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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
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
Andrea Frump, Allison Prewitt, Mark P de Caestecker
Despite the discovery more than 15 years ago that patients with hereditary pulmonary arterial hypertension (HPAH) inherit BMP type 2 receptor ( BMPR2) mutations, it is still unclear how these mutations cause disease. In part, this is attributable to the rarity of HPAH and difficulty obtaining tissue samples from patients with early disease. However, in addition, limitations to the approaches used to study the effects of BMPR2 mutations on the pulmonary vasculature have restricted our ability to determine how individual mutations give rise to progressive pulmonary vascular pathology in HPAH...
April 2018: Pulmonary Circulation
Stefan Uhlig, Wolfgang M Kuebler
Fifty years after the first description of acute respiratory distress syndrome (ARDS), none of the many positive drug studies in animal models have been confirmed in clinical trials and translated into clinical practice. This bleak outcome of so many animal experiments shows how difficult it is to model ARDS. Lungs from patients are characterized by hyperinflammation, permeability edema, and hypoxemia; accordingly, this is what most models aim to reproduce. However, in animal models it is very easy to cause inflammation in the lungs, but difficult to cause hypoxemia...
April 2018: Pulmonary Circulation
Wei Xiong, Yunfeng Zhao, Sugang Gong, Qinhua Zhao, Jinming Liu
The long-term oxygen therapy (LTOT) for patients with chronic obstructive pulmonary disease (COPD) has been shown to increase survival in patients with severe resting hypoxemia. The adherence to LTOT may also simultaneously affect the development of concomitant pulmonary hypertension (PH) due to COPD with hypoxemia. We retrospectively reviewed 276 cases of COPD with or without PH assessed by right heart catheterization (RHC) to investigate whether adherence to continuous LTOT had a prophylactic effect on the development of PH in a time interval of two years...
April 2018: Pulmonary Circulation
Toshitaka Nakaya, Ichizo Tsujino, Hiroshi Ohira, Takahiro Sato, Taku Watanabe, Noriko Oyama-Manabe, Masaharu Nishimura
Right ventricular (RV) function is an important determinant of the prognosis in patients with pulmonary arterial hypertension (PAH). In the context of recent therapeutic progress, there is an increasing need for better monitoring of RV function for management of PAH. We present the case of a 42-year-old woman with idiopathic PAH who was treated with three oral pulmonary vasodilators, i.e. tadalafil, ambrisentan, and beraprost. At the baseline assessment, the mean pulmonary arterial pressure (mPAP) was 45 mmHg, cardiac index (CI) was 1...
April 2018: Pulmonary Circulation
Nima Moghaddam, Bahar Moghaddam, Natasha Dehghan, Nathan W Brunner
Pulmonary artery (PA) vasculitis occurs in association with primary vasculitides-in particular, giant cell arteritis, Takayasu's arteritis, or Behçet's disease-or secondary vasculitis as a result of infections or malignancy. However, PA vasculitis in isolation and with concomitant aneurysmal dilation is an unusual finding. We present a rare case of PA aneurysm secondary to isolated PA vasculitis in an asymptomatic patient with no features of systemic vasculitis. This case highlights one of the first cases of PA vasculitis managed with surgical resection alone...
April 2018: Pulmonary Circulation
Fanny Loisel, Bastien Provost, François Haddad, Julien Guihaire, Myriam Amsallem, Bojan Vrtovec, Elie Fadel, Georges Uzan, Olaf Mercier
Pulmonary arterial hypertension (PAH) is an incurable disease characterized by an increase in pulmonary arterial pressure due to pathological changes to the pulmonary vascular bed. As a result, the right ventricle (RV) is subject to an increased afterload and undergoes multiple changes, including a decrease in capillary density. All of these dysfunctions lead to RV failure. A number of studies have shown that RV function is one of the main prognostic factors for PAH patients. Many stem cell therapies targeting the left ventricle are currently undergoing development...
