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https://www.readbyqxmd.com/read/29470642/real-world-switching-to-riociguat-management-and-practicalities-in-patients-with-pah-and-cteph
#1
Henning Gall, Jean-Luc Vachiéry, Nobuhiro Tanabe, Michael Halank, Mauricio Orozco-Levi, Lisa Mielniczuk, MiKyung Chang, Kai Vogtländer, Ekkehard Grünig
PURPOSE: A proportion of patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) do not achieve treatment goals or experience side effects on their current therapy. In such cases, switching patients to a new drug while discontinuing the first may be a viable and appropriate treatment option. CAPTURE was designed to investigate how physicians manage the switching of patients to riociguat in real-world clinical practice. Observations from the study were used to assess whether recommendations in the riociguat prescribing information are reflected in clinical practice...
February 22, 2018: Lung
https://www.readbyqxmd.com/read/29450722/incidence-and-characteristics-of-chronic-thromboembolic-pulmonary-hypertension-in-germany
#2
Thorsten Kramm, Heinrike Wilkens, Jan Fuge, Hans-Joachim Schäfers, Stefan Guth, Christoph B Wiedenroth, Bettina Weingard, Doerte Huscher, David Pittrow, Serghei Cebotari, Marius M Hoeper, Eckhard Mayer, Karen M Olsson
BACKGROUND: The incidence of chronic thromboembolic pulmonary hypertension (CTEPH) is unknown. Previous studies from the United Kingdom and Spain have reported incidence rates of 1.75 and 0.9 per million, respectively. These figures, however, may underestimate the true incidence of CTEPH. METHODS: We prospectively enrolled patients newly diagnosed with CTEPH within 2016 in Germany. Data were obtained from the three German referral centers and from the German branch of COMPERA, a European pulmonary hypertension registry...
February 15, 2018: Clinical Research in Cardiology: Official Journal of the German Cardiac Society
https://www.readbyqxmd.com/read/29435740/rv-fractional-area-change-and-tapse-as-predictors-of-severe-right-ventricular-dysfunction-in-pulmonary-hypertension-a-cmr-study
#3
Susana Hoette, Nicolas Creuzé, Sven Günther, David Montani, Laurent Savale, Xavier Jaïs, Florence Parent, Olivier Sitbon, Carlos Eduardo Rochitte, Gerald Simonneau, Marc Humbert, Rogerio Souza, Denis Chemla
BACKGROUND: The right ventricular ejection fraction (RVEF) is a surrogate marker of right ventricular function in pulmonary hypertension (PH), but its measurement is complicated and time consuming. The tricuspid annular plane systolic excursion (TAPSE) measures only the longitudinal component of RV contraction while the right ventricular fractional area change (RVFAC) takes into account both the longitudinal and the transversal components. The aim of our study was to evaluate the relationship between RVEF, RVFAC, and TAPSE according to hemodynamic severity in two groups of patients with PH: pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH)...
February 12, 2018: Lung
https://www.readbyqxmd.com/read/29435739/multiparametric-magnetic-resonance-imaging-in-the-assessment-of-pulmonary-hypertension-initial-experience-of-a-one-stop-study
#4
Gisela M B Meyer, Fernanda B Spilimbergo, Stephan Atmayer, Gabriel S Pacini, Matheus Zanon, Guilherme Watte, Edson Marchiori, Bruno Hochhegger
INTRODUCTION: Our goal was to assess the diagnostic performance of magnetic resonance imaging (MRI) as a single method to diagnose pulmonary hypertension (PH) compared to right heart catheterization (RHC), computed tomography (CT), and ventilation/perfusion (V/Q) scintigraphy. METHODS: We identified 35 patients diagnosed with PH by RHC in our institution who have also undergone a CT, a scintigraphy, and an MRI within a month. All cases were discussed in multidisciplinary meetings...
