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Pulmonary HTN

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By Gregory Gustafson Interventional Cardiologist
David Jenkins
Chronic thromboembolic pulmonary hypertension (CTEPH) can be defined as a type of precapillary pulmonary hypertension (PH) resulting from incomplete resolution of pulmonary embolism. Symptoms are exertional breathlessness and most patients come to a cardiologist's attention with a dilated right heart on echocardiography. Patients with suspected CTEPH should be referred for evaluation to a PH specialist centre to confirm the diagnosis. There are now three treatment options available, dependent on the anatomical level of the obstruction: pulmonary endarterectomy surgery, balloon pulmonary angioplasty and pulmonary arterial hypertension (PAH)-targeted drugs...
September 2018: Heart: Official Journal of the British Cardiac Society
Nobuhiro Yaoita, Hiroaki Shimokawa
No abstract text is available yet for this article.
May 1, 2018: American Journal of Physiology. Heart and Circulatory Physiology
Steven Hsu, Kristen M Kokkonen-Simon, Jonathan A Kirk, Todd M Kolb, Rachel L Damico, Stephen C Mathai, Monica Mukherjee, Ami A Shah, Fredrick M Wigley, Kenneth B Margulies, Paul M Hassoun, Marc K Halushka, Ryan J Tedford, David A Kass
BACKGROUND: Patients with systemic sclerosis (SSc)-associated pulmonary arterial hypertension (PAH) have a far worse prognosis than those with idiopathic PAH (IPAH). In the intact heart, SSc-PAH exhibits depressed rest and reserve right ventricular (RV) contractility compared with IPAH. We tested whether this disparity involves underlying differences in myofilament function. METHODS: Cardiac myocytes were isolated from RV septal endomyocardial biopsies from patients with SSc-PAH, IPAH, or SSc with exertional dyspnea but no resting PAH (SSc-d); control RV septal tissue was obtained from nondiseased donor hearts (6-7 per group)...
May 29, 2018: Circulation
Anish Desai, Shilpa A Desouza
Pulmonary hypertension (PH) is defined by a mean pulmonary artery pressure ≥ 25 mmHg, as determined by right heart catheterization. Pulmonary arterial hypertension (PAH) can no longer be considered an orphan disease given the increase in awareness and availability of new drugs. PH carries with it a dismal prognosis and leads to significant morbidity and mortality. Symptoms can range from dyspnea, fatigue and chest pain to right ventricular failure and death. PH is divided into five groups by the World Health Organization (WHO), based on etiology...
2017: Vascular Health and Risk Management
Ayodeji Adegunsoye, Mary E Strek
Among the interstitial lung diseases (ILDs), idiopathic pulmonary fibrosis (IPF), chronic hypersensitivity pneumonitis, and fibrotic connective tissue disease-related ILD are associated with a worse prognosis, with death occurring as a result of both respiratory failure and serious associated comorbidities. The recent development and approval of the antifibrotic agents nintedanib and pirfenidone, both of which reduced the rate of decline in lung function in patients with IPF in clinical trials, offer hope that it may be possible to alter the increased mortality associated with IPF...
December 2016: Chest
Shannon W Finks, Toby C Trujillo, Paul P Dobesh
OBJECTIVE: To review clinical data on direct oral anticoagulants (DOACs) used in the acute treatment of venous thromboembolism (VTE) as well as practical considerations when using these products. DATA SOURCES: Searches of PubMed and Google Scholar for VTE, deep vein thrombosis, pulmonary embolism, and relevant drug international nonproprietary names were conducted. Additional online searches were conducted for prescribing information. STUDY SELECTION AND DATA EXTRACTION: Relevant articles on dabigatran, rivaroxaban, apixaban, and edoxaban for the management of VTE compared with oral vitamin K antagonists (VKAs; published between 1966 and December 2015) were reviewed and summarized, together with information on dosing, pharmacokinetics/pharmacodynamics, and drug-drug interactions...
June 2016: Annals of Pharmacotherapy
Charles D Burger, Lesley D'Albini, Susan Raspa, Janis A Pruett
Prostacyclins for the treatment of pulmonary arterial hypertension (PAH) have historically been covered under the insurance medical benefit because they require durable medical equipment and are administered by an intravenous, subcutaneous, or inhalation route. However, more treatment options that target the prostacyclin pathway have become available. As the number and type of options expand, an improved understanding of these drugs will aid managed care decision makers in evaluating new treatment options and making clinically sound and cost-effective treatment decisions...
