Prevalence of Pulmonary Hypertension

Study objectives: To characterize the prevalence ofundiagnosedpulmonary hypertension in patients with limited and diffuse scleroderma. Design: Prospective cross-sectional study. Setting: University-based outpatient clinic. Patients: Thirty-four consecutive patients with limited (n=29) or diffuse (n=5) scleroderma but without the clinical diagnosis of pulmonary hypertension. Measurements and results: All patients had 12-lead ECGs and two-dimensional and Doppler echocardiograms. The pulmonary artery systolic pressure (PAg) was calculated as the sum of the Doppler transtricuspid pressure gradient and the right atrial pressure as estimated by the caval respiratory index. Thirty-three patients (97%) had adequate spectral signals of tricuspid regurgita¬ tion. The velocity of tricuspid regurgitation ranged from 1.6 to 4.5 m/s. The calculated PAs ranged from 15 to 95 (mean±SD=30± 14 mm Hg). Twelve patients (35% ofthe total cohort) had pulmonary hypertension defined as PAS of 30 mm Hg or greater. Conclusions: Undiagnosed elevation of PAS is common in patients with scleroderma. Noninvasive assessment of PA^ can be performed accurately in most patients independent of chnical signs of pulmonary hypertension. If successful treatment strategies are identified, it may be possible to identify patients early in the development of pulmonary hypertension and intervene before signif¬ icant end-organ damage occurs. (CHEST 1996; 110:1515-19)