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Experience and Reason

Robyn J. Barst, MD
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Affiliations: New York, NY 
 ,  Dr. Barst is Associate Professor of Pediatrics and Medicine, Columbia University College of Physicians and Surgeons.

Correspondence to: Robyn J. Barst, MD, Columbia University, Department of Pediatrics, 630 West 168th St, New York, NY 10032



Chest. 2000;117(1):2-5. doi:10.1378/chest.117.1.2
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“In medicine one must pay attention not to plausible theorizing but to experience and reason together … I agree that theorizing is to be approved, provided that it is based on facts, and systematically makes its deductions from what is observed … But conclusions drawn from unaided reasons can hardly be serviceable; only those drawn from observed fact.”

Hippocrates

Epoprostenol (prostaglandin I2, prostacyclin) is a potent vasodilator and inhibitor of platelet aggregation produced by vascular endothelium. Epoprostenol decreases pulmonary vascular resistance and increases cardiac output and systemic oxygen delivery when administered acutely to patients with primary pulmonary hypertension. Furthermore, continuous IV epoprostenol has been demonstrated to significantly improve quality of life and hemodynamics as well as increased survival in patients with severe primary pulmonary hypertension who fail conventional medical therapy,1ie, warfarin anticoagulation and oral vasodilators. Pulmonary hypertension is also associated with other conditions, including collagen vascular disease, congenital heart disease, liver disease, and thromboembolic disease. However, treatment of pulmonary hypertension associated with these other conditions has not been successful with conventional therapy. As opposed to patients with primary pulmonary hypertension in whom approximately 20% of patients respond with acute vasodilator drug testing and who can be effectively treated with conventional medical therapy,2 the experience reported by Robbins et al in the current issue of CHEST (see page 14) as well as previous reports by Sanchez et al3 demonstrate a significantly smaller proportion of patients acutely responding with vasodilator testing. Although Sanchez et al3 reported that 15 of 57 patients with pulmonary hypertension associated with connective tissue disease had a significant fall in total pulmonary resistance with acute vasodilator testing, only 4 had a combined fall (> 20%) in both pulmonary arterial pressure and total pulmonary resistance (7% as opposed to the reported 20% acute response rate with primary pulmonary hypertension patients). Furthermore, despite anecdotal case reports45 demonstrating acute pulmonary vasoreactivity in some patients with pulmonary hypertension associated with collagen vascular diseases, especially with CREST (calcinosis, Reynaud’s phenomenon, esophageal motility disorders, sclerodactyly, and telangiectasia) patients, long-term efficacy has not been demonstrated. These data confirm the lack of efficacy with conventional medical therapy in patients with pulmonary hypertension associated with connective tissue disease. Although pulmonary hypertension occurs in only 0.5 to 14% of cases with systemic lupus erythematosus,9 and much more rarely in patients with rheumatoid arthritis, Sjögren’s syndrome, and dermatomyositis, the prevalence varies from 2.3 to 35% in scleroderma1012 and may be as high as 50% in the CREST variant.11 Furthermore, the reported 2-year survival rate of only 40% in patients with CREST syndrome and pulmonary hypertension, compared with 88% in patients with CREST syndrome without pulmonary hypertension, underscores our need for effective therapy with these patients.10 In addition, patients with connective tissue diseases are often excluded from lung transplantation because of previous immunosuppressive therapy and possible involvement of other organs, such as the kidneys or liver with the underlying disease.


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