Identical twins with primary pulmonary hypertension: beraprost vs epoprostenol.

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.