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Pulmonary Circulation

Alessandra Greco, Sara Plumitallo, Laura Scelsi, Giannantonio Maggi, Matteo Sobrero, Annalisa Turco, Claudia Raineri, Natalia Arseni, Donata Cappelletti, Luigi Oltrona Visconti, Fabio Pagella, Giuseppe Spinozzi, Stefano Ghio, Carla Olivieri, Cesare Danesino
Hereditary hemorrhagic telangiectasia (HTT) is an autosomal dominant disease, most frequently caused by a mutation in either ENG or ACVRL1, which can be associated with pulmonary arterial hypertension (PAH). In this report, we describe a new unpublished ACVRL1 mutation segregating in three members of the same family, showing three different types of pulmonary hypertension (PH) in the absence of BMPR2 mutations. The first patient has a form of heritable PAH (HPAH) in the absence of hepatic arteriovenous malformations (AVMs); the second one has a severe form of portopulmonary hypertension (PoPAH) associated with multiple hepatic AVMs; the third one has hepatopulmonary syndrome (HPS) with numerous hepatic arteriovenous fistulas and a form of post-capillary PH due to high cardiac output...
October 2018: Pulmonary Circulation
Kazuhiko Nakayama, Noriaki Emoto, Naoki Tamada, Mitsumasa Okano, Yuto Shinkura, Kenichi Yanaka, Hiroyuki Onishi, Mana Hiraishi, Shinichiro Yamada, Hidekazu Tanaka, Toshiro Shinke, Ken-Ichi Hirata
Inhaled iloprost efficiently improves pulmonary hemodynamics, exercise capacity, and quality of life in patients with pulmonary arterial hypertension (PAH). However, the process of inhalation is laborious for patients suffering from resting dyspnea. We describe a 75-year-old man with idiopathic PAH and a low gas transfer. Investigations excluded significant parenchymal lung disease and airflow obstruction (presuming FEV1/FVC ration > 70%). The patient struggled to complete iloprost inhalation due to severe dyspnea and hypoxemia...
October 2018: Pulmonary Circulation
Carol Keen, Sally Fowler-Davis, Sionnadh McLean, Jane Manson
Pulmonary hypertension (PH) is a life-limiting disease affecting circulation to the lungs. The primary symptom of PH is breathlessness, yet research has shown that patients with PH can exercise safely and can benefit from exercise to improve exercise capacity and maintain quality of life. This study aimed to investigate the nature of physiotherapy delivered to patients with PH in the UK. This was a two-phase sequential, exploratory, mixed-methods study. Interviews were conducted with seven lead physiotherapists at specialist pulmonary hypertension centers and three patients...
July 2018: Pulmonary Circulation
Christoph B Wiedenroth, H Ardeschir Ghofrani, Miriam S D Adameit, Andreas Breithecker, Moritz Haas, Steffen Kriechbaum, Andreas Rieth, Christian W Hamm, Eckhard Mayer, Stefan Guth, Christoph Liebetrau
Riociguat is the treatment of choice for inoperable patients with chronic thromboembolic pulmonary hypertension (CTEPH). We addressed here whether additional balloon pulmonary angioplasty (BPA) provides further benefits. A prospective series of 36 consecutive patients with inoperable CTEPH were treated with riociguat at least three months before BPA. All patients underwent diagnostic workup at baseline, before BPA treatments, and six months after final intervention. The main outcome measures were pulmonary hemodynamic parameters and World Health Organization (WHO) functional class (FC)...
July 2018: Pulmonary Circulation
(no author information available yet)
No abstract text is available yet for this article.
July 2018: Pulmonary Circulation
Lili Xiang, Ying Li, Xu Deng, Djuro Kosanovic, Ralph Theo Schermuly, Xiaohui Li
Pulmonary arterial hypertension (PAH) is a severe disease characterized by progressive remodeling of distal pulmonary arteries and persistent elevation of pulmonary vascular resistance (PVR), which leads to right ventricular dysfunction, heart failure, and eventually death. Although treatment responsiveness for this disease is improving, it continues to be a life-threatening condition. With the clinical efficacy of natural plant products being fully confirmed by years of practice, more and more recognition and attention have been obtained from the international pharmaceutical industry...
July 2018: Pulmonary Circulation
Rebecca F Hough, Sunita Bhattacharya, Jahar Bhattacharya
Crosstalk signaling between the closely juxtaposed epithelial and endothelial membranes of pulmonary alveoli establishes the lung's immune defense against inhaled and blood-borne pathogens. The crosstalk can occur in a forward direction, as from alveolus to capillary, or in a reverse direction, as from capillary to alveolus. The crosstalk direction likely depends on the site at which pathogens first initiate signaling. Thus, forward crosstalk may occur when inhaled pathogens encounter the alveolar epithelium, while reverse crosstalk may result from interactions of blood-borne pathogens with the endothelium...
