Pulmonary arterial hypertension: an update
TLDR
Treatment includes general measures and drugs targeting the pulmonary artery tone and vascular remodelling, which has significantly improved morbidity and mortality in patients with PAH in the last decade.Abstract:
Pulmonary arterial hypertension (PAH), defined as group 1 of the World Heart Organisation (WHO) classification of pulmonary hypertension, is an uncommon disorder of the pulmonary vascular system. It is characterised by an increased pulmonary artery pressure, increased pulmonary vascular resistance and specific histological changes. It is a progressive disease finally resulting in right heart failure and premature death. Typical symptoms are dyspnoea at exercise, chest pain and syncope; furthermore clinical signs of right heart failure develop with disease progression. Echocardiography is the key investigation when pulmonary hypertension is suspected, but a reliable diagnosis of PAH and associated conditions requires an intense work-up including invasive measurement by right heart catheterisation. Treatment includes general measures and drugs targeting the pulmonary artery tone and vascular remodelling. This advanced medical therapy has significantly improved morbidity and mortality in patients with PAH in the last decade. Combinations of these drugs are indicated when treatment goals of disease stabilisation are not met. In patients refractory to medical therapy lung transplantation should be considered an option.read more
Citations
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Chronic thromboembolic pulmonary hypertension
Marius M. Hoeper,Michael M. Madani,Norifumi Nakanishi,Bernhard C. Meyer,Serghei Cebotari,Lewis J. Rubin +5 more
TL;DR: Surgical pulmonary endarterectomy remains the treatment of choice for CTEPH and is associated with excellent long-term results and a high probability of cure, and various medical and interventional therapies are being developed.
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Pulmonary Hypertension in Pregnancy: Critical Care Management
Adel Bassily-Marcus,Carol Yuan,John Oropello,Anthony Manasia,Roopa Kohli-Seth,Ernest Benjamin +5 more
TL;DR: Pulmonary-hypertension-targeted therapies have been utilized in pregnant women with understanding of their safety profile, and mainstay therapy for pulmonary emblism is anticoagulation, and the treatment for amniotic fluid embolism remains supportive care.
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Considerations for numerical modeling of the pulmonary circulation-a review with a focus on pulmonary hypertension
TL;DR: A clinical overview of pulmonary hypertension is provided with a focus on the hemodynamics, current treatments, and their limitations, and the tasks involved in developing a computational model of pulmonary blood flow, namely vasculature reconstruction, meshing, and boundary conditions are reviewed.
Journal ArticleDOI
Pulmonary hypertension complicating pulmonary sarcoidosis.
TL;DR: The diagnostic work-up and treatment of PH in sarcoidosis is complex, and should therefore be determined by a multidisciplinary expert team in a specialised centre.
Journal ArticleDOI
Chronic thromboembolic pulmonary hypertension.
Bastiaan E. Schölzel,Repke J. Snijder,Johannes J. Mager,H. W. van Es,H. W. M. Plokker,Herre J. Reesink,Wim J. Morshuis,Martijn C. Post +7 more
TL;DR: A substantial number of patients are not candidates for pulmonary endarterectomy due to either distal pulmonary vascular obstruction or significant comorbidities, therefore, careful selection of surgical candidates in expert centres is paramount.
References
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Journal ArticleDOI
Guidelines for the diagnosis and treatment of pulmonary hypertension: the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT)
Nazzareno Galiè,Marius M. Hoeper,Marc Humbert,Adam Torbicki,Jean-Luc Vachiery,J.A. Barberà,Maurice Beghetti,Paul A. Corris,Sean Gaine,J S R Gibbs,Miguel Ángel Gómez-Sánchez,Guillaume Jondeau,Walter Klepetko,Christian Opitz,Andrew J. Peacock,LJ Rubin,Michael J. Zellweger,Gérald Simonneau +17 more
TL;DR: In this paper, a review of the published evidence for management and/or prevention of a given condition is carried out by experts in the field and a critical evaluation of diagnostic and therapeutic procedures is performed including assessment of the risk/benefit ratio.
Journal ArticleDOI
A Comparison of Continuous Intravenous Epoprostenol (Prostacyclin) with Conventional Therapy for Primary Pulmonary Hypertension
Robyn J. Barst,Lewis J. Rubin,Walker Long,Michael D. McGoon,Stuart Rich,David B. Badesch,Bertron M. Groves,Victor F. Tapson,Robert C. Bourge,Bruce H. Brundage,Spencer K. Koerner,David Langleben,Cesar A. Keller,Srinivas Murali,Barry F. Uretsky,Linda M. Clayton,Maria M. Jöbsis,Shelmer D. Blackburn,Denise Shortino,James W. Crow +19 more
TL;DR: As compared with conventional therapy, the continuous intravenous infusion of epoprostenol produced symptomatic and hemodynamic improvement, as well as improved survival in patients with severe primary pulmonary hypertension.
Journal ArticleDOI
Sildenafil citrate therapy for pulmonary arterial hypertension.
Nazzareno Galiè,Hossein Ardeschir Ghofrani,Adam Torbicki,Robyn J. Barst,Lewis J. Rubin,David B. Badesch,Thomas R. Fleming,Tamiza Parpia,Gary Burgess,Angelo Branzi,Friedrich Grimminger,Marcin Kurzyna,Gérald Simonneau +12 more
TL;DR: Among the 222 patients completing one year of treatment with sildenafil monotherapy, the improvement from baseline at one year in the distance walked in six minutes was 51 m, and the incidence of clinical worsening did not differ significantly between the patients treated with s Bildenafils and those treated with placebo.
Journal ArticleDOI
Pulmonary arterial hypertension in France: results from a national registry.
Marc Humbert,Olivier Sitbon,Ari Chaouat,Michèle Bertocchi,Gilbert Habib,Virginie Gressin,Azzedine Yaici,Emmanuel Weitzenblum,Jean-François Cordier,François Chabot,Claire Dromer,Christophe Pison,Martine Reynaud-Gaubert,Alain Haloun,Marcel Laurent,Eric Hachulla,Gérald Simonneau +16 more
TL;DR: It is shown that PAH is detected late in the course of the disease, with a majority of patients displaying severe functional and hemodynamic compromise, and this contemporary registry highlights current practice.
Journal ArticleDOI
Inhaled iloprost for severe pulmonary hypertension.
Horst Olschewski,Gérald Simonneau,Nazzareno Galiè,T. Higenbottam,Robert Naeije,Lewis J. Rubin,Sylvia Nikkho,Rudolf Speich,Marius M. Hoeper,Jürgen Behr,Jörg Winkler,Olivier Sitbon,Wladimir Popov,H. Ardeschir Ghofrani,Alessandra Manes,David G. Kiely,R Ewert,Andreas Meyer,Paul A. Corris,Marion Delcroix,Miguel Ángel Gómez-Sánchez,Harald Siedentop,Werner Seeger +22 more
TL;DR: Inhaled Iloprost is an effective therapy for patients with severe pulmonary hypertension and hemodynamic values were significantly improved at 12 weeks when measured after iloprost inhalation, and were significantly worse in the placebo group.
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