest that abnormal cardiac murmur in patients with history of HCC should prompt investigation for tumor involvement of the heart. We also emphasize individualized treatment as well as prognostic measurement accordingly.

| S-EPMC6940437 | biostudies-literature

A late systemic and brain metastasis from subcutaneous leiomyosarcoma of the right forearm: a case report and review of the literature.

Project description:BackgroundLeiomyosarcomas are rare malignant tumors which originate from smooth muscle cells and very seldom give rise to intracerebral metastases. Nearly all cases of intracranial metastases stem from leiomyosarcomas of the uterus. We present a 61-year-old Caucasian man who developed multiple intracranial and extracranial metastases from leiomyosarcoma of the right forearm, diagnosed and treated 9 years before the current presentation.Case presentationThe Caucasian patient presented to the emergency department due to a progressive hemiparesis on the left side. Magnetic resonance imaging scans of the neurocranium showed multiple intracerebral masses with perifocal edema. One of these was located in the right parietal lobe, corresponding to the hemiparesis. The patient underwent microsurgical complete resection of the parietal mass and was subsequently subjected to further radiotherapy. Histopathological studies revealed metastasis of the former leiomyosarcoma.ConclusionsLeiomyosarcomas represent a rare entity of mesenchymal tumors. Intracerebral metastasis of these tumors is even less frequent. This case shows the importance of long-term follow-up in patients with leiomyosarcoma.

| S-EPMC7814649 | biostudies-literature

Arrhythmogenic Right Ventricular Cardiomyopathy in an Endurance Athlete Presenting with Ventricular Tachycardia and Normal Right Ventricular Function.

Project description:Arrhythmogenic right ventricular cardiomyopathy, a genetically inherited disease that results in fibrofatty replacement of normal cardiac myocytes, has been associated with sudden cardiac death in athletes. Long-term participation in endurance exercise hastens the development of both the arrhythmic and structural arrhythmogenic right ventricular cardiomyopathy phenotypes. We describe the unusual case of a 34-year-old, symptomatic, female endurance athlete who had arrhythmogenic right ventricular cardiomyopathy in the presence of a structurally normal right ventricle. Clinicians should be aware of this infrequent presentation when evaluating athletic patients who have ventricular arrhythmias and normal findings on cardiac imaging studies.

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Right ventricular failure due to pulmonary banding

Project description:Right ventricular failure was induced thourgh pulmonary banding in 11 pigs. Right ventricular failure was defined as a SRVP >50 mmHg during two hours. After right ventricular failure was induced, half the pigs were treatmed with a Glenn-shunt combined with pulmonary banding for one hour, and the other half served as control group with pulmonary banding only. The aim was to study the change in global gene expression during right ventricular failure due to pulmonary banding, and the effect of volume unloading during pulmonary banding. 11 pigs. Samples at the following time periods: 1) Baseline 2) Right ventricular failure 3) Treatment with modified Glenn-shunt/Control. After Right ventricular failure, pigs were divided into two groups a) Treatment with modified Glenn-shunt or b) Control group

2013-06-11 | E-GEOD-47814 | biostudies-arrayexpress

Expression data from human heart right ventricular wall

Project description:Gene expression in the right ventricle is different in control patients as compared to either idiopathic dilated cardiomyopathy or pulmonary arterial hypertension Two human hearts obtained at autopsy from each of control, pulmonary hypertension, and dilated cardiomyopathy

2015-04-02 | E-GEOD-67492 | biostudies-arrayexpress

Right ventricular strain by MR quantitatively identifies regional dysfunction in patients with arrhythmogenic right ventricular cardiomyopathy.

