Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects
Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects
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DOI：
10.1161/01.cir.99.14.1858
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发表时间：
1999-04-13
期刊：
影响因子：
37.8
通讯作者：
Barst, RJ
中科院分区：
文献类型：
--
作者：
Rosenzweig, EB;Kerstein, D;Barst, RJ
尽管长期应用前列环素(PGI2)已被证明能改善原发性肺动脉高压患者的血流动力学、生活质量和生存率，但其在肺动脉高压(PHT)和相关先天性心脏病(CHD)患者中的应用尚未得到评估。方法和结果-20名PHT和相关CHD患者(15+/-14岁)，包括9例房间隔缺损，7例室间隔缺陷，4例大血管转位，3例动脉导管，3例部分肺静脉异常回流，1例主肺窗，这些患者未能接受常规治疗(包括洋地黄、利尿剂、氧气、华法林、钙通道阻断，如有指征；和手术，如果可以手术的话)用慢性前列腺素I2治疗。11例患者有心脏手术经历，年龄中位数为3岁(5天至47岁)。20例患者中有11例存在残余的体肺分流。在PGI2治疗前和治疗一年后测量血流动力学、NYHA功能分级和运动能力。所有患者对PGI2治疗均无明显反应。尽管没有急性反应，但慢性前列腺素I2患者的平均肺动脉压下降了21%：77+/-20至61+/-15毫米汞柱(P
Background-Although long-term prostacyclin (PGI2) has been shown to improve hemodynamics, quality of life, and survival in patients with primary pulmonary hypertension, its use in patients with pulmonary hypertension (PHT) and associated congenital heart defects (CHD) has not been evaluated.Methods and Results-Twenty patients (15+/-14 years) with PHT and associated CHD (9 atrial septal defect, 7 ventricular septal defect, 4 transposition of the great vessels, 3 patient ductus arteriosus, 3 partial anomalous pulmonary venous return, and 1 aortopulmonary window) who failed conventional therapy (including digitalis; diuretics; oxygen; warfarin; calcium channel blockade, if indicated; and surgery, if operable) were treated with chronic PGI2. Eleven patients had previous cardiac surgery at a median age of 3 years (range, 5 days to 47 years). Eleven of 20 patients had residual systemic to pulmonary shunts. Hemodynamics, NYHA functional class, and exercise capacity were measured at baseline and after I year of PGI2 therapy. None of the patients acutely responded to PGI2 administration. Despite lack of an acute response, mean pulmonary artery pressure decreased 21% on chronic PGI2: 77+/-20 to 61+/-15 mm Hg (P