Unloading the right ventricle in the CREST syndrome variant of progressive systemic sclerosis (scleroderma)

A patient with severe pulmonary hypertension secondary to the CREST syndrome variant of scleroderma is described. Acute reductions in both pulmonary artery pressure and total pulmonary resistance were seen with nifedipine and oxygen administration. Reductions in resistance were maintained for over one month with this combination. These results raise the possibility that some of the pulmonary hypertension seen in this condition is reversible.