围手术期口服西地那非治疗成人先天性心脏病重度肺动脉高压

Treatment of severe pulmonary arterial hypertension with oral sildenafil in patients with congenital heart disease during perioperative period

目的研究围手术期口服西地那非对成人先天性心脏病（先心病）重度肺动脉高压的治疗效果。方法 2010年8月至2013年8月,经右心导管检查证实平均肺动脉压（MPAP）≥45 mmHg的42例先心病重度肺动脉高压患者纳入研究,平均年龄（31.2±16.1）岁,其中房间隔缺损（ASD组）22例,室间隔缺损（VSD组）12例,动脉导管未闭（PDA组）8例。所有患者术前2~3周开始口服西地那非,每次0.5 mg/kg,3次/d,观察并比较服药前后血流动力学指标变化;术后待循环稳定后继续用药,其中19例患者麻醉诱导后放置漂浮导管,比较这些患者术后服药后0.5、1、4、24 h的MPAP、平均动脉压（MAP）、MPAP/MAP、心脏指数（CI）、肺血管阻力指数（PVRI）、体循环阻力指数（SVRI）变化趋势。结果 42例患者术前服药（17.51±7.29）d后复查心脏彩超,肺动脉收缩压（SPAP）为（80.13±15.41）mmHg,较服药前[（87.71±14.68）mmHg]明显下降,差异有统计学意义（P〈0.05）。19例放置漂浮导管的患者,术后服药后0.5、1、4、24 h监测MPAP、PVRI,均较服药前明显下降,差异有统计学意义（P〈0.05）;MAP仅服药后0.5 h较服药前下降,差异有统计学意义（P〈0.05）;SVRI、CI与服药前相比差异无统计学意义;MPAP/MAP服药后持续下降,与服药前相比差异有统计学意义（P〈0.05）。所有患者术后均存活,术后随访2~20个月,无晚期死亡。结论围手术期口服西地那非治疗先心病引起的重度肺动脉高压疗效明确、安全而且使用方便。

Objective To study the effects of oral sildenafil for the treatment of severe pulmonary arterial hypertension（ PAH） in patients with congenital heart disease during perioperative period. Methods From August 2010 to August 2013,42 patients（ mean age of 31.2 ±16.1 years old） with congenital heart disease and severe PAH confirmed by base line assessment of cardiac catheterization,and with mean pulmonary artery pressure（ MPAP） ≥ 45mmHg,were chosen for the study. Of all the cases,22 were atrial septal defect（ ASD） cases,12 were ventricular septal defect（ VSD） cases and 8 were patent ductus arteriossus（ PDA） cases. Oral sildenafil at a dosage of 0. 5mg /kg,3 times a day,was administered to all the patients,2 to 3 weeks before surgery. Then,changes in the hemodynamic parameters were observed and compared before and after medication. All the patients resumed medication,after circulatory stability was achieved. Then,19 patients were placed Swan- Ganz catheter following anesthesia. Changes in mean pulmonary artery pressure（ MPAP）,mean artery pressure（ MAP）,MPAP /MAP,cardiac index（ CI）,pulmonary vascular resistance index（ PVRI）and systemic vascular resistance index（ SVRI） were observed and compared 0. 5 h,1h,4h and 24h after medication following anesthesia. Results For the 42 patients,who received medication for（ 17. 51 ± 7. 29） days before surgery,color cardiac ultrasonography indicated that systolic pulmonary artery pressure（ SPAP） was（ 80. 13 ± 15. 41） mmHg,which was significantly lower than that before medication [（ 87. 71 ± 14. 68） mmHg],with statistical significance（ P〈0. 05）. In the 19 patients who received floating catheter,MPAP and PVRI decreased significantly,0. 5 h,1 h,4 h and 24 h after medication,also with statistical significance（ P〈0. 05）.MAP was lower 0. 5h after medication,when it was compared with that before medication,also with statistical significance（ P〈0. 05）.No statistical significance could be noted in SVRI and CI,when they were compared with those before medication（ P〈0. 05）. MPAP /MAP ratio continued to decline after medication and statistical differences could be noted,when they were compared with those before medication（ P〈0. 05）. All the patients survived after surgery. Two-to-20 month follow- ups after surgery indicated that there were no late- stage deaths（ P〈0. 05）. Conclusion During perioperative period,oral sildenafil for the treatment of severe PAH induced by congenital heart disease could produce good therapeutic effects and proved to be safe and convenient.