Lee Meng-Luen
Division of Pediatric Cardiology, Department of Pediatrics, Changhua Christian Hospital, Changhua 50050, Taiwan.
Pediatr Neonatol. 2016 Apr;57(2):145-8. doi: 10.1016/j.pedneo.2013.06.007. Epub 2013 Oct 6.
An 8.7-year-old boy was affected by exertional dyspnea with cyanosis of the lip at 6 years old. Oxygen saturation (SpO2) was 66%. A bidirectional Glenn shunt (BGS) was constructed to successfully elevate SpO2 to 88%. Unfortunately, he again experienced exertional dyspnea with flagrant cyanosis of the lip at 8.5 years old. SpO2 decreased to 65%. Echocardiography revealed a dysplastic pulmonary valve with severe stenosis. Considering the potential growth of the right ventricle and the branch pulmonary arteries, transjugular balloon pulmonary valvuloplasty (BPV) through a BGS was performed as a palliative treatment for cyanosis in this boy because of inaccessible femoral veins. After gradational BPV, the opening of the pulmonary valve was dilated from 2.59 mm to 6.65 mm, the pressure gradient decreased from 60 mmHg to 25 mmHg, and the SpO2 increased to 85%. He became physically active and was free of exertional dyspnea at the 12-month follow-up. BGS is irrefutably an alternative vascular access through which transjugular BPV could be performed to ameliorate cyanosis due to dysplastic pulmonary valve stenosis in patients with inaccessible femoral vessels.
一名8.7岁男孩在6岁时出现劳力性呼吸困难伴口唇发绀。血氧饱和度（SpO2）为66%。进行了双向格林分流术（BGS），成功将SpO2提高到88%。不幸的是，他在8.5岁时再次出现劳力性呼吸困难伴明显的口唇发绀。SpO2降至65%。超声心动图显示肺动脉瓣发育不良并伴有严重狭窄。考虑到右心室和肺动脉分支可能生长，由于股静脉难以穿刺，对该男孩通过BGS进行经颈静脉球囊肺动脉瓣成形术（BPV），作为发绀的姑息治疗。经过分级BPV后，肺动脉瓣开口从2.59毫米扩大到6.65毫米，压力阶差从60毫米汞柱降至25毫米汞柱，SpO2升至85%。在12个月的随访中，他活动自如，不再有劳力性呼吸困难。无可争议的是，BGS是一种可供选择的血管通路，通过它可以进行经颈静脉BPV，以改善因股血管难以穿刺的患者中肺动脉瓣发育不良狭窄引起的发绀。
