Combination therapy with prostacyclin and tadalafil for severe pulmonary arterial hypertension: A pilot study
Combination therapy with prostacyclin and tadalafil for severe pulmonary arterial hypertension: A pilot study
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DOI：
10.1111/j.1440-1843.2007.01176.x
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发表时间：
2008-09-01
期刊：
影响因子：
6.9
通讯作者：
Kramer, Mordechai R.
中科院分区：
文献类型：
--
作者：
Bendayan, Daniele;Shitrit, David;Kramer, Mordechai R.
尽管引入了改变肺动脉高压（PAH）病程的新药，但一些患者仍然难以治疗并迅速恶化。长效磷酸二酯酶-5抑制剂是一类对PAH有效的新型药物。这项前瞻性研究评估了前列环素和他达拉非联合治疗重度PAH的潜力。我们报告了4例重度PAH病例，尽管接受了前列环素治疗，但病情仍恶化。两名患者患有艾森曼格综合征，一名患有硬皮病相关的肺动脉高压，一名患有组织细胞增多症X。除前列环素外，所有患者均接受他达拉非10-20 mg每日一次治疗。治疗3个月后，所有患者的临床症状均得到改善，平均6 MWD从214 m增加至272 m。3例患者的纽约心脏协会功能分级从IV级降至III级。超声心动图显示肺动脉压无明显变化。尽管本研究受样本量小的限制，但它表明他达拉非联合前列环素是重度PAH的有效治疗。他达拉非可能有益于晚期疾病患者的治疗。
Despite the introduction of new drugs that have changed the course of pulmonary arterial hypertension (PAH), some patients are still refractory to treatment and deteriorate rapidly. Long-acting phosphodiesterase-5 inhibitors are a new class of drugs that are effective in PAH. This prospective study assessed the potential of combination therapy with prostacyclin and tadalafil for treatment of severe PAH. We report four cases of severe PAH that deteriorated despite prostacyclin therapy. Two patients had Eisenmenger syndrome, one had pulmonary hypertension associated with scleroderma and one had histiocytosis X. All were treated with tadalafil, 10-20 mg once daily, in addition to prostacyclin. After 3 months of treatment, all patients improved clinically, with an increase in mean 6MWD from 214 to 272 m. In three patients, the New York Heart Association functional class decreased from IV to III. Echocardiograms showed no significant changes in pulmonary arterial pressure. Although this study was limited by the small sample size, it suggests that tadalafil in combination with prostacyclin is an effective treatment for severe PAH. Tadalafil may be beneficial for the treatment of patients with advanced disease.