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Assessing early pulmonary hypertension in scleroderma

Associate Professor Dr. David Prior - Department of Cardiology & Rheumatology, St. Vincent's Hospital, Melbourne

High Blood pressure in the lungs or pulmonary arterial hypertension occurs in 25% of patients with scleroderma, causing breathlessness and eventually heart failure and death. Treatment, which can improve symptoms and life expectancy, is usually started late because current tests for the disease only detect an abnormality after there is significant irreversible damage to lung blood vessels. This study will examine whether a new ultrasound stress test can detect early abnormalities of lung blood flow in patients with scleroderma.

 

Dr. Prior is a cardiologist and researcher with interests in heart failure, echocardiography and pulmonary hypertension. He has been an invited speaker at national and international meetings in these areas and is currently head of the Cardiology Outpatients Clinic at St. Vincent's Hospital, Melbourne.

Scientific Report

Investigators

Associate Professor Dr. David Prior & Dr Wendy Stevens

The aims of this project were to develop a new non-invasive method for assessment of early changes in the pulmonary vasculature in patients with scleroderma at risk of pulmonary arterial hypertension (PAH). The Arthritis Australia project grant allowed us to commence this project in 2009 by providing funding for consumables for performing the low dose dobutamine stress echocardiograms, test strips to measure B-type natriuretic peptide) BNP levels and a part-time study nurse to perform 6 minute walk tests.

Thus far we have recruited 42 patients with scleroderma (37 without known PAH and 5 with known PAH on therapy) and 3 normal control subjects. We have been able to show that the low dose dobutamine testing protocol is safe in this population and provides measurable changes in pulmonary pressure and flow which can be used to derive an index of pulmonary vascular resistance. We have been able to obtain additional funding to allow us to proceed with the full study as planned. We continue to recruit new subjects and have begun follow-up testing in some of the participants to test the stability of our new index. Drs Jennifer Coller and Ben Pang have assisted with patient recruitment, stress test supervision and data analysis.

It is anticipated that the study will be completed some time in 2011 and final results will be available by the end of that year.

Lay Report

This project aims to examine changes in pressure within the lung arteries when the flow through the lungs is increased using a drug called dobutamine in patients who have scleroderma and may be at risk of developing high blood pressure in the lungs (pulmonary arterial hypertension). We would like to find out whether we can identify early changes in the lung arteries which may progress to pulmonary arterial hypertension using a new ultrasound test to measure flow and pressure in the lungs.

The Arthritis Australia grant allowed us to commence this project in 2009 by providing funding for a part-time study nurse to perform walking tests on study participants and for the drugs and consumables needed for performing the low dose dobutamine stress ultrasounds.

Thus far 45 volunteers have participated in the study (37 who are not known to have high lung blood pressure, 5 with known high lung pressure and 3 normal subjects for comparison). We have been able to show that the testing is safe in these participants and provides measurable changes in lung artery pressure and flow which can be used to derive an index of lung blood vessel function. We have been able to obtain additional funding to allow us to proceed with the full study as planned. We continue to recruit new participants and have begun follow-up testing in some of the participants to test the stability of our new test findings. Drs Jennifer Coller and Ben Pang have assisted with patient recruitment, stress test supervision and data analysis.

It is anticipated that the study will be completed sometime in 2011 and final results will be available by the end of that year.