April 2018: Pulmonary Circulation
Anneloes Dummer, Nina Rol, Robert Szulcek, Kondababu Kurakula, Xiaoke Pan, Benjamin I Visser, Harm Jan Bogaard, Marco C DeRuiter, Marie-José Goumans, Beerend P Hierck
Pulmonary arterial hypertension (PAH) is a syndrome characterized by progressive lung vascular remodelling, endothelial cell (EC) dysfunction, and excessive inflammation. The primary cilium is a sensory antenna that integrates signalling and fine tunes EC responses to various stimuli. Yet, cilia function in the context of deregulated immunity in PAH remains obscure. We hypothesized that cilia function is impaired in ECs from patients with PAH due to their inflammatory status and tested whether cilia length changes in response to cytokines...
April 2018: Pulmonary Circulation
Li Huang, Li Li, Enci Hu, Guo Chen, Xianmin Meng, Changming Xiong, Jianguo He
Whether pulmonary arterial hypertension (PAH) is reversible in congenital heart disease (CHD) is important for the operability of CHD. However, little is known about that. Our research was aimed at exploring novel biomarkers and targets in the reversibility of CHD-PAH. CHD-PAH patients diagnosed with right heart catheterization (RHC) were enrolled (n = 14). Lung biopsy was performed during the repair surgery. After one year follow-up, mean pulmonary arterial pressures (mPAP) were evaluated by RHC to determine the diagnosis of reversible (mPAP < 25 mmHg, n = 10) and irreversible (mPAP ≥ 25 mmHg, n = 4) PAH...
April 2018: Pulmonary Circulation
Sook Kyung Yum, Min-Sung Kim, Yoojin Kwun, Cheong-Jun Moon, Young-Ah Youn, In Kyung Sung
We aimed to evaluate the association between the presence of histologic chorioamnionitis (HC) and development of pulmonary hypertension (PH) during neonatal intensive care unit (NICU) stay. Data of preterm infants born at 32 weeks of gestation or less were reviewed. The development of PH and other respiratory outcomes were compared according to the presence of HC. Potential risk factors associated with the development of PH during NICU stay were used for multivariable logistic regression analysis. A total of 188 infants were enrolled: 72 in the HC group and 116 in the no HC group...
April 2018: Pulmonary Circulation
Wolfgang M Kuebler, Sébastien Bonnet, Arata Tabuchi
While pulmonary hypertension (PH) has traditionally not been considered as a disease that is directly linked to or, potentially, even caused by inflammation, a rapidly growing body of evidence has demonstrated the accumulation of a variety of inflammatory and immune cells in PH lungs, in and around the wall of remodeled pulmonary resistance vessels and in the vicinity of plexiform lesions, respectively. Concomitantly, abundant production and release of various inflammatory mediators has been documented in both PH patients and experimental models of PH...
April 2018: Pulmonary Circulation
Basil Al-Sabeq, Sabe De, Ryan Davey
Pulmonary hypertension (PH) in adults with congenital heart disease (CHD) and significant systemic-to-pulmonary shunting is a significant cause of morbidity and mortality. Its pathophysiology is incompletely understood, but involves a flow-induced pulmonary arteriopathy characterized by endothelial cell dysfunction and vascular remodeling that alters pulmonary arterial vasoreactivity. There is a paucity of literature linking PH with left-to-right shunting due to ruptured sinus of Valsalva aneurysms (SOVA). We present a unique case of reversible, flow-associated PH due to a ruptured congenital right SOVA fistulizing into the right atrium (RA), with emphasis on non-invasive and invasive assessment of pulmonary hemodynamics before and after surgical intervention...
