Effective shunt closure for pulmonary hypertension and liver dysfunction in congenital portosystemic venous shunt
Effective shunt closure for pulmonary hypertension and liver dysfunction in congenital portosystemic venous shunt
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DOI：
10.1002/ppul.23944
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发表时间：
2018-04-01
影响因子：
3.1
通讯作者：
Ohga, Shouichi
中科院分区：
文献类型：
--
作者：
Uike, Kiyoshi;Nagata, Hazumu;Ohga, Shouichi
先天性门体静脉分流（CPSVS）是一种罕见的血管畸形，具有肺动脉高压（PAH）的高死亡风险，但CPSVS闭合的治疗结果仍然难以捉摸。我们的目的是调查的临床特征，并建立最佳的管理CPSVS或没有PAH.MethodsTwenty-four患者在九州大学医院治疗1990年和2015年之间的CPSVS被纳入本研究。患者分为PAH组（n=9）和非PAH组（n=15）。临床特点和结果进行了evaluated.ResultsThe第一个表现的CPSVS在诊断（28.5 [1-216]个月）是高半乳糖血症13（54%）或PAH 6（25%）例。PAH是三例死亡的原因。PAH组的血清总胆汁酸、锰和总胆红素水平高于非PAH组，沿着肺血管阻力指数（PVRI）也高于非PAH组（7.2 [5.1-38.1] vs 1.2 [0.5-3.3]单位/m2，P
ObjectiveCongenital portosystemic venous shunt (CPSVS) is a rare vascular malformation with a high risk of mortality from pulmonary arterial hypertension (PAH), but the treatment outcome of CPSVS closure remains elusive. Our aim was to investigate the clinical features and establish the optimal management of CPSVS with or without PAH.MethodsTwenty-four patients with CPSVS treated in Kyushu University Hospital between 1990 and 2015 were enrolled in this study. The patients were divided into a PAH group (n=9) and a non-PAH group (n=15). Clinical characteristics and outcomes were evaluated.ResultsThe first manifestation of CPSVS at diagnosis (28.5 [1-216] months) was hypergalactosemia in 13 (54%) or PAH in six (25%) patients. PAH was the cause of all three deaths. The PAH group had higher levels of serum total bile acid, manganese, and total bilirubin, along with higher pulmonary vascular resistance index (PVRI) than the non-PAH group (7.2 [5.1-38.1] vs 1.2 [0.5-3.3] unit/m(2), P