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Identical Twins With Primary Pulmonary Hypertension*: Beraprost vs Epoprostenol

Erika Berman Rosenzweig; Kelly A. Schmitt; Robert Garofano; Robyn J. Barst
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*From Children’s Hospital of New York, Columbia University, College of Physicians and Surgeons, New York, NY.

Correspondence to: Erika Berman Rosenzweig, MD, Children’s Hospital of New York-Presbyterian, 3959 Broadway, 2 North, New York, NY 10032; e-mail: esb14@columbia.edu



Chest. 2004;125(3):1157-1160. doi:10.1378/chest.125.3.1157
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Background: The course of 12-year-old, homozygotic twins with primary pulmonary hypertension (PPH) treated with different vasoactive agents, beraprost vs epoprostenol, is described.

Methods: Clinical, exercise, and hemodynamic assessments were made at baseline, and at 9 months and 24 months of treatment.

Findings: Twin A had a rapid improvement with epoprostenol. In contrast, twin B, initially treated with beraprost, had progressive worsening with subsequent improvement on epoprostenol.

Interpretation: Epoprostenol was efficacious for identical twins with PPH. A 9-month delay in initiating epoprostenol for twin B did not appear to have irreversible short-term detrimental effects.

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