Patients affected by pulmonary arterial hypertension (PAH) show a typical pattern of abnormalities on cardiopulmonary exercise testing (CPET). However, CPET is not routinely used as a screening method. We discuss a patient with hereditary PAH in whom CPET revealed onset of disease. Furthermore, we show that the abnormalities observed can improve in part by PAH-specific treatment.

Pulmonary arterial hypertension (PAH) is a rare and severe condition that may present as familial/heritable disease [1–3]. In recent years, there have been considerable advances in the management of PAH and disease-specific therapies have improved survival rates [3–5]. Nevertheless, PAH remains a devastating disease with progressively debilitating symptoms and high mortality even in the modern ...

Background Pulmonary arterial hypertension (PAH) is a potentially severe disease that can lead to exercise intolerance, venous congestion, and heart failure. As a result of increased pulmonary pressures, dilation of the pulmonary arterial vasculature occurs. The extraction of these anatomical changes from magnetic resonance angiogram (MRA) may eliminate the need for invasive measurements. The a...

1361 Pulmonary hypertension is broadly recognized as a group of disorders that share the common characteristic of an elevated mean pulmonary arterial pressure (≥25 mm Hg). Pulmonary arterial hypertension (PAH; Group 1) is a subset of the pulmonary hypertension disorders that is also characterized by pulmonary arterial remodeling, the formation of plexiform lesions, and poor clinical outcomes. W...

The case history is presented of a male infant who was thought to have idiopathic pulmonary arterial hypertension (PAH) at 3 months of age. Subsequently the PAH decreased unexpectedly and diffuse pulmonary arteriovenous malformations (PAVMs) were seen at 6.9 years of age for the first time. Hereditary haemorrhagic telangiectasia type 1 (HHT1) related to an endoglin mutation was diagnosed. At 10...

Pulmonary arterial hypertension (PAH) is an incurable disease characterized by a progressive increase in pulmonary vascular resistance leading to right heart failure. Carbon monoxide (CO) has emerged as a potently protective, homeostatic molecule that prevents the development of vascular disorders when administered prophylactically. The data presented in this paper demonstrate that CO can also ...

Martin YN, Pabelick CM. Sex differences in the pulmonary circulation: implications for pulmonary hypertension. Am J Physiol Heart Circ Physiol 306: H1253–H1264, 2014. First published March 7, 2014; doi:10.1152/ajpheart.00857.2013.—Pulmonary arterial hypertension (PAH), a form of pulmonary hypertension, is a complex disease of multifactorial origin. While new developments regarding pathophysiolo...

Pulmonary arterial hypertension (PAH) is a chronic, progressive, and debilitating disease associated with increased cardiopulmonary morbidity mortality. Current knowledge derived from registries landmark clinical trials, although concern exists that minorities are underrepresented in these datasets, it remains unknown if there intrinsic differences disadvantaged groups. The Hypertension Associa...

Pulmonary arterial hypertension (PAH) contributes to morbidity and mortality of patients with lung and heart diseases. We demonstrated that hypoxia induced PAH and increased pulmonary arterial wall thickness in wild-type mice. Mice deficient in toll-like receptor 4 (TLR4-/-) spontaneously developed PAH, which was not further enhanced by hypoxia. Echocardiography determined right ventricular hyp...

Pulmonary arterial hypertension (PAH) is a heterogeneous condition with a wide range of causes. The diagnosis is often delayed or missed. PAH is covert in its early stages, when its detection and treatment should have the most impact. Access in Australia to effective PAH therapies has lagged behind that in other affluent countries. New agents for PAH, now becoming available, improve symptoms an...