BACKGROUND The clinical relevance of exercise-induced pulmonary arterial hypertension (PAH) is uncertain, and its existence has never been well studied by direct measurements of central hemodynamics. Using invasive cardiopulmonary exercise testing, we hypothesized that exercise-induced PAH represents a symptomatic stage of PAH, physiologically intermediate between resting pulmonary arterial hyp...

The Third World Symposium on Pulmonary Arterial Hypertension served not only as a forum for the presentation of state-of-the art overviews of the pathobiologic and clinical aspects of pulmonary arterial hypertension (PAH), but also afforded an opportunity to the international scientific community to explore future directions of research and collaboration. This summary provides a brief overview ...

INTRODUCTION Pulmonary arterial hypertension (PAH), characterized by increased pulmonary artery pressures in the absence of elevated pulmonary venous pressures, is a progressive disease associated with reduced exercise capacity and increased mortality risk. Current treatments for PAH include nonspecific vasodilators, prostacyclin and related analogs, and endothelin receptor antagonists. Since p...

The respective abundance of circulating endothelial cells and endothelial progenitor cells may reflect the balance between vascular injury and repair. As pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) can share features of pulmonary remodelling, we postulated that the two disorders might be associated with different types of pulmonary endothelial...

BACKGROUND To study the oxygen uptake efficiency and determine usefulness of submaximal parameters of oxygen uptake in systemic lupus erythematosus associated pulmonary arterial hypertension (SLE PAH) on performing a cardiopulmonary exercise test (CPET). METHODS CPET was performed in 21 SLE PAH patients, equal number of idiopathic pulmonary arterial hypertension (IPAH) patients and controls. ...

Pulmonary arteriovenous fistulas (PAVFs) are rare. Diffuse type PAVFs with pulmonary arterial hypertension (PAH) are even rarer and can elude anatomy imaging like a plain chest film or a computed tomography. The rapid blood flow that ensues due to lack of a capillary bed leads to various degrees of ischemia depending on the number and size of the PAVF. This is a case report of diffuse PAVF in a...

Pulmonary arterial hypertension (PAH) is a rare but serious clinical condition characterized by a progressive increase of pulmonary arterial pressure and resistance leading to right ventricular and premature death.1) Although PAH clinically includes 9 different subgroups, it has a common final pathology, which is obstructive thickening/hypertrophy in the vascular wall components, mainly affecti...

Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by persistent thromboemboli in the pulmonary arteries, which cause pulmonary hypertension and result in right heart failure and death [1]. CTEPH occurs in 2–4% of patients with acute pulmonary embolism (PE) [2], indicating a pathological relationship between the two diseases. Several common risk factors for CTEPH and PE have...

Pulmonary arterial hypertension (PAH) is a condition that is characterised by increased pulmonary arterial pressure and vascular resistance that can lead to right ventricular failure and death. A variety of disturbances in pulmonary vascular endothelial and smooth muscle function are present in PAH, including reduced production of vasodilator and antiproliferative substances, such as nitric oxi...

Pulmonary arterial hypertension (PAH) is still a fatal disease, affecting patients of all ages including children [1]. In the last 20 years, significant progress has been achieved in the understanding of the disease, and in particular, in the medical approach, with the introduction of new therapies. Goal-oriented strategy represents a significant progress in PAH management and has emerged as th...