February 12, 2018: Lung
https://www.readbyqxmd.com/read/29424201/hereditary-hyperhomocysteinemia-associated-with-nephrotic-syndrome-complicated-by-artery-thrombosis-and-chronic-thromboembolic-pulmonary-hypertension-a-case-report
#5
Paolo Ruggeri, Federica Lo Bello, Francesco Nucera, Michele Gaeta, Francesco Monaco, Gaetano Caramori, Giuseppe Girbino
We present here the case of a 30-year-old man with a long term history of nephrotic syndrome (NS) who developed an episode of acute left main pulmonary artery thrombosis complicated by a lung abscess. During the hospital admission was also identified a concomitant hyperhomocysteinemia. After an atypical resection of the left upper pulmonary lobe and the starting of long term anticoagulation the patient was discharged but did not attend the planned follow up visits until one year later when he was seen again for severe dyspnea and exercise intolerance...
December 19, 2017: Monaldi Archives for Chest Disease, Archivio Monaldi Per le Malattie del Torace
https://www.readbyqxmd.com/read/29416329/sex-specific-cardiopulmonary-exercise-testing-indices-to-estimate-the-severity-of-inoperable-chronic-thromboembolic-pulmonary-hypertension
#6
Tian-Xiang Chen, Bigyan Pudasaini, Jian Guo, Su-Gang Gong, Rong Jiang, Lan Wang, Qin-Hua Zhao, Wen-Hui Wu, Ping Yuan, Jin-Ming Liu
Background: Sex differences in chronic thromboembolic pulmonary hypertension (CTEPH) have been revealed in few studies. Although right heart catheterization (RHC) is the gold standard for clinical diagnosis and assessment of prognosis in pulmonary hypertension (PH), cardiopulmonary exercise testing (CPET) has been a more widely used assessment of functional capacity, disease severity, prognosis, and treatment response in PH. We hypothesized that the "sex-specific" CPET indices could estimate the severity of inoperable CTEPH...
2018: International Journal of Chronic Obstructive Pulmonary Disease
https://www.readbyqxmd.com/read/29399522/can-thrombus-age-guide-thrombolytic-therapy
#7
REVIEW
Christopher Czaplicki, Hassan Albadawi, Sasan Partovi, Ripal T Gandhi, Keith Quencer, Amy R Deipolyi, Rahmi Oklu
Venous thrombosis (VT) is a common yet complex clinical condition that has shown minimal alteration in clinical management for decades. It is well known that thrombus evolves structurally over time, with complex changes resulting from the interplay between coagulation factors, cytokines, leukocytes and a myriad of other factors. Our current treatment options are most effective in the acute thrombus, which is composed predominantly of a loose mesh of fibrin and red blood cells (RBCs), making current anticoagulation therapies and thrombolytics quite effective in treatment...
December 2017: Cardiovascular Diagnosis and Therapy
https://www.readbyqxmd.com/read/29393580/bosentan-therapy-for-pulmonary-arterial-hypertension-and-chronic-thromboembolic-pulmonary-hypertension-a-systemic-review-and-meta-analysis
#8
Xinwang Chen, Zhenguo Zhai, Ke Huang, Wanmu Xie, Jun Wan, Chen Wang
BACKGROUND: Bosentan therapy has been recommended for pulmonary arterial hypertension (PAH) and might be beneficial for chronic thromboembolic pulmonary hypertension (CTEPH). We aimed to evaluate the specific effects of bosentan for PAH and CTEPH. METHODS: We performed a systemic review and meta-analysis of randomized controlled trials (RCTs) comparing efficacy and safety of bosentan treatment for PAH and CTEPH through major biomedical database. RESULTS: A total of 10 RCTs including 1185 patients were enrolled...
February 2, 2018: Clinical Respiratory Journal
https://www.readbyqxmd.com/read/29388467/emerging-therapeutics-in-pulmonary-hypertension
#9
Matthew K Hensley, Andrea Levine, Mark T Gladwin, Yen-Chun Lai
Pulmonary hypertension (PH) is a progressive and often fatal illness presenting with nonspecific symptoms of dyspnea, lower extremity edema, and exercise intolerance. Pathologically, endothelial dysfunction leads to abnormal intimal and smooth muscle proliferation along with reduced apoptosis, resulting in increased pulmonary vascular resistance (PVR) and elevated pulmonary pressures. PH is subdivided into five WHO groups based on the disease pathology and specific cause. While there are FDA-approved medications for the treatment of pulmonary arterial hypertension (PAH, Group 1 PH), as well as for chronic thromboembolic pulmonary hypertension (CTEPH, Group 4 PH), the morbidity and mortality remain high...
