Michael Madani, Takeshi Ogo, Gérald Simonneau
For patients with chronic thromboembolic pulmonary hypertension (CTEPH), the current standard of care involves surgical removal of fibro-thrombotic obstructions by pulmonary endarterectomy. While this approach has excellent outcomes, significant proportions of patients are not eligible for surgery or suffer from persistent/recurrent pulmonary hypertension after the procedure. The availability of balloon pulmonary angioplasty and the approval of the first medical therapy for use in CTEPH have significantly improved the outlook for patients ineligible for pulmonary endarterectomy...
December 31, 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
Marco Guazzi, Robert Naeije
Pulmonary hypertension is a common hemodynamic complication of heart failure. Interest in left-sided pulmonary hypertension has increased remarkably in recent years because its development and consequences for the right heart are now seen as mainstay abnormalities that begin in the early stages of the disease and bear unfavorable prognostic insights. However, some knowledge gaps limit our ability to influence this complex condition. Accordingly, attention is now focused on: 1) establishing a definitive consensus for a hemodynamic definition, perhaps incorporating exercise and fluid challenge; 2) implementing the limited data available on the pathobiology of lung capillaries and small arteries; 3) developing standard methods for assessing right ventricular function and, hopefully, its coupling to pulmonary circulation; and 4) searching for effective therapies that may benefit lung vessels and the remodeled right ventricle...
April 4, 2017: Journal of the American College of Cardiology
Vallerie V McLaughlin, Sanjiv J Shah, Rogerio Souza, Marc Humbert
Pulmonary hypertension (PH) is common and may result from a number of disorders, including left heart disease, lung disease, and chronic thromboembolic disease. Pulmonary arterial hypertension (PAH) is an uncommon disease characterized by progressive remodeling of the distal pulmonary arteries, resulting in elevated pulmonary vascular resistance and, eventually, in right ventricular failure. Over the past decades, knowledge of the basic pathobiology of PAH and its natural history, prognostic indicators, and therapeutic options has exploded...
May 12, 2015: Journal of the American College of Cardiology
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
Susan R Wilcox, Christopher Kabrhel, Richard N Channick
Pulmonary hypertension is a hemodynamic condition, defined as a mean pulmonary artery pressure by right-sided heart catheterization of at least 25 mm Hg at rest. It is classified into 5 general groups based on the underlying cause, with left ventricular failure and chronic obstructive pulmonary disease being 2 of the most common causes in the United States. Although the specifics of the pathophysiology will vary with the cause, appreciating the risks of pulmonary hypertension and right ventricular failure is critical to appropriately evaluating and resuscitating pulmonary hypertension patients in the emergency department (ED)...
December 2015: Annals of Emergency Medicine
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
Guidelines summarize and evaluate all available evidence on a particular issue at the time of the writing process, with the aim of assisting health professionals in selecting the best management strategies for an individual patient with a given condition, taking into account the impact on outcome, as well as the risk-benefit ratio of particular diagnostic or therapeutic means. Guidelines and recommendations should help health professionals to make decisions in their daily practice. However, the final decisions concerning an individual patient must be made by the responsible health professional(s) in consultation with the patient and caregiver as appropriate...
October 2015: European Respiratory 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
Marc Humbert, Hossein-Ardeschir Ghofrani
Until recently, three classes of medical therapy were available for the treatment of pulmonary arterial hypertension (PAH)--prostanoids, endothelin receptor antagonists and phosphodiesterase type 5 (PDE5) inhibitors. With the approval of the soluble guanylate cyclase stimulator riociguat, an additional drug class has become available targeting a distinct molecular target in the same pathway as PDE5 inhibitors. Treatment recommendations currently include the use of all four drug classes to treat PAH, but there is a lack of comparative data for these therapies...
January 2016: Thorax
Vallerie McLaughlin, Richard N Channick, Hossein-Ardeschir Ghofrani, Jean-Christophe Lemarié, Robert Naeije, Milton Packer, Rogério Souza, Victor F Tapson, Jonathan Tolson, Hikmet Al Hiti, Gisela Meyer, Marius M Hoeper
The safety and efficacy of adding bosentan to sildenafil in pulmonary arterial hypertension (PAH) patients was investigated.In this prospective, double-blind, event-driven trial, symptomatic PAH patients receiving stable sildenafil (≥20 mg three times daily) for ≥3 months were randomised (1:1) to placebo or bosentan (125 mg twice daily). The composite primary end-point was the time to the first morbidity/mortality event, defined as all-cause death, hospitalisation for PAH worsening or intravenous prostanoid initiation, atrial septostomy, lung transplant, or PAH worsening...
August 2015: European Respiratory Journal
Irene Marthe Lang, Michael Madani
No abstract text is available yet for this article.
