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pulmonary vascular disease

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https://www.readbyqxmd.com/read/29903860/a-potential-therapeutic-role-for-angiotensin-converting-enzyme-2-in-human-pulmonary-arterial-hypertension
#1
Anna R Hemnes, Anandharajan Rathinasabapathy, Eric A Austin, Evan L Brittain, Erica J Carrier, Xinping Chen, Joshua P Fessel, Candice D Fike, Peter Fong, Niki Fortune, Robert E Gerszten, Jennifer A Johnson, Mark Kaplowitz, John H Newman, Robert Piana, Meredith E Pugh, Todd W Rice, Ivan M Robbins, Lisa Wheeler, Chang Yu, James E Loyd, James West
Rationale: Pulmonary arterial hypertension (PAH) is a deadly disease with no cure. Alternate conversion of Angiotensin II to Angiotensin-(1-7) (Ang-(1-7)) by angiotensin converting enzyme 2 (ACE2) resulting in Mas receptor (Mas1) activation improves rodent models of PAH. Effects of rhACE2 in human PAH are unknown. Objectives: To determine the effects of rhACE2 in PAH. Methods: We defined the molecular effects of Mas1 activation using porcine pulmonary arteries, measured AngII/Ang-(1-7) levels in human PAH and conducted a phase IIa open-label pilot study of a single infusion of rhACE2 (GSK2586881, 0...
June 14, 2018: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/29901088/fasudil-preserves-lung-endothelial-function-and-reduces-pulmonary-vascular-remodeling-in-a-rat-model-of-end%C3%A2-stage-pulmonary-hypertension-with-left-heart-disease
#2
Rulin Zhuang, Jinfu Wu, Fang Lin, Lu Han, Xiaoting Liang, Qingshu Meng, Yuyu Jiang, Zhulin Wang, Aixue Yue, Yuying Gu, Huimin Fan, Xiaohui Zhou, Zhongmin Liu
Pulmonary hypertension (PH) due to left heart disease (LHD) is a common condition associated with significant morbidity. It contributes to the elevation of pulmonary vascular resistance and mean pulmonary pressure, eventually leading to heart failure and even mortality. The present study aimed to explore the potential efficacy of late and long‑term treatment with a Rho‑kinase (ROCK) signaling inhibitor, namely fasudil, in a rat model of end‑stage PH‑LHD. The PH‑LHD model was established by supracoronary aortic banding, and the effect of fasudil treatment on the progression of PH‑LHD was monitored...
June 13, 2018: International Journal of Molecular Medicine
https://www.readbyqxmd.com/read/29899023/nedd9-targets-col3a1-to-promote-endothelial-fibrosis-and-pulmonary-arterial-hypertension
#3
Andriy O Samokhin, Thomas Stephens, Bradley M Wertheim, Rui-Sheng Wang, Sara O Vargas, Lai-Ming Yung, Minwei Cao, Marcel Brown, Elena Arons, Paul B Dieffenbach, Jason G Fewell, Majed Matar, Frederick P Bowman, Kathleen J Haley, George A Alba, Stefano M Marino, Rahul Kumar, Ivan O Rosas, Aaron B Waxman, William M Oldham, Dinesh Khanna, Brian B Graham, Sachiko Seo, Vadim N Gladyshev, Paul B Yu, Laura E Fredenburgh, Joseph Loscalzo, Jane A Leopold, Bradley A Maron
Germline mutations involving small mothers against decapentaplegic-transforming growth factor-β (SMAD-TGF-β) signaling are an important but rare cause of pulmonary arterial hypertension (PAH), which is a disease characterized, in part, by vascular fibrosis and hyperaldosteronism (ALDO). We developed and analyzed a fibrosis protein-protein network (fibrosome) in silico, which predicted that the SMAD3 target neural precursor cell expressed developmentally down-regulated 9 (NEDD9) is a critical ALDO-regulated node underpinning pathogenic vascular fibrosis...
June 13, 2018: Science Translational Medicine
https://www.readbyqxmd.com/read/29897297/abstracts-from-the-11th-international-conference-on-neonatal-and-childhood-pulmonary-vascular-disease-2018
#4
(no author information available yet)
No abstract text is available yet for this article.
