Negoi R I, Ghiorghiu I, Filipoiu F, Hostiuc M, Negoi I, Ginghina C
Anatomy Department, "Carol Davila" University of Medicine and Pharmacy, Bucharest, Romania.
Cardiology Department, "C.C. Iliescu" Institute for Emergencies in Cardiovascular Diseases, Bucharest, Romania; "Carol Davila" University of Medicine and Pharmacy, Bucharest, Romania.
J Med Life. 2017 Apr-Jun;10(2):131-138.
The aim of this study was to compare the effects of Sildenafil, Bosentan and combined therapy in patients with congenital cardiac shunts associated pulmonary artery hypertension (CCS-PAH). Prospective observational study (February 2011 - January 2014) with a historical control group (January 2009 - January 2011). "CC Iliescu" Institute for Emergency Cardiovascular Diseases of Bucharest, a tertiary university-affiliated center. All cases with CCS-PAH. Specific vasodilatory therapy: Sildenafil, Bosentan or combined therapy. The primary outcome was the overall survival at 24 months. Out of 108 patients with pulmonary arterial hypertension, there were 79 patients with CCS-PAH, 55 presenting a severe form of the disease. The mean age of the patients was 34.42±21.15 years, with 37 (67,3%) female patients. 23 patients received specific vasodilatory treatment (thirteen Sildenafil, seven Bosentan, three combined treatment), with 32 patients in the control group, without specific vasodilatory therapy. The specific vasodilatory therapy was associated with improved WHO/ NYHA functional class (p=0.025), oxygen saturation at the end of the six-minute walk test (p=0.011), decreased pulmonary artery systolic (p=0.002) and diastolic (p=0.004) pressures, and an increased S' wave in Tissue Doppler Imaging (p=0.008). Despite the complexity of CCS-PAH, with a complex constellation of underlying congenital heart defects, there are short-term benefits of a specific vasodilatory therapy.
本研究旨在比较西地那非、波生坦及联合治疗对先天性心脏分流相关性肺动脉高压（CCS-PAH）患者的疗效。采用前瞻性观察性研究（2011年2月至2014年1月），并设立历史对照组（2009年1月至2011年1月）。研究地点为布加勒斯特“CC伊利埃斯库”紧急心血管疾病研究所，这是一家隶属于大学的三级中心。研究对象为所有CCS-PAH病例。采用特定的血管舒张疗法：西地那非、波生坦或联合治疗。主要结局指标为24个月时的总生存率。在108例肺动脉高压患者中，有79例为CCS-PAH患者，其中55例为重症患者。患者的平均年龄为34.42±21.15岁，女性患者37例（占67.3%）。23例患者接受了特定的血管舒张治疗（13例使用西地那非，7例使用波生坦，3例接受联合治疗），对照组有32例患者未接受特定的血管舒张治疗。特定的血管舒张治疗与世界卫生组织/纽约心脏协会（WHO/NYHA）功能分级改善（p=0.025）、6分钟步行试验结束时的血氧饱和度提高（p=0.011）、肺动脉收缩压（p=0.002）和舒张压（p=0.004）降低以及组织多普勒成像中S波增加（p=0.008）相关。尽管CCS-PAH病情复杂，伴有一系列复杂的潜在先天性心脏缺陷，但特定的血管舒张治疗仍有短期益处。