February 1, 2018: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/29361351/power-of-resting-echocardiographic-measurements-to-classify-pulmonary-hypertension-patients-according-to-european-society-of-cardiology-exercise-testing-risk-stratification-cut-offs
#10
Michaela B Rehman, Rodrigue Garcia, Luc Christiaens, Elisa Larrieu-Ardilouze, Luke S Howard, Petros Nihoyannopoulos
BACKGROUND: Right ventricular function is the major determinant of morbidity and mortality in pulmonary arterial hypertension (PAH). The ESC risk assessment strategy for PAH is based on clinical status, exercise testing, NTproBNP, imaging and haemodynamics but does not include right ventricular function. Our aims were to test the power of resting echocardiographic measurements to classify PAH patients according to ESC exercise testing risk stratification cut-offs and to determine if the classification power of echocardiographic parameters varied in chronic thrombo-embolic pulmonary hypertension (CTEPH)...
January 17, 2018: International Journal of Cardiology
https://www.readbyqxmd.com/read/29360065/balloon-pulmonary-angioplasty-relieves-hemodynamic-stress-towards-untreated-side-pulmonary-vasculature-and-ameliorates-its-resistance-in-patients-with-chronic-thromboembolic-pulmonary-hypertension
#11
Kazuya Hosokawa, Kohtaro Abe, Koshin Horimoto, Yuzo Yamasaki, Michinobu Nagao, Hiroyuki Tsutsui
AIMS: Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by organized thrombotic obliteration of major vessels and small-vessel arteriopathy in the non-thrombosed vessels. The aim of this study was to investigate the impact of balloon pulmonary angioplasty (BPA) on the non-BPA-side pulmonary vasculature in patients with CTEPH. METHODS AND RESULTS: This study explored the outcomes of 20 unilateral BPA sessions in 13 CTEPH patients. We measured the pulmonary vascular resistance (PVR), pulmonary artery (PA) flow in the BPA-side and non-BPA-side lungs, respectively, using phase contrast MRI and cardiac catheterization...
January 23, 2018: EuroIntervention
https://www.readbyqxmd.com/read/29356440/adherence-and-medication-belief-in-patients-with-pulmonary-arterial-hypertension-or-chronic-thromboembolic-pulmonary-hypertension-a-nationwide-population-based-cohort-survey
#12
Bodil Ivarsson, Roger Hesselstrand, Göran Rådegran, Barbro Kjellström
BACKGROUND: Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are rare diseases with a gradual decline in physical health. Adherence to treatment is crucial in these very symptomatic and life threatening diseases. OBJECTIVE: To describe PAH and CTEPH patients experience of their self-reported medication adherence, beliefs about medicines and information about treatment. METHODS: A quantitative, descriptive, national cohort survey that included adult patients from all PAH-centres in Sweden...
January 22, 2018: Clinical Respiratory Journal
https://www.readbyqxmd.com/read/29332912/impairment-of-iodine-123-metaiodobenzylguanidine-123i-mibg-uptake-in-patients-with-pulmonary-artery-hypertension
#13
Kenjuro Higo, Kayoko Kubota, Sunao Miyanaga, Masaaki Miyata, Masatoyo Nakajo, Megumi Jinguji, Mitsuru Ohishi
According to recent studies, lung uptake of iodine-123-metaiodobenzylguanidine (123I-MIBG) is impaired in many lung diseases and low lung uptake of 123I-MIBG suggests endothelial dysfunction of the pulmonary artery. 123I-MIBG scintigraphy in patients with pulmonary hypertension (PH) has not yet been clinically evaluated. We hypothesized that the lung uptake of 123I-MIBG is reduced in patients with PH and differs among PH subtypes. The purpose of the present study was to analyze the lung uptake of 123I-MIBG in patients with PH and compare it with the data obtained by echocardiography or right heart catheterization...
