Fujino Takeo, Yao Atsushi, Hatano Masaru, Inaba Toshiro, Muraoka Hironori, Minatsuki Shun, Imamura Teruhiko, Maki Hisataka, Kinugawa Koichiro, Ono Minoru, Nagai Ryozo, Komuro Issei
Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo.
Int Heart J. 2015;56(1):86-93. doi: 10.1536/ihj.14-183. Epub 2015 Jan 7.
Therapeutic strategies for pulmonary arterial hypertension (PAH) associated with atrial septal defect (ASD) remain a matter of debate.
We identified 5 outpatients who had been diagnosed with ASD-PAH and undergone ASD closure in combination with targeted therapy with certified PAH drugs. We assessed changes in hemodynamic parameters and exercise capacity. The combination of ASD closure and targeted therapy significantly increased systemic blood flow (Qs) from the baseline (from 3.3 ± 0.6 L/minute to 4.2 ± 1.0 L/minute, P < 0.05) with a significant improvement in the World Health Organization Functional Class (WHO-FC; from 2.8 ± 0.4 to 1.6 ± 0.5, P < 0.05). The hemodynamic data before and after ASD closure without targeted therapy showed further elevation of pulmonary vascular resistance shortly after ASD closure (678 dyne · s/cm(5) to 926 dyne · s/cm(5)) in 1 case, as well as after a long time since ASD closure (491.0 ± 53.7 dyne · s/cm(5) to 1045.0 ± 217.8 dyne · s/cm(5)) in 2 cases. This worsening was reversed after the targeted therapy, accompanied by an increase in Qs and an improvement in WHO-FC in all cases.
Targeted therapy should be added to ASD closure in adult patients with ASD-PAH.
与房间隔缺损（ASD）相关的肺动脉高压（PAH）的治疗策略仍存在争议。
我们确定了5例被诊断为ASD-PAH并接受ASD封堵术联合经认证的PAH药物靶向治疗的门诊患者。我们评估了血流动力学参数和运动能力的变化。ASD封堵术与靶向治疗相结合显著增加了全身血流量（Qs），较基线水平升高（从3.3±0.6升/分钟增至4.2±1.0升/分钟，P<0.05），世界卫生组织功能分级（WHO-FC）也有显著改善（从2.8±0.4改善至1.6±0.5，P<0.05）。1例患者在未进行靶向治疗的情况下，ASD封堵术后不久肺血管阻力进一步升高（从678达因·秒/厘米⁵升至926达因·秒/厘米⁵），2例患者在ASD封堵术后较长时间也出现这种情况（从491.0±53.7达因·秒/厘米⁵升至1045.0±217.8达因·秒/厘米⁵）。靶向治疗后这种恶化情况得到逆转，所有病例的Qs均增加，WHO-FC也得到改善。
对于成年ASD-PAH患者，ASD封堵术应联合靶向治疗。
