scholarly journals Angiopoietin-2 predicts morbidity in adults with Fontan physiology

2019 ◽  
Vol 9 (1) ◽  
Author(s):  
Aditya S. Shirali ◽  
Gentian Lluri ◽  
Pierre J. Guihard ◽  
Miles B. Conrad ◽  
Helen Kim ◽  
...  

AbstractMorbidity in patients with single-ventricle Fontan circulation is common and includes arrhythmias, edema, and pulmonary arteriovenous malformations (PAVM) among others. We sought to identify biomarkers that may predict such complications. Twenty-five patients with Fontan physiology and 12 control patients with atrial septal defects (ASD) that underwent cardiac catheterization were included. Plasma was collected from the hepatic vein and superior vena cava and underwent protein profiling for a panel of 20 analytes involved in angiogenesis and endothelial dysfunction. Ten (40%) of Fontan patients had evidence of PAVM, eighteen (72%) had a history of arrhythmia, and five (20%) were actively in arrhythmia or had a recent arrhythmia. Angiopoietin-2 (Ang-2) was higher in Fontan patients (8,875.4 ± 3,336.9 pg/mL) versus the ASD group (1,663.6 ± 587.3 pg/mL, p < 0.0001). Ang-2 was higher in Fontan patients with active or recent arrhythmia (11,396.0 ± 3,457.7 vs 8,118.2 ± 2,795.1 pg/mL, p < 0.05). A threshold of 8,500 pg/mL gives Ang-2 a negative predictive value of 100% and positive predictive value of 42% in diagnosing recent arrhythmia. Ang-2 is elevated among adults with Fontan physiology. Ang-2 level is associated with active or recent arrhythmia, but was not found to be associated with PAVM.

2020 ◽  
Author(s):  
Nan Liu ◽  
Ying Xing ◽  
Chen Wang

AbstractPulmonary arterial hypertension (PAH) is a vascular disorder associated with significant morbidity and mortality. The pathophysiology of PAH remains controversial, but the only currently available therapies for PAH are pharmacological pulmonary artery vasodilation, decreasing right ventricular (RV) afterload, and relieving symptoms. By now, there is no therapy being able to minimize vascular remodeling processes and thus to reverse or delay the natural history of the disease. It has been generally thought that reduction of RV preload was detrimental, which deteriorated the systemic hemodynamics. In the present study, however, we repetitively and briefly occluded (RBO) both superior vena cava and inferior vena cava by ligation (occlusion for less than 5 seconds then re-open for 30 seconds and repeated 5 cycles as one sequence, 1 sequence every 6 hours) to intermittently restrict RV preload, for continuous 24 hours, total 5 sequences, in the Sugen 5416 (VEGF receptor blocker) and hypoxia induced PAH rat models and we found this strategy was beneficial for lowering pulmonary vascular resistance (PVR).


2013 ◽  
Vol 2013 ◽  
pp. 1-3 ◽  
Author(s):  
Imran Shaikh ◽  
Kenneth Berg ◽  
Nicholas Kman

Superior vena cava syndrome has historically been associated with malignancy. With the increasing use of indwelling central lines, catheters, and pacemakers in the past decade, there have been an increasing number of cases associated with thrombosis rather than by direct external compression. Patients presenting to the ED with an acute process of SVC syndrome need to be assessed in a timely fashion. Computed tomography angiography (CTA) or magnetic resonance angiogram (MRA) are superb modalities for diagnosis and can quickly be used in the ED. Treatment is oriented towards the underlying cause of the syndrome. In cases of thrombogenic catheter-associated SVC syndrome, anticoagulation is the mainstay of treatment. We present a case report and discussion of a 56-year-old male with a history of metastatic colorectal cancer and an indwelling central venous port with acute signs and symptoms of superior vena cava syndrome.


2004 ◽  
Vol 61 (2) ◽  
Author(s):  
K. Malagari ◽  
S. Papiris

Idiopathic fibrosing mediastinitis is a rare entity involving more severely the more compliant structures within the mediastinum. In this report a rare case of simultaneous involvement of both the superior vena cava (SVC) and pulmonary veins is described in a 16 – year old male with progressive dyspnea on exertion, cough and a three months’ history of blood – tinged sputum. Physical examination and imaging studies revealed signs of pulmonary venous hypertension (PVH) and SVC stenosis. Fibrosing mediastinitis was confirmed by multiple biopsy samples.


2016 ◽  
Vol 8 (2) ◽  
pp. 215-219 ◽  
Author(s):  
Matthew C. Schwartz ◽  
David Nykanen ◽  
William DeCampli ◽  
Kamal Pourmoghadam

Staged palliation to achieve a total cavopulmonary connection is a common treatment strategy in patients with single ventricle congenital heart disease. Patients with bilateral superior caval veins (bilateral SVC) often require the creation of bilateral superior cavopulmonary connections as part of the staged palliation, and these patients are at increased risk of morbidity. We describe a novel technique used in two patients with bilateral SVC and very small (1-2 mm) bridging vein that encouraged bridging vein growth and facilitated creation of a unilateral superior cavopulmonary connection.