January 2016: American Journal of Managed Care
Olga Rafikova, Ruslan Rafikov, Archana Kangath, Ning Qu, Saurabh Aggarwal, Shruti Sharma, Julin Desai, Taylor Fields, Britta Ludewig, Jason X-Y Yuan, Danny Jonigk, Stephen M Black
The development of pulmonary hypertension (PH) involves the uncontrolled proliferation of pulmonary smooth muscle cells via increased growth factor receptor signaling. However, the role of epidermal growth factor receptor (EGFR) signaling is controversial, as humans with advanced PH exhibit no changes in EGFR protein levels and purpose of the present study was to determine whether there are post-translational mechanisms that enhance EGFR signaling in PH. The EGFR inhibitor, gefinitib, significantly attenuated EGFR signaling and prevented the development of PH in monocrotaline (MCT)-exposed rats, confirming the contribution of EGFR activation in MCT induced PH...
June 2016: Free Radical Biology & Medicine
Fabrice Antigny, Aurélie Hautefort, Jolyane Meloche, Milia Belacel-Ouari, Boris Manoury, Catherine Rucker-Martin, Christine Péchoux, François Potus, Valérie Nadeau, Eve Tremblay, Grégoire Ruffenach, Alice Bourgeois, Peter Dorfmüller, Sandra Breuils-Bonnet, Elie Fadel, Benoît Ranchoux, Philippe Jourdon, Barbara Girerd, David Montani, Steeve Provencher, Sébastien Bonnet, Gérald Simonneau, Marc Humbert, Frédéric Perros
BACKGROUND: Mutations in the KCNK3 gene have been identified in some patients suffering from heritable pulmonary arterial hypertension (PAH). KCNK3 encodes an outward rectifier K(+) channel, and each identified mutation leads to a loss of function. However, the pathophysiological role of potassium channel subfamily K member 3 (KCNK3) in PAH is unclear. We hypothesized that loss of function of KCNK3 is a hallmark of idiopathic and heritable PAH and contributes to dysfunction of pulmonary artery smooth muscle cells and pulmonary artery endothelial cells, leading to pulmonary artery remodeling: consequently, restoring KCNK3 function could alleviate experimental pulmonary hypertension (PH)...
April 5, 2016: Circulation
Bradley A Maron, Edward Hess, Thomas M Maddox, Alexander R Opotowsky, Ryan J Tedford, Tim Lahm, Karen E Joynt, Daniel J Kass, Thomas Stephens, Maggie A Stanislawski, Erik R Swenson, Ronald H Goldstein, Jane A Leopold, Roham T Zamanian, Jean M Elwing, Mary E Plomondon, Gary K Grunwald, Anna E Barón, John S Rumsfeld, Gaurav Choudhary
BACKGROUND: Pulmonary hypertension (PH) is associated with increased morbidity across the cardiopulmonary disease spectrum. Based primarily on expert consensus opinion, PH is defined by a mean pulmonary artery pressure (mPAP) ≥25 mm Hg. Although mPAP levels below this threshold are common among populations at risk for PH, the relevance of mPAP <25 mm Hg to clinical outcome is unknown. METHODS AND RESULTS: We analyzed retrospectively all US veterans undergoing right heart catheterization (2007-2012) in the Veterans Affairs healthcare system (n=21,727; 908-day median follow-up)...
March 29, 2016: Circulation
Marion Delcroix, Irene Lang, Joanna Pepke-Zaba, Pavel Jansa, Andrea M D'Armini, Repke Snijder, Paul Bresser, Adam Torbicki, Sören Mellemkjaer, Jerzy Lewczuk, Iveta Simkova, Joan A Barberà, Marc de Perrot, Marius M Hoeper, Sean Gaine, Rudolf Speich, Miguel A Gomez-Sanchez, Gabor Kovacs, Xavier Jaïs, David Ambroz, Carmen Treacy, Marco Morsolini, David Jenkins, Jaroslav Lindner, Philippe Dartevelle, Eckhard Mayer, Gérald Simonneau
BACKGROUND: Chronic thromboembolic pulmonary hypertension, a rare complication of acute pulmonary embolism, is characterized by fibrothrombotic obstructions of large pulmonary arteries combined with small-vessel arteriopathy. It can be cured by pulmonary endarterectomy, and can be clinically improved by medical therapy in inoperable patients. A European registry was set up in 27 centers to evaluate long-term outcome and outcome correlates in 2 distinct populations of operated and not-operated patients who have chronic thromboembolic pulmonary hypertension...