January 15, 2018: International Heart Journal
https://www.readbyqxmd.com/read/29329761/n-terminal-pro-b-type-natriuretic-peptide-for-monitoring-after-balloon-pulmonary-angioplasty-for-chronic-thromboembolic-pulmonary-hypertension
#14
Steffen D Kriechbaum, Christoph B Wiedenroth, Jan Sebastian Wolter, Regula Hütz, Moritz Haas, Andreas Breithecker, Fritz C Roller, Till Keller, Stefan Guth, Andreas Rolf, Christian W Hamm, Eckhard Mayer, Christoph Liebetrau
BACKGROUND: Balloon pulmonary angioplasty (BPA) is an emerging interventional treatment option for chronic thromboembolic pulmonary hypertension (CTEPH). The non-invasive monitoring of CTEPH patients is a clinical challenge. In this study we examined changes in N-terminal pro-B-type natriuretic peptide (NT-proBNP) in patients undergoing BPA for inoperable CTEPH and related them to peri-procedural success. METHODS: In this study we analyzed a total of 51 consecutive patients who underwent BPA treatment and completed a 6-month follow-up (6-MFU) between March 2014 and March 2017...
December 8, 2017: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/29321405/reversible-parkinsonism-and-multiple-cerebral-infarctions-after-pulmonary-endarterectomy-in-a-patient-with-antiphospholipid-syndrome
#15
Mitsumasa Okano, Kazuhiko Nakayama, Naoki Tamada, Yuto Shinkura, Ken-Ichi Yanaka, Hiroyuki Onishi, Hidekazu Tanaka, Toshiro Shinke, Hiroshi Tanaka, Yutaka Okita, Noriaki Emoto, Ken-Ichi Hirata
Antiphospholipid syndrome (APS) is a cause of chronic thromboembolic pulmonary hypertension (CTEPH) and it is associated with an increased risk of postoperative neurological complications. We experienced a case of reversible parkinsonism after pulmonary endarterectomy (PEA) and subsequent multiple cerebral infarctions under standard anticoagulation therapy in a patient with CTEPH associated with APS. Strict management using a combination of antiplatelet and anticoagulation therapy should be considered in patients with a high titer of triple antiphospholipid antibodies in the perioperative period...
January 11, 2018: Internal Medicine
https://www.readbyqxmd.com/read/29319041/successful-pulmonary-thromboendarterectomy-in-a-patient-with-sickle-cell-disease-and-associated-resolution-of-a-leg-ulcer
#16
Abhinav Agrawal, Rakesh Shah, Matthew D Bacchetta, Arunabh Talwar
Pulmonary hypertension (PH) is a relatively frequent and severe complication of sickle cell disease (SCD). PH associated with SCD is classified as Group 5 PH. The exact pathogenesis of PH in SCD in not known. There are also very limited treatment options available at this time for such patients with Group 5 PH. Patients with SCD are predisposed to a hypercoagulable state and thus can also suffer from chronic thromboembolism. These patients can have associated chronic thromboembolic pulmonary hypertension (CTEPH), thus being classified as Group 4 PH...
January 2018: Lung India: Official Organ of Indian Chest Society
https://www.readbyqxmd.com/read/29318791/impact-of-balloon-pulmonary-angioplasty-on-hemodynamics-and-clinical-outcomes-in-patients-with-chronic-thromboembolic-pulmonary-hypertension-the-initial-korean-experience
#17
Woochan Kwon, Jeong Hoon Yang, Taek Kyu Park, Sung A Chang, Dong Seop Jung, Young Seok Cho, Sung Mok Kim, Tae Jung Kim, Hye Yoon Park, Seung Hyuk Choi, Duk Kyung Kim
BACKGROUND: The treatment of choice for chronic thromboembolic pulmonary hypertension (CTEPH) is pulmonary endarterectomy (PEA). However, not all patients are eligible for PEA, and some patients experience recurrence of pulmonary hypertension even after PEA. METHODS: Patients who underwent balloon pulmonary angioplasty (BPA) between December 2015 and April 2017 were enrolled from the Samsung Medical Center CTEPH registry. Enrolled patients underwent right heart catheterization, echocardiography, and 6-minute walk distance (6MWD) at baseline, 4 and 24 weeks after their first BPA session...
