Berman Rosenzweig Erika, Schmitt Kelly A, Garofano Robert, Barst Robyn J
Children's Hospital of New York, Columbia University, College of Physicians and Surgeons, New York, NY, USA.
Chest. 2004 Mar;125(3):1157-60. doi: 10.1378/chest.125.3.1157.
The course of 12-year-old, homozygotic twins with primary pulmonary hypertension (PPH) treated with different vasoactive agents, beraprost vs epoprostenol, is described.
Clinical, exercise, and hemodynamic assessments were made at baseline, and at 9 months and 24 months of treatment.
Twin A had a rapid improvement with epoprostenol. In contrast, twin B, initially treated with beraprost, had progressive worsening with subsequent improvement on epoprostenol.
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.
描述了一对12岁同卵双胞胎原发性肺动脉高压（PPH）患者接受不同血管活性药物（贝拉前列腺素与依前列醇）治疗的病程。
在基线、治疗9个月和24个月时进行临床、运动和血流动力学评估。
双胞胎A使用依前列醇后迅速改善。相比之下，双胞胎B最初接受贝拉前列腺素治疗，病情逐渐恶化，随后改用依前列醇后有所改善。
依前列醇对患有PPH的同卵双胞胎有效。双胞胎B开始使用依前列醇延迟9个月似乎并未产生不可逆的短期有害影响。
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