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a Pulmonary Vascular Unit, Department of Respiratory
Medicine, b Clinical Research Initiative, University of Glasgow,
Department of Cardiology, c West Glasgow
Hospitals University NHS Trust, Western Infirmary, Glasgow G11 6NT, UK
Centre for Rheumatic Diseases, Glasgow Royal Infirmary
University NHS Trust, Royal Infirmary, Glasgow G4 0SF, UK
Correspondence to: Dr A J Peacock.
Received 7 October 1997; Returned to authors 21 January 1998; Revised version received 16 March 1998; Accepted for publication 26 May 1998
BACKGROUND
The specific contribution of
secondary pulmonary hypertension to the morbidity and mortality of
patients with underlying lung disease can be difficult to assess from
single measurements of pulmonary artery pressure. We have studied
patients with secondary pulmonary hypertension using an ambulatory
system for measuring continuous pulmonary artery pressure (PAP). We
chose to study patients with connective tissue disease because they
represent a group at high risk of pulmonary vascular disease, but with
little disturbance of lung function.
METHODSSix patients (five with progressive
systemic sclerosis and one with systemic lupus erythematosis) were
studied. They underwent preliminary cardiopulmonary investigations
followed by Doppler echocardiography, right heart catheterisation, and
ambulatory pulmonary artery pressure monitoring to measure changes in
pressure over a 24 hour period including during a formal exercise test.
RESULTSAll patients had pulmonary
hypertension as measured by Doppler echocardiography with estimated
pulmonary artery systolic pressures of 40-100 mm Hg. Pulmonary
function testing revealed virtually normal spirometric values (mean
FEV1 86.9% predicted) but marked reduction in CO gas
transfer factor (KCO 57.8% predicted). Exercise responses
were impaired with mean VO2max 50.6%
predicted. Ambulatory PAP monitoring indicated significant changes in
pressures with variation in posture and activity throughout 24 hours.
Resting PAP did not predict the change in PAP seen on exercise.
CONCLUSIONConventional methods of
assessment of the pulmonary circulation based on single measurements in
the supine position may underestimate the stresses faced by the right
side of the circulation. This ambulatory system allows monitoring of
pulmonary haemodynamics continuously over 24 hours during normal
activities of daily living. These measurements may increase our
understanding of the contribution made by secondary pulmonary
hypertension to the morbidity and mortality of the underlying lung disease.
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