July 2018: Pulmonary Circulation
https://www.readbyqxmd.com/read/29896859/right-ventricular-base-apex-ratio-in-the-assessment-of-pediatric-pulmonary-arterial-hypertension-results-from-the-european-pediatric-pulmonary-vascular-disease-network
#5
Martin Koestenberger, Alexander Avian, Andreas Gamillscheg, Hannes Sallmon, Gernot Grangl, Ante Burmas, Sabrina Schweintzger, Stefan Kurath-Koller, Gerhard Cvirn, Georg Hansmann
BACKGROUND: Echocardiographic determination of the RV end-systolic base/apex (RVESb/a) ratio was proposed to be of clinical value for the assessment of pulmonary arterial hypertension (PAH) in adults. HYPOTHESIS: We hypothesized that the RVES b/a ratio will be affected in children with PAH and aimed to correlate the RVES b/a ratio with conventionally used echocardiographic and hemodynamic variables, and with NYHA functional class (FC). METHODS: We first determined normal pediatric values for the RVES b/a ratio in 157 healthy children (68 male; age range: 0...
June 12, 2018: Clinical Cardiology
https://www.readbyqxmd.com/read/29895486/left-ventricular-assist-devices-in-pulmonary-hypertension-group-2-with-significantly-elevated-pulmonary-vascular-resistance-a-bridge-to-cure
#6
Ahmed M Selim, Lalit Wadhwani, Adam Burdorf, Eugenia Raichlin, Brian Lowes, Ronald Zolty
BACKGROUND: Pulmonary hypertension secondary to left heart disease (WHO Group 2) is a known risk factor in patients with heart failure. The favourable effect of left ventricular assist devices (LVAD) on pulmonary hypertension has been demonstrated before, although this effect has not been well-studied in advanced pulmonary arterial bed disease with a significant elevation in pulmonary vascular resistance. METHODS: We reviewed the records of 258 LVAD patients in our institution...
May 18, 2018: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/29893303/primary-pulmonary-synovial-sarcoma-a-reappraisal
#7
REVIEW
Manoj Kumar Panigrahi, Gourahari Pradhan, Nibedita Sahoo, Pritinanda Mishra, Susama Patra, Prasanta Raghab Mohapatra
Synovial sarcoma (SS) is a malignant mesenchymal tumor with variable epithelial differentiation that affects mostly young adults and can arise at any anatomic site. Primary intrathoracic SS is very rare accounting for <0.5% of all lung tumors. Most commonly, it arises from the lung followed by pleura and mediastinum. Primary pulmonary SS (PPSS) affects both sexes equally with no preference for any hemithorax. The morphology, immunostaining properties, cytogenetic features, and management strategy of PPSS are similar to that of soft tissue SS...
April 2018: Journal of Cancer Research and Therapeutics
https://www.readbyqxmd.com/read/29891245/a-single-center-experience-of-1500-lung-transplant-patients
#8
Keki R Balsara, Alexander S Krupnick, Jennifer M Bell, Ali Khiabani, Masina Scavuzzo, Ramsey Hachem, Elbert Trulock, Chad Witt, Derek E Byers, Roger Yusen, Bryan Meyers, Benjamin Kozower, G Alexander Patterson, Varun Puri, Daniel Kreisel
OBJECTIVE: Over the past 30 years, lung transplantation has emerged as the definitive treatment for end-stage lung disease. In 2005, the lung allocation score (LAS) was introduced to allocate organs according to disease severity. The number of transplants performed annually in the United States continues to increase as centers have become more comfortable expanding donor and recipient criteria and have become more facile with the perioperative and long-term management of these patients...
April 4, 2018: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29886624/-establishment-and-evaluation-of-a-new-method-for-determining-hemodynamics-of-pulmonary-hypertension-rats
#9
Q Y Zheng, M D Kuang, Y Li, X T Wu, J Y Huang, C T Zhang, H W Liu, W J Lu, J Wang, Y Q Chen
Objective: By evaluating the hemodynamic parameters such as cardiac output (CO), right ventricular pressure (RVP), pulmonary artery pressure (PAP) and total pulmonary resistance index (TPRI) in pulmonary hypertension rat model, we established a more comprehensive hemodynamic evaluation system, which objectively evaluated the severity of disease and exercise tolerance in rats with pulmonary hypertension. Methods: SD rats were randomly divided into a control group and a model group with 5 rats in each group. The model group was intraperitoneally injected with SU5416 (20 mg/kg) and placed in an oxygen chamber at a 10% oxygen concentration for 21 days and then placed in a normoxic environment for 14 days...
