Xiao Yunbin, Li Wenfeng, Deng Xicheng, Chen Zhi, Peng Yuming, Wang Yefeng, Zeng Yunhong, Xiao Zhenghui
Department of Cardiology, Hunan Children's Hospital.
Academy of Pediatrics, University of South China.
Medicine (Baltimore). 2020 Aug 21;99(34):e21849. doi: 10.1097/MD.0000000000021849.
Patent ductus venosus (PDV) is a rare and critical disease, and the majority of patients present with pulmonary arterial hypertension (PAH) or hepatopulmonary syndrome due to congenital portosystemic shunt. We reported that both PAH and hypersplenism were major complications of PDV in this case. This case report can assist the treatment and recovery of the patients with similar symptoms.
A 4-year-old male patient presented to our institution with a history of recurrent respiratory infections accompanied by leukocytopenia, thrombocytopenia and presented with tachypnoea. upon mild exertion.
A wide communication, 10 mm in diameter, between the portal vein and inferior vena cava was identified in the subcostal echocardiogram and computed tomography images. Echocardiography showed an estimated systolic pulmonary artery pressure of 106 mm Hg. Right-sided cardiac catheterization indicated a mean pulmonary arterial pressure of 30 mm Hg and a pulmonary vascular resistance of 3 Wood units. Chest X-ray revealed cardiomegaly with a prominent pulmonary segment.
The patient was treated with combination pharmacotherapy of bosentan and tadalafil and PDV ligation.
A year later, the boy showed normal exercise tolerance and weight gain. Liver and spleen parameters, liver function, blood cells and the general condition of the boy improved.
Initial combination therapy of bosentan and tadalafil is safe and effective in children with PAH associated with PDV. When PDV banding test shows normal portal pressure, PDV ligation is considered acceptable in children with PAH and hypersplenism associated with PDV.
静脉导管未闭（PDV）是一种罕见且严重的疾病，大多数患者因先天性门体分流而出现肺动脉高压（PAH）或肝肺综合征。我们报道了该病例中PAH和脾功能亢进均为PDV的主要并发症。本病例报告有助于治疗有类似症状的患者并促进其康复。
一名4岁男性患者因反复呼吸道感染病史伴白细胞减少、血小板减少就诊于我院，且轻度活动后出现呼吸急促。
在肋下超声心动图和计算机断层扫描图像中发现门静脉与下腔静脉之间存在直径为10毫米的广泛交通。超声心动图显示估计的收缩期肺动脉压为106毫米汞柱。右心导管检查显示平均肺动脉压为30毫米汞柱，肺血管阻力为3伍德单位。胸部X线显示心脏扩大，肺动脉段突出。
该患者接受了波生坦和他达拉非联合药物治疗以及PDV结扎术。
一年后，该男孩运动耐量正常且体重增加。肝脏和脾脏参数、肝功能、血细胞以及男孩的总体状况均有所改善。
对于与PDV相关的PAH儿童，初始波生坦和他达拉非联合治疗安全有效。当PDV结扎试验显示门静脉压力正常时，对于与PDV相关的PAH和脾功能亢进儿童，PDV结扎术被认为是可以接受的。
