PREVALENCE OF PULMONARY-HYPERTENSION IN LIMITED AND DIFFUSE SCLERODERMA

Study objectives: To characterize the prevalence of undiagnosed pulmon ary hypertension in patients with limited and diffuse scleroderma. Des ign: Prospective cross-sectional study. Setting: University-based outp atient clinic. Patients: Thirty-four consecutive patients with limited (n=29) or diffuse (n=5) scleroderma but without the clinical diagnosi s of pulmonary hypertension, Measurements and results: All patients ha d 12-lead ECGs and two-dimensional and Doppler echocardiograms. The pu lmonary artery systolic pressure (PA(s)) as calculated as the sum of t he Doppler transtricuspid pressure gradient and the right atrial press ure as estimated by the caval respiratory index. Thirty-three patients (97%) had adequate spectral signals of tricuspid regurgitation. Tile velocity of tricuspid regurgitation ranged from 1.6 to 4.5 mis. The ca lculated PA(s) ranged from 15 to 95 (mean+/-SD=30+/-14 mm Hg). Twelve patients (35% of the total cohort) had pulmonary hypertension defined as PA(s) of 30 mm Hg or greater. Conclusions: Undiagnosed elevation of PA(s) is common in patients with scleroderma. Noninvasive assessment of PA, can be performed accurately in most patients independent of cli nical signs of pulmonary hypertension. If successful treatment strateg ies are identified, it may be possible to identify patients early in t he development of pulmonary hypertension and intervene before signific ant end-organ damage occurs.