June 12, 2018: Chinese Journal of Tuberculosis and Respiratory Diseases
https://www.readbyqxmd.com/read/29886623/-pulmonary-vascular-remodeling-is-prior-to-the-increase-in-pulmonary-hypertension-of-chronic-obstructive-pulmonary-disease
#10
X T Guo, Y C Fan, B W Hao, X Cao, L Zhu, J X He, X Sun, J Chen
Objective: To explore the remodeling of pulmonary arterioles in chronic obstructive pulmonary disease (COPD), and its effect on hemorheology of proximal pulmonary arteries, right ventricular structure and function , and the potential mechanisms. Method: A total of 34 patients undergoing surgical treatment for lung tumors admitted to the General Hospital of Ningxia Medical University were included in the study. According to the preoperative lung function, there were 15 patients with COPD complicated with lung tumor (COPD group) and 19 patients with normal pulmonary function with lung tumor (control group)...
June 12, 2018: Chinese Journal of Tuberculosis and Respiratory Diseases
https://www.readbyqxmd.com/read/29886475/-pulmonary-veno-occlusive-disease-pulmonary-capillary-hemangiomatosis-a-case-report-and-literature-review
#11
Xianliang Xiong, Tengteng Zhu, Jiang Li
Pulmonary veno-occlusive disease (PVOD)/pulmonary capillary hemangiomatosis (PCH) is a rare form of pulmonary vascular disease that causes pulmonary arterial hypertension. The diagnosis of PVOD/PCH can be established by the combination of clinical features, physical examination, radiological findings, lung function, bronchoscopy and other resources. There is no established medical therapy for PVOD/PCH, and the only curative therapy for PVOD/PCH is lung transplantation. A girl with PVOD/PCH was diagnosed in the Second Xiangya Hospital...
May 28, 2018: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
https://www.readbyqxmd.com/read/29886070/inhalation-of-repurposed-drugs-to-treat-pulmonary-hypertension
#12
Tobias Gessler
Pulmonary arterial hypertension (PAH) is a rare, but severe and life-threatening disease characterized by vasoconstriction and remodeling of the pulmonary arterioles, leading to progressive increase in pulmonary vascular resistance and ultimately to right-heart failure. In the last two decades, significant progress in treatment of PAH has been made, with currently 12 drugs approved for targeted therapy. Among these, the stable prostacyclin analogues iloprost and treprostinil have been repurposed for inhalation...
June 7, 2018: Advanced Drug Delivery Reviews
https://www.readbyqxmd.com/read/29885631/the-role-of-platelets-in-the-development-and-progression-of-pulmonary-arterial-hypertension
#13
REVIEW
Remigiusz Kazimierczyk, Karol Kamiński
Pulmonary arterial hypertension is a multifactorial disease characterized by vasoconstriction, vascular remodeling, inflammation and thrombosis. Although an increasing number of research confirmed that pulmonary artery endothelial cells, pulmonary artery smooth muscle cells as well as platelets have a role in the pulmonary arterial hypertension pathogenesis, it is still unclear what integrates these factors. In this paper, we review the evidence that platelets through releasing a large variety of chemokines could actively impact the pulmonary arterial hypertension pathogenesis and development...
June 6, 2018: Advances in Medical Sciences
https://www.readbyqxmd.com/read/29884912/sex-specific-contributions-of-endothelin-to-hypertension
#14
REVIEW
Eman Y Gohar, David M Pollock
PURPOSE OF REVIEW: Men and women differ in the prevalence, pathophysiology and control rate of hypertension in an age-dependent manner. The renal endothelin system plays a central role in sex differences in blood pressure regulation by control of sodium excretion and vascular function. Improving our understanding of the sex differences in the endothelin system, especially in regard to blood pressure regulation and sodium homeostasis, will fill a significant gap in our knowledge and may identify sex-specific therapeutic targets for management of hypertension...
June 8, 2018: Current Hypertension Reports
https://www.readbyqxmd.com/read/29881726/using-omics-to-understand-and-treat-pulmonary-vascular-disease
#15
REVIEW
Anna R Hemnes
Pulmonary arterial hypertension (PAH) is a devastating disease for which there is no cure. Presently this condition is differentiated from other diseases of the pulmonary vasculature by a practitioner's history, physical examination, and clinical studies with clinical markers of disease severity primarily guiding therapeutic choices. New technologies such as next generation DNA sequencing, high throughput RNA sequencing, metabolomics and proteomics have greatly enhanced the amount of data that can be studied efficiently in patients with PAH and other rare diseases...
