前列地尔联合西地那非在房缺重度肺动脉高压的临床应用(附2例病例报告)

Combined treatment with intravenous Lipo-prostaglandin E_1 and sildenafil in patients with pulmonary arterial hypertension (PAH) secondary to atrial septal defects(ASD): report of 2 cases

目的本研究总结了前列地尔联合西地那非控制两例房缺合并严重肺动脉高压(PAH)的疗效. 方法两例患者均为青年女性,有心功能不全及劳力性紫绀,心导管检查(PAP)几乎与动脉压相等,前列腺素降压实验无反应,影像学检查肺动脉及右心系统均表现扩张与肥厚,由于考虑患者年龄及静息状态血气,在充分准备下进行了手术处里,术前巯甲丙脯酸6.25 mg口服2/d,西地那非12.5 mg口服2/d,前列地尔[前列腺素E1脂微球制剂(Lipo-PGE1),北京泰德公司]40 μg,静滴1次/d,半月后于全麻体外循环(ECC)心脏不停跳下行房缺修补术,术中前列地尔持续泵入.术后持续Lipo-PGE1 1～3 ng/(kg·min)、硝普钠0.01～0.3 μg/(kg·min),术后第4天开始服用西地那非12.5 mg 2/d,巯甲丙脯酸12.5 mg 2/d,前列地尔20 μg VD 1/d. 结果出院后停前列地尔,口服西地那非依次12.5 mg 3/d,25 mg 2/d,25 mg 3/d,50 mg 2/d,各1周.最后Lipo-PGE1 20 μg 静滴,1/d,共10 d,用药期间多次出现头痛,随访5～10个月心功能Ⅰ级(NYHA)已经恢复原办公室工作. 结论 Lipo-PGE1和西地那非联合应用是控制先心病合并严重PAH的理想方案.

Objective To report the experience obtained from a combined treatment program with intravenous (i v) lipo prostaglandin E 1(Lipo PGE 1,Alprostadil, Beijing Taide pharmaceutical co LTD )and oral sildenafil in patients with severe pulmonary hypertension secondary to atrial septal defects(ASD) during peri operation Methods The two ASD patients were diagnosed PAH by right heart catheterization Their pulmonary pressure almost equal to artery blood pressure The changes of clinical manifestation (NYHA functional class), heart rate, echocardiography (including to estimate pulmonary pressure) were evaluated before and after treatment The treatment program was intravenous (i v) lipo prostaglandin E 1 20 μg daily 2 weeks, and 2~4 ng/(kg·min) from operation to 4ths day Then, sildenafil was added to the treatment Initial sildenafil dose was 12 5 mg 2 times daily for a week So in succession 12 5 mg 3 times daily, 25 mg 2 times, 25 mg 3 times,50 mg 2 times daily respectively a week and another intravenous (i v) lipo prostaglandin E 1 20 μg daily 2 weeks Results By the end of the program the symptoms of dyspnea were disappeared Their cardiac function converted to class I (previously classⅡ and Ⅲ) The systolic pulmonary artery pressure decreased in 2 patients (average reduction 47%) The right ventricular were diminished apparently The only side effect of sildenafil seen was mild headache They all recovered to their original work positions when the follow up were 5,10 months Conclusion Our experience supports the value of our program in the treatment of PAH It is useful for rescuing patients who has severe pulmonary hypertension secondary to congenital heart disease