Articles |

Evidence for Systemic Endothelial Dysfunction in Patients and First-Order Relatives With Pulmonary Arterial Hypertension* FREE TO VIEW

R. Hughes; J. Tong; C. Oates; J. Lordan; P.A. Corris
Author and Funding Information

*From the Regional Cardiothoracic Centre and Regional Medical Physics Department, Freeman Hospital, Freeman Rd, Newcastle upon Tyne, England, UK.

Correspondence to: Paul A. Corris, Regional Cardiothoracic Centre and Regional Medical Physics Department, Freeman Hospital, Freeman Rd, Newcastle upon Tyne, England, UK, NE7 7DN; e-mail: paul.corris@ncl.ac.uk

Chest. 2005;128(6_suppl):617S. doi:10.1378/chest.128.6_suppl.617S
Text Size: A A A
Published online

Dysfunction of pulmonary endothelial cells plays an important role in the pathogenesis of pulmonary arterial hypertension (PAH). We were interested in studying patients with idiopathic PAH, their relatives, and patients with PAH secondary to systemic sclerosis to see whether there was evidence of systemic endothelial cell function. We chose to study both nitric oxide (NO)-dependent and NO-independent mechanisms by studying brachial artery hyperemia flow-mediated vasodilation following occlusion (NO-dependent) and response to sublingual glyceryl trinitrate (GTN) [NO-independent]. We studied 10 control subjects, 10 patients with PAH due to idiopathic pulmonary hypertension, 10 of their relatives, and 10 patients with PAH due to systemic sclerosis. Brachial artery dilation was measured using a 12-MHz ultrasound probe. Brachial arterial dilation was measured 60 s after the release of the occluding cuff and 3 min after sublingual GTN administration. There were significant reductions in brachial artery dilation seen in patients with idiopathic PAH and in those with systemic sclerosis compared to control subjects (2.7% vs 6.3%, respectively [p < 0.05]; and 0.7% vs 6.3%, respectively [p < 0.05]). Most interesting was the observation that there was a trend toward a reduced response in family members of the idiopathic PAH patients (2.7% vs 6.3%, respectively). Three family members showed a particularly reduced response.

No significant difference between response to GTN were seen in either patient groups or family members compared to controls. We are proceeding to genotyping the patients and family members For BMPR II mutations. We describe evidence in support of systemic endothelial dysfunction in patients with PAH and their family members.

Abbreviations: GTN = glyceryl trinitrate; NO = nitric oxide; PAH = pulmonary arterial hypertension




Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s "Cited By" API will populate this tab (http://www.crossref.org/citedby.html).

Some tools below are only available to our subscribers or users with an online account.

Related Content

Customize your page view by dragging & repositioning the boxes below.

CHEST Journal Articles
PubMed Articles
  • CHEST Journal
    Print ISSN: 0012-3692
    Online ISSN: 1931-3543