2018: Frontiers in Medicine
https://www.readbyqxmd.com/read/29880538/restrictive-lung-defects-parenchymal-chest-wall-and-neuromuscular
#16
Anne-Marie Shanks, Sujal R Desai, Alexandra Rice, Stephen R Thomas, Michael I Polkey, Peter M George
Sarcoidosis is a multisystem condition which may affect a number of organs and, within the cardiopulmonary system, most commonly manifests as parenchymal, airway-centred, nodal, vascular or cardiac disease. Pleural involvement is rare, but well described, and often presents as pleural effusions or pleural thickening. Here, we present the first case of active sarcoidosis manifesting as bilateral pleural calcification. We highlight the importance of a nuanced understanding of pulmonary physiology when dissecting coexistent extrathoracic and intrathoracic pulmonary restriction...
June 7, 2018: Thorax
https://www.readbyqxmd.com/read/29880507/abnormal-ventilation-perfusion-scan-is-associated-with-pulmonary-hypertension-in-sickle-cell-adults
#17
Alem Mehari, Norris Igbineweka, Darlene Allen, Jim Nichols, Swee Lay Thein, Nargues A Weir
Pulmonary hypertension (PH) in adults with sickle cell disease (SCD) is associated with early mortality. Chronic thromboembolic pulmonary hypertension (CTEPH) is an important complication and contributor to PH in SCD, but likely underappreciated. Guidelines recommend ventilation-perfusion scintigraphy (V/Q) as imaging modality of choice to exclude CTEPH. Data on V/Q are limited in SCD. Objective: To compare the performance of V/Q with computed tomography pulmonary angiography (CTPA) and to report clinical outcomes associated with abnormal V/Q...
June 7, 2018: Journal of Nuclear Medicine: Official Publication, Society of Nuclear Medicine
https://www.readbyqxmd.com/read/29876357/pulmonary-hypertension-in-aortic-and-mitral-valve-disease
#18
Micha T Maeder, Lukas Weber, Marc Buser, Marc Gerhard, Philipp K Haager, Francesco Maisano, Hans Rickli
In patients with aortic and/or mitral valve disease the presence of pulmonary hypertension (PH) indicates a decompensated state of the disease with left ventricular and left atrial dysfunction and exhausted compensatory mechanism, i.e., a state of heart failure. Pulmonary hypertension in this context is the consequence of the backwards transmission of elevated left atrial pressure. In this form of PH, pulmonary vascular resistance is initially normal (isolated post-capillary PH). Depending on the extent and chronicity of left atrial pressure elevation additional pulmonary vascular remodeling may occur (combined pre- and post-capillary PH)...
2018: Frontiers in Cardiovascular Medicine
https://www.readbyqxmd.com/read/29876337/the-role-of-inflammation-in-venous-thromboembolism
#19
REVIEW
Brian R Branchford, Shannon L Carpenter
Venous thromboembolism (VTE), comprising deep vein thrombosis (DVT), and pulmonary embolism (PE), is becoming increasingly recognized as a cause of morbidity and mortality in pediatrics, particularly among hospitalized children. Furthermore, evidence is accumulating that suggests the inflammatory response may be a cause, as well as consequence, of VTE, but current anticoagulation treatment regimens are not designed to inhibit inflammation. In fact, many established clinical VTE risk factors such as surgery, obesity, cystic fibrosis, sepsis, systemic infection, cancer, inflammatory bowel disease, and lupus likely modulate thrombosis through inflammatory mediators...
2018: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/29875311/myeloperoxidase-aggravates-pulmonary-arterial-hypertension-by-activation-of-vascular-rho-kinase
#20
Anna Klinke, Eva Berghausen, Kai Friedrichs, Simon Molz, Denise Lau, Lisa Remane, Matthias Berlin, Charlotte Kaltwasser, Matti Adam, Dennis Mehrkens, Martin Mollenhauer, Kashish Manchanda, Thorben Ravekes, Gustavo A Heresi, Metin Aytekin, Raed A Dweik, Jan K Hennigs, Lukas Kubala, Erik Michaëlsson, Stephan Rosenkranz, Tanja K Rudolph, Stanley L Hazen, Hans Klose, Ralph T Schermuly, Volker Rudolph, Stephan Baldus
Pulmonary arterial hypertension (PAH) remains a disease with limited therapeutic options and dismal prognosis. Despite its etiologic heterogeneity, the underlying unifying pathophysiology is characterized by increased vascular tone and adverse remodeling of the pulmonary circulation. Myeloperoxidase (MPO), an enzyme abundantly expressed in neutrophils, has potent vasoconstrictive and profibrotic properties, thus qualifying as a potential contributor to this disease. Here, we sought to investigate whether MPO is causally linked to the pathophysiology of PAH...
June 7, 2018: JCI Insight
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