摘要
目的:探讨磷酸二酯酶抑制剂西地那非治疗先天性心脏病(congenital heart disease,CHD)所致重度肺动脉高压(pulmonary arterial hypertension,PAH)的有效性及安全性。方法:对体-肺分流型CHD所致重度PAH患者33例给予西地那非治疗6个月。以6分钟步行距离试验、心电图、心脏X线、彩色多普勒超声心动图、右心导管、血细胞和血生化检查检测评估治疗效果和不良反应。结果:西地那非治疗6个月后,所有患者6分钟步行距离显著改善(P<0.01),18例(54%)患者自觉症状明显改善,左心室射血分数明显增加(P<0.05)。右心导管检查示肺/体循环血流量比值明显提高(P<0.01),肺血管阻力(P<0.05),肺/体循环压力比值(P<0.01)和血管阻力比值(P<0.01)明显下降,但有8例(24%)患者肺/体循环血流量比值下降同时肺/体循环血管阻力比值增大。不良反应事件包括脸红(n=3)、头痛(n=2)、鼻充血(n=1),异常阴茎勃起(n=1)等。实验室检查显示红细胞和血红蛋白较治疗前显著降低(P<0.01),而肌酐轻度升高(P<0.05)。结论:CHD并发PAH患者接受西地那非治疗6个月耐受性良好,大部分患者运动耐量和血流动力学明显改善。
AIM： To evaluate the efficacy and safety of sildenafil in treatment of patients with severe pulmonary arterial hypertension （ PAH ） secondary to congenital heart disease （ CHD ）. METHODS ： Patients with severe PAH secondary to systemic-to-pulmonary shunt CHD were treated with sildenafil for 6 months. The dose of sildenafil was 25 mg three times daily in patients weighing ≥50 kg and 0. 5 mg/kg thrice daily in children weighing 〈 50 kg. Six-minute walk distance test, echocardiography, cardiac catheterization, complete blood count, and hepatic as well as renal functions were assessed at baseline and after the 6-month therapy. RESULTS： After the 6-month sildenafil treatment, all the patients had a significant increase in the 6-minute walk distance （P 〈0. 01 ） and 18 of them had symptomatic improve- ment. No significant changes were observed in echocardiograhic parameters, including fight ventricular end-diastolic diameter, left ventricular end-diastolic diameter and volume, and tricuspid regurgitation peak velocity, except the left ventricular ejection fraction （P 〈 0.05 ）. Right heart catheterization showed significant increase in pulmonary-to-systemic flow （Qp/Qs） ratio （P 〈0. 01 ） and significant decrease in pulmonary vascular resistance （ P 〈 0. 05 ） and pulmonary-to-systemic vascular resistance （Rp/Rs） ratio. Simultaneous decrease in Qp/Qs ratio and increase in Rp/Rs ratio were found in 8 （24. 2% ） of the 33 patients. Adverse events were observed in seven patients, including headache in two patients, flushing in three patients, nasal congestion in one patient, and abnormal penile erection in one teenage male. Signif- icant decreases of red blood cell count （P 〈0. 01 ） and hemoglobin （P 〈0. 01 ） accompanied by a mild increase of creatinine （P 〈 0.05 ） were observed after the 6-month therapy. CONCLUSION ： Six-month sildenafil therapy is well tolerated and the therapy markedly improves the exercise capacity and hemodynamics in most patients w
出处
《心脏杂志》
CAS
2012年第5期600-603,共4页
Chinese Heart Journal
关键词
肺动脉高压
心脏病
先天性
西地那非
磷酸二酯酶抑制剂
有效性
安全性
pulmonary arterial hypertension
congenital heart disease
sildenafil
phosphodiesterase inhibitor
efficacy
safety
