HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:
Guest Access | Sign In via User Name/Password

This Article Full Text (PDF) Free Submit a response Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to My Personal Article Archive Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Hughes, R. Articles by Corris, P.A. Search for Related Content PubMed PubMed Citation Articles by Hughes, R. Articles by Corris, P.A.

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

^* 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{at}ncl.ac.uk

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.

	Footnotes

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

This Article Full Text (PDF) Free Submit a response Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to My Personal Article Archive Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Hughes, R. Articles by Corris, P.A. Search for Related Content PubMed PubMed Citation Articles by Hughes, R. Articles by Corris, P.A.