Copyright © 2005, The European Society of Cardiology
Abnormal pulmonary vascular responses in patients registered with a systemic autoimmunity database: Pulmonary Hypertension Assessment and Screening Evaluation using stress echocardiography (PHASE-I)
aCardiovascular Unit, John Hunter Hospital, Lookout Road, New Lambton, Newcastle, NSW 2305, Australia
bHunter Area Pathology Service, John Hunter Hospital, Newcastle, Australia
Received 30 June 2005; received in revised form 20 November 2005; accepted after revision 4 December 2005.
* Corresponding author. C/-11 Orchard Road, Beecroft, NSW 2119, Australia. Tel.: +1 416 726 9452. nandl_collins{at}yahoo.com.au
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Abstract |
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Patients with autoimmune disease, and in particular limited systemic sclerosis (CREST syndrome), are at risk of developing pulmonary artery hypertension (PAH) which is associated with a poor prognosis. With improvements in therapy offering improved survival and functional capacity, there has been an emphasis on screening to identify patients at risk. Assessment of patients during exercise may enable early identification of patients with this condition.
Aims and methods We aimed to assess the ability of exercise stress echocardiography to evaluate the change in pulmonary artery pressure in 51 patients with autoimmune disease (systemic lupus erythamatosus (SLE), limited systemic sclerosis (LSS or "CREST") and diffuse systemic sclerosis (DSS)). Systolic pulmonary artery pressure (sPAP) was estimated using interrogation of the tricuspid incompetence jet before and after exercise. PAH was classified as normal, mild, moderate or severe using echocardiographic assessment of sPAP.
Results We were able to estimate pre-exercise and post-exercise sPAP in 92% and 90% of patients, respectively.
Pulmonary pressures rose or remained unchanged in all screened individuals, with a mean rise during stress of 14.1mmHg (±1.1). Pulmonary artery pressure rose significantly in each of three subgroups (p<0.05).
Stress echocardiography demonstrated PAH (using a cut-off of >35mmHg) in 59% of all individuals with systemic autoimmunity.
Conclusion Stress echocardiography is a useful tool in identifying individuals with autoimmune disease who may have underlying pulmonary arterial disease that may be amenable to therapy. We noted a consistent elevation in sPAP across all autoimmune subtypes, suggesting an abnormal pulmonary vascular response to exercise exists in these patients.
Keywords: PAH; Pulmonary artery hypertension; PAP; Pulmonary artery pressure; RHC; Right heart catheterisation; PHASE-I; Pulmonary Hypertension Assessment and Screening Evaluation; SE; Stress echocardiography; SLE; Systemic lupus erythamatous; SLARC; Scleroderma-Lupus Autoimmune Resource Centre; DSS; Diffuse systemic sclerosis; LSS; Limited systemic sclerosis; BDI; Borg dyspnoea index; WHO; World Health Organisation; 6MWT; Six-minute walk test; Ox0; Oximetry at rest; Ox6; Oximetry after 6MWT; PFT; Pulmonary function testing; DLCO; Carbon dioxide diffusing capacity; TLC; Total lung capacity; HRCT; High Resolution Computed Tomography; ECG; Electrocardiogram; TR; Tricuspid regurgitant; sPAP; Systolic pulmonary artery pressure; sPAPPRE; Systolic pulmonary artery pressure before exercise; sPAPPOST; Systolic pulmonary artery pressure after exercise; 
PAP; Change in PAP; CXR; Chest X-ray; VQ; Lung ventilation perfusion
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