Prevalence of pulmonary hypertension in limited and diffuse scleroderma

Study objectives: To characterize the prevalence of undiagnosed pulmonary hype 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 (PA(s)) as 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 regurgitation. The velocity of tricuspid regurgitation ranged from 1.6 to 4.5 mis. The calculated 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 clinical 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 significant end-organ damage occurs.