Pulmonary arterial hypertension (PAH) is an important cause of mortality in patients with systemic sclerosis (scleroderma). Steen et al used exercise testing to evaluate patients with scleroderma at high risk for PAH. The authors are from Georgetown University.


Patient selection - A patient with scleroderma was considered at high risk for PAH if one or more of the following is present:

(1) dyspnea on exertion

(2) diffuse capacity of carbon monoxide (DLco) < 60% of predicted

(3) forced vital capacity (FVC) < 70% of predicted

(4) ratio of percent of predicted FVC to percent of predicted DLco > 1.6

(5) resting right ventricular systolic pressure (RVSP) > 35 mm Hg


Screening exercise: standard Bruce protocol on a treadmill with target 85% of predicted maximum heart rate


increase in RVSP with exercise =

= (RVSP with exercise) - (RVSP at rest)


Indications for right heart catheterization - one of the following:

(1) RVSP > 40 mm Hg

(2) increase in RVSP with exercise >= 20 mm Hg

(3) RVSP < 40 mm Hg and symptomatic


A patient with a mean pulmonary arterial pressure < 25 mm Hg and pulmonary arterial occlusion pressure < 15 mm Hg underwent supine exercise with dumbbells to exhaustion or 85% of predicted maximum heart rate.


A patient was said to have exercise-induced PAH if during supine exercise:

(1) mean PAP > 30 mm Hg AND

(2) pulmonary arterial occlusion pressure < 18 mm Hg


To read more or access our algorithms and calculators, please log in or register.