Patient selection: systemic sclerosis with no severe pulmonary function abnormalities
Parameters:
(1) peak velocity of tricuspid regurgitation in m/s by Doppler ultrasonography
(2) clinical findings
(3) findings on right heart catheterization at rest
(4) findings on right heart catheterization during exercise
(5) pulmonary artery wedge pressure (PAWP)
Reasons to conclude that PAH is absent:
(1) peak velocity of tricuspid regurgitation < 2.5 m/s
(2) peak velocity of tricuspid regurgitation 2.5 to 3.0 m/s AND either no dyspnea or dyspnea that can be explained by another cause
(3) peak velocity of tricuspid regurgitation 2.5 to 3.0 m/s AND unexplained dyspnea AND mean pulmonary artery pressure < 25 mm Hg at rest AND mean pulmonary artery pressure < 30 mm Hg during exercise
(4) peak velocity of tricuspid regurgitation > 3.0 m/s AND mean pulmonary artery pressure < 25 mm Hg at rest AND mean pulmonary artery pressure < 30 mm Hg during exercise
(5) PAWP >= 15 mm Hg (indicates left-sided heart disease)
Reasons to conclude that PAH is present:
(1) peak velocity of tricuspid regurgitation 2.5 to 3.0 m/s AND unexplained dyspnea AND mean pulmonary artery pressure < 25 mm Hg at rest AND mean pulmonary artery pressure >= 30 mm Hg during exercise
(2) peak velocity of tricuspid regurgitation 2.5 to 3.0 m/s AND unexplained dyspnea AND mean pulmonary artery pressure >= 25 mm Hg at rest AND PAWP < 15 mm Hg
(3) peak velocity of tricuspid regurgitation > 3.0 m/s AND mean pulmonary artery pressure < 25 mm Hg at rest AND mean pulmonary artery pressure >= 30 mm Hg during exercise
(4) peak velocity of tricuspid regurgitation > 3.0 m/s AND mean pulmonary artery pressure >= 25 mm Hg at rest AND PAWP < 15 mm Hg
