tery pressure response to exercise and hypoxia, an early indicator of altered pulmonary vascular function [2] . The pathogenesis of PAH is complex and involves a variety of cellular and molecular mechanisms, including the serotoninand nitric oxide system. Therefore, genetic modifiers have been suggested to be involved in the development of PAH [3] . Are genetic changes relevant in our current c...

This study explores the racial and ethnic differences in presentation, severity, and treatment of patients with pulmonary arterial hypertension (PAH) in a large multicenter registry. African American and Hispanic patients are more likely to present with associated PAH compared to non-Hispanic whites. Hispanic patients with PAH were less likely to be treated with PAH-specific medications compare...

PurposeOur prior findings demonstrated the additive value of echocardiographic parameters - specifically left ventricle end diastolic (LV ED) eccentricity index to REVEAL lite 2.0 in early prediction disease progression pulmonary arterial hypertension (PAH). The latest 2022 ESC/ERS PH guidelines suggested use Tricuspid Annular Plane Systolic Excursion Pulmonary Arterial Pressure (TAPSE/sPAP) as...

In chronic thromboembolic pulmonary hypertension (CTEPH) increased pulmonary vascular resistance is caused by fibrotic organisation of unresolved thromboemboli. CTEPH mainly differs from pulmonary arterial hypertension (PAH) by the proximal location of pulmonary artery obliteration, although distal arteriopathy can be observed as a consequence of non-occluded area over-perfusion. Accordingly, t...

Pulmonary arterial hypertension (PAH) is a progressive disease with poor prognosis if left untreated. Despite remarkable achievements in understanding pathophysiology, specific treatments, and therapeutic strategies, we are still far from definitive cure for the disease, numerous evidences have underlined importance of early diagnosis treatment to improve prognosis. Cardiopulmonary exercise tes...

P ulmonary arterial hypertension (PAH) is a progressive and devastating condition that is characterized by pulmonary vascular remodeling resulting in increased pulmonary vascular resistance and raised pulmonary arterial pressure, eventually leading to right ventricular (RV) failure and premature death. 1,2 PAH carries a poor prognosis. Even in the current treatment era, the average life expecta...

We read with interest the report by MONTANI et al. [1] of fatal rupture of pulmonary arteriovenous malformation (AVM) in a patient with hereditary haemorrhagic telangiectasis (HHT) and severe pulmonary arterial hypertension (PAH) confirmed by right heart catheterisation. The authors hypothesised that bleeding from pulmonary AVM may have been facilitated by increased mean pulmonary artery pressu...

Pulmonary arterial hypertension (PAH) is a rare and devastating disease characterized by progressive increases in pulmonary arterial pressure and pulmonary vascular resistance which eventually leads to right ventricular failure and death. Early thought process was that exercise and increased physical activity may be detrimental to PAH patients however many small cohort trials have proven otherw...

www.chestpubs.org CHEST / 141 / 4 / APRIL, 2012 923 P arterial hypertension (PAH) is a lifethreatening disease that may occur either in idiopathic form or in the setting of different associated medical conditions. PAH is characterized by a marked and sustained elevation of pulmonary vascular resistance, leading to an increase in pulmonary artery pressure, right ventricular failure, and ultimate...

Pulmonary arterial hypertension (PAH) is a specific, rare disease characterized by a well-described pattern of pulmonary vascular remodeling. The elevated pulmonary artery pressure in PAH results in increased right ventricular afterload, which, if untreated, leads rapidly to right ventricular failure and death. Recent marked expansion in knowledge about PAH has resulted in the development of ef...