Project description:BACKGROUND:Analysis of regional wall motion of the right ventricle (RV) is primarily qualitative with large interobserver variation in clinical practice. Thus, the purpose of this study was to use feature tracking to analyze regional wall motion abnormalities in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). METHODS:We enrolled 110 subjects (39 overt ARVC [mutation+/phenotype+] (35.5%), 40 preclinical ARVC [mutation+/phenotype-] (36.3%), and 31 control subjects (28.2%)). Cine steady state free precession cardiac MR was performed with temporal resolution ?40 ms in the horizontal long axis (HLA), axial, and short axis directions. Regional strain was analyzed using feature tracking software and reproducibility was assessed by means of intraclass correlation coefficient. Dunnett's test was used in univariate analysis for comparisons to control subjects; cumulative odds logistic regression was used for minimally and fully adjusted multivariate models. RESULTS:Strain was significantly impaired in overt ARVC compared with control subjects both globally (P < 0.01) and regionally (all segments of HLA view, P < 0.01). In the HLA view, regional reproducibility was excellent within (intraclass correlation coefficient [ICC] = 0.81) and moderate between (ICC = 0.62) observers. Using a threshold of -31% subtricuspid strain in the HLA view, the sensitivity and specificity for overt ARVC were 75.0% and 78.2%, respectively. In multivariable analysis involving all three groups, subtricuspid strain less than -31% (beta = 1.38; P = 0.014) and RV end diastolic volume index (beta = 0.06; P = 0.001) were significant predictors of disease presence. CONCLUSION:RV strain can be reproducibly assessed with MR feature tracking, and regional strain is abnormal in overt ARVC compared with control subjects.

| S-EPMC4837004 | biostudies-literature

"Double Ball Valve Mechanism Obstructing Both Right Ventricular Inflow and Outflow": Atypical Presentation of Right Atrial Myxoma Presenting as Right Ventricular Mass.

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| S-EPMC5755196 | biostudies-literature

Dual Endothelin Receptor Blockade Abrogates Right Ventricular Remodeling and Biventricular Fibrosis in Isolated Elevated Right Ventricular Afterload.

Project description:BackgroundPulmonary arterial hypertension is usually fatal due to right ventricular failure and is frequently associated with co-existing left ventricular dysfunction. Endothelin-1 is a powerful pro-fibrotic mediator and vasoconstrictor that is elevated in pulmonary arterial hypertension. Endothelin receptor blockers are commonly used as pulmonary vasodilators, however their effect on biventricular injury, remodeling and function, despite elevated isolated right ventricular afterload is unknown.MethodsElevated right ventricular afterload was induced by progressive pulmonary artery banding. Seven rabbits underwent pulmonary artery banding without macitentan; 13 received pulmonary artery banding + macitentan; and 5 did not undergo inflation of the pulmonary artery band (sham-operated controls).ResultsRight and left ventricular collagen content was increased with pulmonary artery banding compared to sham-operated controls and ameliorated by macitentan. Right ventricular fibrosis signaling (connective tissue growth factor and endothelin-1 protein levels); extra-cellular matrix remodeling (matrix-metalloproteinases 2 and 9), apoptosis and apoptosis-related peptides (caspases 3 and 8) were increased with pulmonary artery banding compared with sham-operated controls and decreased with macitentan.ConclusionIsolated right ventricular afterload causes biventricular fibrosis, right ventricular apoptosis and extra cellular matrix remodeling, mediated by up-regulation of endothelin-1 and connective tissue growth factor signaling. These pathological changes are ameliorated by dual endothelin receptor blockade despite persistent elevated right ventricular afterload.

| S-EPMC4713098 | biostudies-literature

Right ventricular dyssynchrony: from load-independent right ventricular function to wall stress in severe pulmonary arterial hypertension.

Project description:Right ventricular (RV) dyssynchrony has been related to outcome in pulmonary arterial hypertension. Prospectively, we performed echocardiography with measurement of right ventricular dyssynchrony and pressure-volume loop catheterization in 27 pulmonary arterial hypertension patients. Afterload and diastolic function emerged as determinates of wall stress, which results in dyssynchrony.

| S-EPMC7235672 | biostudies-literature

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