January 22, 2018: Journal of Korean Medical Science
https://www.readbyqxmd.com/read/29290029/the-clinical-value-of-assessing-right-ventricular-diastolic-function-after-balloon-pulmonary-angioplasty-in-patients-with-chronic-thromboembolic-pulmonary-hypertension
#18
Hidenori Moriyama, Mitsushige Murata, Toshimitsu Tsugu, Takashi Kawakami, Masaharu Kataoka, Takahiro Hiraide, Mai Kimura, Sarasa Isobe, Jin Endo, Takashi Kohno, Yuji Itabashi, Keiichi Fukuda
Chronic thromboembolic pulmonary hypertension (CTEPH) has a poor prognosis because of the associated progressive right heart failure. Accurate evaluation of right ventricular (RV) function would thus be useful to predict prognosis. However, the significance of RV diastolic function remains unclear. We aimed to identify which echocardiographic measures are most accurate, and potentially useful, in assessing RV diastolic function in patients with CTEPH, and to study the effects of balloon pulmonary angioplasty (BPA) on them...
December 30, 2017: International Journal of Cardiovascular Imaging
https://www.readbyqxmd.com/read/29284512/quantifying-the-learning-curve-for-pulmonary-thromboendarterectomy
#19
Smita Sihag, Bao Le, Alison S Witkin, Josanna M Rodriguez-Lopez, Mauricio A Villavicencio, Gus J Vlahakes, Richard N Channick, Cameron D Wright
BACKGROUND: Pulmonary thromboendarterectomy (PTE) is an effective treatment for chronic thromboembolic pulmonary hypertension (CTEPH), but is a technically challenging operation for cardiothoracic surgeons. Starting a new program allows an opportunity to define a learning curve for PTE. METHODS: A retrospective case review was performed of 134 consecutive PTEs performed from 1998 to 2016 at a single institution. Outcomes were compared using either a two-tailed t-test for continuous variables or a chi-squared test for categorical variables according to experience of the program by terciles (T)...
December 28, 2017: Journal of Cardiothoracic Surgery
https://www.readbyqxmd.com/read/29282032/riociguat-in-patients-with-chronic-thromboembolic-pulmonary-hypertension-results-from-an-early-access-study
#20
Vallerie V McLaughlin, Pavel Jansa, Jens E Nielsen-Kudsk, Michael Halank, Gérald Simonneau, Ekkehard Grünig, Silvia Ulrich, Stephan Rosenkranz, Miguel A Gómez Sánchez, Tomás Pulido, Joanna Pepke-Zaba, Joan Albert Barberá, Marius M Hoeper, Jean-Luc Vachiéry, Irene Lang, Francine Carvalho, Christian Meier, Katharina Mueller, Sylvia Nikkho, Andrea M D'Armini
BACKGROUND: Following positive results from the Phase III CHEST-1 study in patients with inoperable or persistent/recurrent chronic thromboembolic pulmonary hypertension (CTEPH), the Phase IIIb CTEPH early access study (EAS) was designed to assess the safety and tolerability of riociguat in real-world clinical practice, as well as to provide patients with early access to riociguat before launch. Riociguat is approved for the treatment of inoperable and persistent/recurrent CTEPH. METHODS: We performed an open-label, uncontrolled, single-arm, early access study in which 300 adult patients with inoperable or persistent/recurrent CTEPH received riociguat adjusted from 1 mg three times daily (tid) to a maximum of 2...
December 28, 2017: BMC Pulmonary Medicine
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