Prognosis of systemic sclerosis (SSc) is affected by pulmonary artery hypertension (PAH).Methods:
Among 202 patients (mean age: 46.1 ± 13.3 years; 177 women) with SSc, those with a tricuspid regurgitation (TR) jet maximal velocity at 2D-echocardiography (2DE) < 2.8 m/second were not considered at high risk for PAH, whereas those with a TR velocity >3 m/second or between 2.8 and 3 m/second and associated with dyspnea were.Results:
Among 22 patients at risk, 15 (mean age: 50.4 ± 14.3 years) had definite precapillary PAH on right heart catheterization (RHC). The delay period between recognitions of SSc and PAH was 12.9 ± 5.2 years. Dyspnea was present in all 15 patients, 11 (73.3%) being in the New York Heart Association class III or IV. The 2DE showed normal left ventricular geometrics and function (n = 15), enlargement of the right-sided cardiac chambers (n = 12), increased pulmonary arterial resistances with a TR velocity to pulmonary time-velocity integral ratio of > 0.2 (n = 15) and impaired right ventricle function (n = 15). RHC showed severe PAH in all 15 patients (mean pulmonary artery pressure: 48 ± 17 mm Hg and mean right atrial pressure: 11.8 ± 4.4 mm Hg) and a reduced cardiac index (2.2 L/m2). There was no statistical difference between patients with and without PAH regarding age, sex ratio, duration from onset of disease, diffuse or cutaneous limited type of SSc, Rodnan severity score and presence of digital ulcerations or autoantibodies. Telangiectasia (P = 0.01) and New York Heart Association class III or IV heart failure (P = 0.001) were more frequent in patients with PAH.Conclusion:
A combined clinical and Doppler-coupled 2DE screening of PAH risk in patients with SSc is useful to select those who can undergo RHC.