April 2018: Pulmonary Circulation
Wen Li, Chang-Ming Xiong, Qing Gu, Xiao-Tong Wang, Xiao-Ling Cheng, Li Huang, Tao Yang, Qin Luo, Zhi-Hui Zhao, Xin-Hai Ni, Zhi-Hong Liu, Jian-Guo He
Pulmonary arterial hypertension (PAH) is a severe complication of hereditary hemorrhagic telangiectasia (HHT); however, little is known about its clinical characteristics and prognosis. Nine newly diagnosed HHT-PAH patients were prospectively recruited between October 2007 and January 2016 and were followed up every half-year. Eighteen idiopathic pulmonary arterial hypertension (IPAH) patients, matched with HHT-PAH patients on mean pulmonary arterial pressure, pulmonary capillary wedge pressure, pulmonary vascular resistance, cardiac index, and World Health Organization (WHO) functional class (FC), were recruited...
April 2018: Pulmonary Circulation
Bin Q Yang, Tufik R Assad, Jared M O'Leary, Meng Xu, Stephen J Halliday, Reid W D'Amico, Eric H Farber-Eger, Quinn S Wells, Anna R Hemnes, Evan L Brittain
African Americans (AA) have a higher incidence of pulmonary hypertension (PH) risk factors. Few studies have examined the racial differences in the prevalence and etiology of PH and direct comparison of invasive hemodynamics between AAs and Caucasians has rarely been reported. In this study, we examined whether racial differences exist in patients referred for right heart catheterization (RHC) and hypothesized that AA race is an independent risk factor for PH and is associated with increased adjusted mortality...
April 2018: Pulmonary Circulation
Rachel K Hopper, Yan Wang, Valerie DeMatteo, Ashley Santo, Steven M Kawut, Okan U Elci, Brian D Hanna, Laura Mercer-Rosa
Pulmonary hypertension (PH) causes significant morbidity and mortality in children due to right ventricular (RV) failure. We sought to determine the effect of prostacyclin analogues on RV function assessed by echocardiography in children with PH. We conducted a retrospective cohort study of children with PH treated with a prostacyclin analogue (epoprostenol or treprostinil) between January 2001 and August 2015 at our center. Data were collected before initiation of treatment (baseline) and at 1-3 and 6-12 months after...
April 2018: Pulmonary Circulation
Xiaofang Zeng, Tianyu Lian, Jianhui Lin, Suqi Li, Haikuo Zheng, Chunyan Cheng, Jue Ye, Zhicheng Jing, Xiaojian Wang, Wei Huang
Sanger sequencing, the traditional "gold standard" for mutation detection, has been wildly used in genetic testing of pulmonary artery hypertension (PAH). However, with the advent of whole-exome sequencing (WES), few studies have compared the accuracy of WES and Sanger sequencing in routine genetic testing of PAH. PAH individuals were enrolled from Fu Wai Hospital and Shanghai Pulmonary Hospital. WES was used to analyze DNA samples from 120 PAH patients whose bone morphogenetic protein receptor type 2 (BMPR2) mutation statuses had been previously studied using Sanger sequencing...
April 2018: Pulmonary Circulation
Ting Wang, Mary E Brown, Gabriel T Kelly, Sara M Camp, Joseph B Mascarenhas, Xiaoguang Sun, Steven M Dudek, Joe G N Garcia
Sphingosine 1-phosphate (S1P) is a potent bioactive endogenous lipid that signals a rearrangement of the actin cytoskeleton via the regulation of non-muscle myosin light chain kinase isoform (nmMLCK). S1P induces critical nmMLCK Y464 and Y471 phosphorylation resulting in translocation of nmMLCK to the periphery where spatially-directed increases in myosin light chain (MLC) phosphorylation and tension result in lamellipodia protrusion, increased cell-cell adhesion, and enhanced vascular barrier integrity. MYLK, the gene encoding nmMLCK, is a known candidate gene in lung inflammatory diseases, with coding genetic variants (Pro21His, Ser147Pro, Val261Ala) that confer risk for inflammatory lung injury and influence disease severity...
April 2018: Pulmonary Circulation
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