E ndothelin receptor antagonists, phosphodiesterase (PDE)-5 inhibitors and prostanoids are effective treatments for pulmonary arterial hypertension (PAH), a severe but relatively rare form of pulmonary hypertension [1–3]. Other forms of pulmonary hypertension as seen, for instance, in chronic lung disease are much more common than PAH and although usually less severe, they are clinically meanin...

Major limitations of pulmonary arterial hypertension (PAH) drug trials include the small number of enrolled patients, short term follow up (12-16 weeks), and lack of morbidity and mortality primary endpoints. The recently published SERAPHIN (Study with an Endothelin Receptor Antagonist in Pulmonary arterial Hypertension to Improve cliNical outcome) trial represents an important landmark in the ...

BACKGROUND Pulmonary arterial hypertension (PAH) is a life-threatening disease, characterized by vascular smooth muscle cell hyperproliferation. The calcium/calmodulin-dependent phosphodiesterase 1 (PDE1) may play a major role in vascular smooth muscle cell proliferation. METHODS AND RESULTS We investigated the expression of PDE1 in explanted lungs from idiopathic PAH patients and animal mode...

In left heart failure, iron supplementation (IS) is a first-line treatment option, regardless of anemia. Pulmonary arterial hypertension (PAH), rare disease leading to right also associated with deficiency. While it much debated topic, recent evidence demonstrate that restoration stores results in improved ventricular function and exercise tolerance. Hence, IS may be considered as an option the...

Pulmonary arterial hypertension (PAH) and ANCA-associated vasculitis (AAV) are both rare and complex diseases with poor prognosis especially when misdiagnosis. We report a rare case of a young woman presented with idiopathic pulmonary arterial hypertension (IPAH), but who was later found to have PAH associated with AAV. This case reminds us the very importance of differential diagnosis to PAH p...

Pulmonary arterial hypertension (PAH) is a term used to classify a variety of conditions that have in common an injury to the pulmonary vasculature that produces elevations in pulmonary arterial pressure. There have been considerable advances in our understanding of the pathogenesis and treatment of PAH over the past decade. The article reviews the classification of diseases associated with PAH...

conclusions the results of the first human pharmacokinetic studies of aminorex were first characterized in 2013. the results suggest that while humans could produce aminorex from levamisole, they probably do not convert very much. even though cases of pah have been reported in cocaine users, the latest pharmacokinetic studies suggest that very little aminorex is actually produced from the inges...

Surgical and interventional therapies for pulmonary arterial hypertension (PAH) in appropriately selected patients have the potential to dramatically improve or, in some cases, cure PAH. These include atrial septostomy, a palliative procedure or bridge to transplantation in patients with refractory right heart failure, pulmonary thromboendarterectomy for pulmonary hypertension associated with c...

The treatment of pulmonary arterial hypertension (PAH) is directed at the underlying cause, such as diastolic heart failure or chronic thromboembolic disease. Patients with idiopathic PAH or PAH associated with connective-tissue disease who have World Health Organization (WHO) functional class II or III PAH should receive a trial of oral bosentan, ambrisentan, and/or sildenafil; inhaled ilopros...

INTRODUCTION Pulmonary arterial hypertension (PAH) is a disease of the distal pulmonary arteries that results in an elevation in pulmonary arterial resistance and pressure [1]. Currently, right heart catheterization is the gold standard for assesment of PAH. The relative cross sectional area change (RAC) of the main pulmonary artery may be a useful non-invasive measure of PAH severity [2]. Here...