Ligation of patent ductus venosus in a child with pulmonary arterial hypertension and hypersplenism A case report
Ligation of patent ductus venosus in a child with pulmonary arterial hypertension and hypersplenism A case report
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DOI：
10.1097/md.0000000000021849
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发表时间：
2020-08-21
期刊：
影响因子：
1.6
通讯作者：
Xiao, Zhenghui
中科院分区：
文献类型：
--
作者：
Xiao, Yunbin;Li, Wenfeng;Xiao, Zhenghui
简介：静脉导管未闭（PDV）是一种罕见的危重疾病，大多数患者因先天性门体分流而表现为肺动脉高压（PAH）或肝硬化综合征。我们报告，肺动脉高压和脾功能亢进都是PDV的主要并发症。本病例报告可为类似症状患者的治疗和康复提供帮助。患者问题：一名4岁男性患者因反复呼吸道感染伴白细胞减少症、血小板减少症和呼吸急促就诊。在轻微的劳累下。诊断：在肋下超声心动图和计算机断层扫描图像中发现门静脉和下腔静脉之间有直径10 mm的广泛交通。超声心动图显示估计的肺动脉收缩压为106 mm Hg。右侧心导管检查显示平均肺动脉压为30 mm Hg，肺血管阻力为3 Wood单位。胸部X线检查显示心脏扩大，肺段突出。干预：患者接受波生坦和他达拉非联合药物治疗和PDV结扎。结果：一年后，男孩表现出正常的运动耐力和体重增加。肝脾参数、肝功能、血细胞和男孩的一般状况有所改善。结论：波生坦和他达拉非初始联合治疗儿童PAH伴PDV安全有效。当PDV带试验显示门静脉压力正常时，认为患有PAH和与PDV相关的脾功能亢进的儿童可以接受PDV结扎。
Introduction: 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. Patient concerns: 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. Diagnosis: 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. Interventions: The patient was treated with combination pharmacotherapy of bosentan and tadalafil and PDV ligation. Outcomes: 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. Conclusion: 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.