2019 ◽  
Vol 9 (1) ◽  
pp. 268-270
Author(s):  
Rosnelifaizur R*,Aizat Sabri I, Krishna K,Lenny SS,Azim I, H Harunarashid

We reported a case of 58 years old gentleman who known case of end stage renal failure and had history of Right IJC cannulation of venous access on 2012, presented with recurrent shortness of breath, chesty cough and intermittent fever. Otherwise he got no hemoptysis, no recent contact with PTB patients and no joint pain. The same presentation occurred last month with a pleural tapping was done and claimed it was a milky content. No further investigation was done at that moment. This current presentation noted a dullness in percussion up to midzone of right lung and reduce air entry on auscultation as well. The pigtail catheter was inserted over the right pleural space and it was confirmed as a chylothorax with a present of cholesterol in a pleural fluid analysis. Computed tomography of the thorax showed complete occlusion of the superior vena cava with an established collateral circulation. Lymphangiogram revealed lipiodol seen opacified lymph node and lymphatic vessels until the level of T3 on the right and T5 on the left. There was no obvious lipiodol opacification seen at the region of the right thorax. Effusion was improved after the instillation of fibrinolytic agent and the the chest radiograph shows improvement.


2020 ◽  
Author(s):  
Teresa L. Capasso ◽  
Sara M. Trucco ◽  
Morgan Hindes ◽  
Tristin Schwartze ◽  
Jamie L. Bloch ◽  
...  

AbstractIn children with single ventricle physiology, the Glenn procedure is performed to redirect venous return from the superior vena cava directly to the pulmonary arteries and route venous return from the inferior vena cava exclusively to the systemic circulation. Although this surgery successfully palliates the hemodynamic stress experienced by the single ventricle, patients frequently develop pulmonary arteriovenous malformations (PAVMs). Interestingly, PAVMs may regress upon rerouting of hepatic venous effluent to the pulmonary vasculature, suggesting the presence of a circulating “hepatic factor” that is required to prevent PAVMs. Here, we test the hypothesis that hepatic factor is bone morphogenetic protein 9 (BMP9) and/or BMP10. These circulating ligands are produced by the liver and activate endothelial endoglin (ENG)/ALK1 signaling, and mutations in ENG and ALK1 cause hereditary hemorrhagic telangiectasia, a genetic disease associated with AVM development. However, we found no within-subject variation in BMP9, BMP10, or BMP9/10 plasma concentrations when sampled from five cardiovascular sites, failing to support the idea that the Glenn would limit access of these ligands to the lung vasculature. Unexpectedly, however, we found a significant decrease in all three ligand concentrations in Glenn cases versus controls. Our findings suggest that BMP9/BMP10/ENG/ALK1 signaling may be decreased in the Glenn vasculature but fail to implicate these ligands as hepatic factor.


Author(s):  
Matthew Borchart ◽  
◽  
Daniel Yu ◽  
Indrajit Nandi

No abstract available. Article truncated after 150 words. History A 74-year- old man with a history of diastolic heart failure, chronic kidney disease (CKD), and chronic lymphocytic leukemia (CLL) presented with a complaint of dyspnea. He has had several hospitalizations in the last year for heart failure exacerbation and his home bumetanide was recently increased from twice to three times daily due to persistently increasing weight. His CLL was diagnosed two years prior and treatment was stopped three months ago due to side effects. In the emergency department he reported three weeks of worsening dyspnea especially when lying flat, as well as increased swelling in his legs, abdomen, arms, and face. His weight was up to 277lbs from 238lbs the month before. His diuretics were transitioned to IV, but over the next few days he remained clinically volume overloaded. A noncontrast chest CT was obtained to help evaluate his ongoing respiratory distress (Figure 1). It demonstrated innumerable lymph …


2021 ◽  
Vol 16 (4) ◽  
pp. 383-392
Author(s):  
Yajuan Zhang ◽  
Jun Yan ◽  
Qiang Wang ◽  
Shoujun Li ◽  
Jing Sun ◽  
...  

2016 ◽  
Vol 2016 ◽  
pp. 1-4
Author(s):  
Daniel Jeong ◽  
Kenneth L. Gage ◽  
Claudia G. Berman ◽  
Jaime L. Montilla-Soler

A 53-year-old female with a history of metastatic left arm melanoma presented for F(18) fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) which showed a moderately FDG avid focus at her port catheter tip near the cavoatrial junction. Although catheter tip related FDG avidity has previously been suggested to be bland thrombus or infection, melanoma can metastasize to unusual locations including the superior vena cava. In addition, the patient had an elevated risk of anticoagulation due to a history of hemorrhagic brain metastases. Therefore, confirmatory cardiac magnetic resonance (CMR) was obtained and findings were consistent with bland catheter-related thrombus.


2012 ◽  
Vol 2012 ◽  
pp. 1-3
Author(s):  
Pelin Oktayoglu ◽  
Figen Cevik ◽  
Mehmet Tahtasiz ◽  
Serda Em ◽  
Mehtap Bozkurt ◽  
...  

We describe a 31-years-old female patient with severe pain in both knees who had been diagnosed as Behcet’s disease (BD) for 12 years. She had had a history of complications due to BD including superior vena cava thrombosis, pulmonary thromboembolism, uveitis, and erythema nodosum and has reported the administration of corticosteroid therapy irregularly. After radiologic evaluation, she has been diagnosed with bone infarction of both left and right knee with the existance of lupus anticoagulants (LA) positivity. Severe joint pain without the evidence of arthritis must alert the clinician to the possibility of bone necrosis of the extremity, although those may rarely occur bilateral in BD.


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