March 1, 2016: Circulation
Stephan Rosenkranz, J Simon R Gibbs, Rolf Wachter, Teresa De Marco, Anton Vonk-Noordegraaf, Jean-Luc Vachiéry
In patients with left ventricular heart failure (HF), the development of pulmonary hypertension (PH) and right ventricular (RV) dysfunction are frequent and have important impact on disease progression, morbidity, and mortality, and therefore warrant clinical attention. Pulmonary hypertension related to left heart disease (LHD) by far represents the most common form of PH, accounting for 65-80% of cases. The proper distinction between pulmonary arterial hypertension and PH-LHD may be challenging, yet it has direct therapeutic consequences...
March 21, 2016: European Heart Journal
Nazzareno Galiè, Marc Humbert, Jean-Luc Vachiery, Simon Gibbs, Irene Lang, Adam Torbicki, Gérald Simonneau, Andrew Peacock, Anton Vonk Noordegraaf, Maurice Beghetti, Ardeschir Ghofrani, Miguel Angel Gomez Sanchez, Georg Hansmann, Walter Klepetko, Patrizio Lancellotti, Marco Matucci, Theresa McDonagh, Luc A Pierard, Pedro T Trindade, Maurizio Zompatori, Marius Hoeper
No abstract text is available yet for this article.
January 1, 2016: European Heart Journal
Isabel S Bazan, Wassim H Fares
Pulmonary hypertension (PH) is a hemodynamic and pathophysiologic state that can be found in multiple conditions with associated symptoms of dyspnea, decreased exercise tolerance, and progression to right heart failure. The World Health Organization has classified PH into five groups. The first group is pulmonary arterial hypertension (PAH), which can be idiopathic, heritable, due to drugs and toxins, or associated with conditions such as connective tissue diseases, congenital heart disease, portal hypertension, and others...
2015: Therapeutics and Clinical Risk Management
George Ruiz, Gary M Besinque, Cassandra A Lickert, Susan Raspa
Pulmonary arterial hypertension (PAH) is a rare, progressive, and potentially fatal cardiopulmonary syndrome that imposes a significant burden on patients in terms of morbidity and mortality, and on managed care organizations in terms of resource utilization. The majority of PAH-approved therapies are high-touch, high-management, high-cost treatments dispensed through specialty pharmacies. Current treatment guidelines recommend combination therapy for patients who show inadequate clinical response or who deteriorate on monotherapy...
June 2015: American Journal of Managed Care
Hanno H Leuchte, Carlos Baezner, Rainer A Baumgartner, Olaf Muehling, Claus Neurohr, Juergen Behr
OBJECTIVE: Idiopathic pulmonary arterial hypertension (IPAH) remains a devastating and incurable, albeit treatable condition. Treatment response is not uniform and parameters that help to anticipate a rather benign or a malignant course of the disease are warranted. Acute pulmonary vasoreactivity testing during right heart catheterisation is recommended to identify a minority of patients with IPAH with sustained response to calcium channel blocker therapy. This study aimed to evaluate the prognostic significance of a residual pulmonary vasodilative reserve in patients with IPAH not meeting current vasoresponder criteria...
June 2015: Heart: Official Journal of the British Cardiac Society
Grégoire Le Gal, Marc Righini, Philip S Wells
No abstract text is available yet for this article.
April 28, 2015: JAMA: the Journal of the American Medical Association
Konstantinos Dimopoulos, Aleksander Kempny, Rafael Alonso-Gonzalez, Stephen J Wort
Chronic thromboembolic pulmonary hypertension (CTEPH) is a common type of pulmonary hypertension, resulting from fibrotic transformation of pulmonary artery clots causing chronic obstruction of major pulmonary arteries and associated vascular remodeling in more distal vessels. The mainstay of CTEPH treatment is pulmonary endarterectomy (PEA), which has the potential to be curative but is possible in less than two thirds of cases. In inoperable patients and those with residual or recurrent CTEPH, medical therapy has been shown to be beneficial, albeit not curative...
2015: International Journal of Cardiology
Kenneth Tan, Mohan B Krishnamurthy, Josie L O'Heney, Eldho Paul, Arvind Sehgal
UNLABELLED: Bronchopulmonary dysplasia (BPD) is associated with a high incidence of pulmonary artery hypertension (PAH) and is frequently treated with sildenafil. The objective was to investigate the echocardiographic and clinical efficacy and safety of sildenafil in this setting. The hypothesis was that treatment would result in significant echocardiographic and clinical improvements. This was a retrospective study of the cohort of infants who were born between 2004 and 2012 and administered sildenafil as in-patients for BPD-associated PAH...
August 2015: European Journal of Pediatrics
2015-03-24 16:57:51
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