July 2018: Pulmonary Circulation
Yadi Huang, Anne Boerema-de Munck, Marjon Buscop-van Kempen, Ilona Sluiter, Ronald de Krijger, Dick Tibboel, Robbert J Rottier
We show that hypoxia inducible factor 2α (HIF2α) is highly expressed in patients with pulmonary hypertension (PH). HIF2α is expressed in every patient with congenital diaphragmatic hernia, while only half of the controls express HIF2α. Our data suggest that HIF2α is a link between hypoxia and the development of PH.
July 2018: Pulmonary Circulation
Cihangir Kaymaz, Ozgur Yasar Akbal, Aykun Hakgor, Hacer Ceren Tokgoz, Seda Tanyeri
Despite the significant mortality and mobidity benefits being obtained with the targeted therapies in patients with pulmonary arterial hypertension (PAH), mid- to long-term survival of patients with this disease has remained unsatisfactory. For earlier and reliable risk stratification in PAH and tailoring the dynamic management strategies, various risk assessment models have been developed. Currently available risk reduction strategy recommended by the European Society of Cardiology (ESC)/European Respiratory Society (ERS) 2015 Pulmonary Hypertension Guidelines has been utilized in three recent registries...
July 2018: Pulmonary Circulation
Weiguang Yang, Alison L Marsden, Michelle T Ogawa, Charlotte Sakarovitch, Keeley K Hall, Marlene Rabinovitch, Jeffrey A Feinstein
Pulmonary arterial hypertension (PAH) is characterized by elevated pulmonary artery pressures (PAP) and pulmonary vascular resistance (PVR). Optimizing treatment strategies and timing for transplant remains challenging. Thus, a quantitative measure to predict disease progression would be greatly beneficial in treatment planning. We devised a novel method to assess right ventricular (RV) stroke work (RVSW) as a potential biomarker of the failing heart that correlates with clinical worsening. Pediatric patients with idiopathic PAH or PAH secondary to congenital heart disease who had serial, temporally matched cardiac catheterization and magnetic resonance imaging (MRI) data were included...
July 2018: Pulmonary Circulation
Hatasu Kobayashi, Risako Kabata, Hideyuki Kinoshita, Takaaki Morimoto, Koh Ono, Midori Takeda, Jungmi Choi, Hiroko Okuda, Wanyang Liu, Kouji H Harada, Takeshi Kimura, Shohab Youssefian, Akio Koizumi
Ring finger 213 ( RNF213) is a susceptibility gene for moyamoya disease (MMD), a progressive cerebrovascular disease. Recent studies suggest that RNF213 plays an important role not only in MMD, but also in extracranial vascular diseases, such as pulmonary hypertension (PH). In this study, we undertook genetic screening of RNF213 in patients with PH and performed functional analysis of an RNF213 variant using mouse models. Direct sequencing of the exons in the C-terminal region of RNF213, where MMD-associated mutations are highly clustered, and of the entire coding exons of BMPR2 and CAV1, the causative genes for PH, was performed in 27 Japanese patients with PH...
July 2018: Pulmonary Circulation
Devaki O'Riordan, David G Kiely, B Ronan O'Driscoll
Deterioration, or a new presentation, of pulmonary arterial hypertension (PAH), are recognized complications of pregnancy. In this report, we describe a patient with a family history of PAH who developed peripartum breathlessness and hypoxemia with ventilation-perfusion mismatch but no evidence of thromboembolism or PAH. Significantly reduced perfusion at both lung bases was noted on perfusion scintigraphy and three-dimensional magnetic resonance (3D-MR) perfusion maps in the immediate postpartum period. These abnormalities spontaneously resolved by 16 weeks postpartum, consistent with reversible pulmonary abnormalities of pulmonary perfusion...
July 2018: Pulmonary Circulation
Satomi Ishihara, Hidetaka Kioka, Tomohito Ohtani, Yoshihiro Asano, Osamu Yamaguchi, Shungo Hikoso, Koichi Toda, Yoshihiko Saito, Yoshiki Sawa, Keiko Yamauchi-Takihara, Yasushi Sakata
Restrictive cardiomyopathy (RCM) is a rare form of cardiomyopathy that is characterized by restrictive ventricular filling. Elevated filling pressure leads to pulmonary hypertension (PH), which often progresses to combined post- and pre-capillary PH (Cpc-PH) with increased diastolic pulmonary vascular pressure gradient (DPG) and pulmonary vascular resistance (PVR) caused by longstanding backward hemodynamic consequences of left heart disease (LHD) leading to morphological changes in the pulmonary vasculature...
