Pulmonary arterial hypertension (PAH) is characterized by a persistent elevation of pulmonary arterial pressure and pulmonary arterial remodeling with unknown etiology. Current therapeutics available for PAH are primarily directed at reducing the pulmonary blood pressure through their effects on the endothelium. It is well accepted that pulmonary arterial remodeling is primarily due to excessiv...

INTRODUCTION Patients with mean pulmonary artery pressures (mPAP) of 21 to 24 mm Hg have a so-called borderline elevation of mPAP (BoPAP)--a condition thought to represent early-stage pulmonary arterial vasculopathy. Based on the DETECT study, this post-hoc analysis examined patient characteristics of systemic sclerosis (SSc) patients with normal mPAP, BoPAP and elevated mPAP, fulfilling pulmon...

AIMS To assess the occurrence and prognostic value of acute vasodilator response (AVR) in paediatric vs. adult pulmonary arterial hypertension, and idiopathic/hereditary pulmonary arterial hypertension (iPAH/HPAH) vs. pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) using three different response criteria. METHODS AND RESULTS Ninety-nine PAH patients underwen...

Pulmonary arterial hypertension (PAH) is a rare disease but PAH treatment cost is very high. Nowadays, there has been no cost-effectiveness information related to PAH treatments in Thailand. The aim of this study was to systematically review economic evaluation studies of drugs treatment in PAH. Studies reporting in term of clinical outcomes and costs during January 1990 to October 2012 were se...

objective(s): the present study was aimed to investigate the influence of thoracic epidural blockade on hypoxia-induced pulmonary hypertension in rats. materials and methods: forty eight wistar rats were randomly divided into 4 equal groups, named normoxia hypoxia hypoxia/ ropivacaine and hypoxia/saline. animals were placed in a hypoxia chamber and instrumented with epidural catheters at the th...

Fernando Torres, MD Head of the Lung Transplant and Pulmonary Hypertension Programs UT Southwestern Medical Center Dallas, TX During the Fifth World Symposium on Pulmonary Hypertension, the working group on diagnosis and assessment was charged with evaluating the definition of pulmonary arterial hypertension (PAH) as it was established at the Fourth World Symposium. The group also covered relat...

BACKGROUND Inflammatory processes seem to be involved in pulmonary arterial hypertension (PAH). CD40 ligand (L) may promote inflammation and thrombus formation, and we hypothesized that CD40L could be involved in the pathogenesis of PAH. METHODS AND RESULTS Several significant findings were revealed when examining the possible role of CD40L in PAH. (1) Patients with primary (n=13) and seconda...

Pulmonary hypertension (PH) is found in a vast array of diseases, with a minority representing pulmonary arterial hypertension (PAH). Idiopathic PAH or PAH in association with other disorders has been associated with poor survival, poor exercise tolerance, progressive symptoms of dyspnea, and decreased quality of life. Left untreated, patients with PAH typically have a progressive decline in fu...

PAPER Zhongming Zhao et al. An evidence-based knowledgebase of pulmonary arterial hypertension to identify genes and pathways relevant to pathogenesis aefg Pulmonary arterial hypertension (PAH) is a major progressive form of pulmonary hypertension (PH) with more than 4800 patients in the United States. In the last two decades, many studies have identified numerous genes associated with this dis...

Background—Incidence and prevalence rates for pediatric pulmonary hypertension (PH) and pulmonary arterial hypertension (PAH) are unknown. This study describes the nationwide epidemiological features of pediatric PH in the Netherlands during a 15-year period and the clinical course of pediatric PAH. Methods and Results—Two registries were used to retrospectively identify children (0–17 years) w...