August 5, 2014: Circulation
Luke S Howard, Pisana Ferrari, Sanjay Mehta
In recent years, many new, effective therapies for pulmonary arterial hypertension (PAH) have become available and are widely used, yet the long-term prognosis for patients with PAH remains poor. In the absence of a cure, physicians' expectations of PAH-specific therapies are to: 1) improve patients' symptoms and functional capacity; 2) slow disease progression; and 3) improve survival. However, patients with PAH may prioritise other more tangible needs, such as improvements in their ability to carry out their daily tasks and increase their quality of life...
December 2014: European Respiratory Review: An Official Journal of the European Respiratory Society
Hossein-Ardeschir Ghofrani, Marc Humbert
Pulmonary arterial hypertension (PAH) is a complex, progressive disease with several pathobiological mechanisms, including the endothelin, nitric oxide and prostacyclin pathways. Current treatments for PAH target one of these pathways and, in more severe cases or instances of disease worsening, may be combined with a view to target multiple pathways in parallel. Treatment combination is performed sequentially (as an intensification from initial monotherapy) or upfront (use of two or more therapies in treatment-naïve patients)...
December 2014: European Respiratory Review: An Official Journal of the European Respiratory Society
Robert Naeije, Alessandra Manes
Pulmonary arterial hypertension (PAH) is a right heart failure syndrome. In early-stage PAH, the right ventricle tends to remain adapted to afterload with increased contractility and little or no increase in right heart chamber dimensions. However, less than optimal right ventricular (RV)-arterial coupling may already cause a decreased aerobic exercise capacity by limiting maximum cardiac output. In more advanced stages, RV systolic function cannot remain matched to afterload and dilatation of the right heart chamber progressively develops...
December 2014: European Respiratory Review: An Official Journal of the European Respiratory Society
Olivier Sitbon, Laurent Bertoletti
No abstract text is available yet for this article.
December 2014: European Respiratory Review: An Official Journal of the European Respiratory Society
Yochai Adir, Sergio Harari
PURPOSE OF REVIEW: Severe pulmonary hypertension worsens the prognosis of patients with chronic obstructive pulmonary disease (COPD) or idiopathic pulmonary fibrosis (IPF). With the aim of better understanding the pathogenesis of this event and identifying the possible targets for therapeutic intervention, a great deal of clinical and translational research is now focused on this relevant field of medicine. RECENT FINDINGS: Some studies that were published last year have helped to better define the clinical and physiological profiles of patients with COPD or IPF and severe pulmonary hypertension...
September 2014: Current Opinion in Pulmonary Medicine
Marc Humbert
No abstract text is available yet for this article.
October 2013: Seminars in Respiratory and Critical Care Medicine
Christophe Guignabert, Peter Dorfmuller
Pulmonary hypertension is a devastating, life-threatening disorder with no curative options, characterized by elevated pulmonary vascular resistance and secondary right ventricular failure. Although the etiologies of pulmonary arterial hypertension are multiple and its pathogenesis is complex, there is growing evidence that inflammation, endothelial dysfunction, aberrant vascular wall cell proliferation, as well as mutations in the bone morphogenetic protein receptor type 2 gene play a crucial role in triggering pathological vascular remodeling...
October 2013: Seminars in Respiratory and Critical Care Medicine
Eric D Austin, James E Loyd
Tremendous progress has been made in understanding the genetics of heritable pulmonary arterial hypertension (HPAH) since its description in the 1950s. Germline mutations in the gene coding bone morphogenetic receptor type 2 (BMPR2) are detectable in the majority of cases of HPAH, and in a small proportion of cases of idiopathic pulmonary arterial hypertension (IPAH). Recent advancements in gene sequencing methods have facilitated the discovery of additional genes with mutations among those with and without familial PAH (CAV1, KCNK3)...
October 2013: Seminars in Respiratory and Critical Care Medicine
Yi Ling, Martin K Johnson, David G Kiely, Robin Condliffe, Charlie A Elliot, J Simon R Gibbs, Luke S Howard, Joanna Pepke-Zaba, Karen K K Sheares, Paul A Corris, Andrew J Fisher, James L Lordan, Sean Gaine, J Gerry Coghlan, S John Wort, Michael A Gatzoulis, Andrew J Peacock
RATIONALE: Incident pulmonary arterial hypertension was underrepresented in most pulmonary hypertension registries and may have a different disease profile to prevalent disease. OBJECTIVES: To determine the characteristics and outcome of a purely incident, treatment-naive cohort of idiopathic, heritable, and anorexigen-associated pulmonary arterial hypertension and to determine the changes in presentations and survival over the past decade in the United Kingdom and Ireland...
October 15, 2012: American Journal of Respiratory and Critical Care Medicine
2014-08-30 20:37:30
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