July 2018: Pulmonary Circulation
Maria Anna Bazmpani, Georgios Arsos, Paul Zarogoulidis, Argyrios Doumas, Theodoros Dimitroulas, George Sianos, Stavros Hadjimiltiades, Konstantinos Kouskouras, Eckhard Mayer, Haralambos Karvounis, George Giannakoulas
Occlusive vasculopathy due to the development and accumulation of granulomas at the level of intima of large vessels, as well as mediastinal lymph nodes and fibrosing mediastinitis secondary to sarcoidosis, causing extrinsic compression of mediastinal vascular structure are uncommon mechanisms of sarcoidosis-associated pulmonary hypertension. We present a case of a 62-year-old woman with a rare manifestation of sarcoidosis, which was misclassified and treated as chronic thromboembolic pulmonary hypertension for a long period...
July 2018: Pulmonary Circulation
Valentina Mercurio, Paul M Hassoun
No abstract text is available yet for this article.
April 2018: Pulmonary Circulation
Ryan Davey
No abstract text is available yet for this article.
April 2018: Pulmonary Circulation
Sasiharan Sithamparanathan, Mariana C Rocha, Jehill D Parikh, Karolina A Rygiel, Gavin Falkous, John P Grady, Kieren G Hollingsworth, Michael I Trenell, Robert W Taylor, Doug M Turnbull, Gráinne S Gorman, Paul A Corris
Mitochondrial dysfunction within the pulmonary vessels has been shown to contribute to the pathology of idiopathic pulmonary arterial hypertension (IPAH). We investigated the hypothesis of whether impaired exercise capacity observed in IPAH patients is in part due to primary mitochondrial oxidative phosphorylation (OXPHOS) dysfunction in skeletal muscle. This could lead to potentially new avenues of treatment beyond targeting the pulmonary vessels. Nine clinically stable participants with IPAH underwent cardiopulmonary exercise testing, in vivo and in vitro assessment of mitochondrial function by 31 P-magnetic resonance spectroscopy (31 P-MRS) and laboratory muscle biopsy analysis...
April 2018: Pulmonary Circulation
Julio D Duarte, Mayank Kansal, Ankit A Desai, Katherine Riden, Meghan J Arwood, Alex A Yacob, Thomas D Stamos, Larisa H Cavallari, Roham T Zamanian, Sanjiv J Shah, Roberto F Machado
The biological mechanisms behind the development of pulmonary hypertension in the setting of left heart failure (HF-PH), including combined pre- and post-capillary pulmonary hypertension (Cpc-PH), remains unclear. This study aimed to use candidate polymorphisms in nitric oxide synthase (NOS) genes to explore the role of NOS in HF-PH. DNA samples from 118 patients with HF-PH were genotyped for the NOS3 rs1799983 and NOS2 rs3730017 polymorphisms. A multiple regression model was used to compare hemodynamic measurements between genotype groups...
April 2018: Pulmonary Circulation
Pratap Karki, Anna A Birukova
The maintenance of endothelial barrier integrity is absolutely essential to prevent the vascular leak associated with pneumonia, pulmonary edema resulting from inhalation of toxins, acute elevation to high altitude, traumatic and septic lung injury, acute lung injury (ALI), and its life-threatening complication, acute respiratory distress syndrome (ARDS). In addition to the long-known edemagenic and inflammatory agonists, emerging evidences suggest that factors of endothelial cell (EC) mechanical microenvironment such as blood flow, mechanical strain of the vessel, or extracellular matrix stiffness also play an essential role in the control of endothelial permeability and inflammation...
April 2018: Pulmonary Circulation
Michelle C Sykes, Christina Ireland, Julia E McSweeney, Emily Rosenholm, Kristofer G Andren, Thomas J Kulik
Pulmonary vein stenosis (PVS) is associated with pulmonary hypertension (PH), but there is little information regarding the impact of PH on right ventricular (RV) systolic function and survival. We conducted a retrospective cohort study of our patients to explore this and other aspects of pulmonary hemodynamics with PVS. RV function was assessed using qualitative two-dimensional echocardiography. The ratio of systolic pulmonary artery (PA) and aortic pressures (PA:Ao) at cardiac catheterization reflected pulmonary hemodynamics...
April 2018: Pulmonary Circulation
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