Middle-Aged Case of Congenital Portosystemic Venous Shunt Complicated With Multiple Visceral Arterial Aneurysms and Aorto-Pulmonary Collateral Vessels
Middle-Aged Case of Congenital Portosystemic Venous Shunt Complicated With Multiple Visceral Arterial Aneurysms and Aorto-Pulmonary Collateral Vessels
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先天性门体静脉分流术并发多发内脏动脉瘤及主肺侧支血管的中年病例
DOI：
10.1161/circimaging.122.013969
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发表时间：
2022
期刊：
影响因子：
--
通讯作者：
Komuro Issei
中科院分区：
文献类型：
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作者：
Goto Kohsaku;Nakayama Yukiteru;Saito Akihito;Minatsuki Shun;Takeda Norifumi;Akamatsu Nobuhisa;Hatano Masaru;Komuro Issei
1例48岁男性因特发性肺动脉高压(PAH)被转诊至本院接受随访治疗。他从小就感到劳累呼吸困难，11岁时体检发现胸部X光和心电图异常，需要进一步评估。右心导管术导致了特发性PAH的诊断，尽管当时的诊断过程不能完全遵循。从那时起，他服用了10微克的贝拉前列素，两年前每天增加1.0毫克的利奥古特。他既没有相关的家族史，也没有其他病史。他的病情符合世界卫生组织功能分级II级。10年前的右心导管术研究显示，他的心输出量高达10.3 L/分钟，平均肺动脉压升高33毫米汞柱。他因重新评估高动力循环状态而入院。入院时，脚趾有棍棒状(图S1)，动脉血气分析显示轻度低氧血症，肺泡-动脉氧分压差增加(表1)。心电图显示V1~V2导联R波幅度高，胸片显示双侧肺动脉增粗(图S2)。超声心动图显示双室功能和容量正常，而三尖瓣返流压差轻微升高至29.4 mm Hg。血液分析显示低蛋白血症、血小板减少和凝血因子水平降低表明肝脏受损，并伴有氨血症和Fisher比率降低(表1)。虽然未发现拍打震颤，但可观察到言语迟缓和运动迟缓。与theta波对应的脑电背景活动减慢支持肝性脑病的临床表现。肝功能Child-Pugh分级B级，门静脉主干引流至下腔静脉，肝动脉代偿性扩张(图S3)。计算机断层扫描显示门腔静脉分流，肝内门静脉缺失。单个肝外血管连接门静脉主干和下腔静脉，导致诊断为1b型先天性门体静脉分流(CPVS；图1)。头部磁共振成像显示与他年龄相仿的轻度脑萎缩和T1加权图像上苍白球的高信号，推测是门体分流引起的锰沉积(图S4)。右心导管术显示平均肺动脉压为33 mm Hg，肺毛细血管楔压为12 mm Hg。用FICK法测得的心输出量为8.1 L/分钟，肺血管阻力为2.6wood单位，体循环阻力为10.0wood单位(表2)。~(99)T大聚集白蛋白肺灌注扫描
A 48-year-old male was referred to our hospital for a follow-up care of idiopathic pulmonary arterial hypertension (PAH). He had felt exertional dyspnea since childhood, and a medical checkup at the age of 11 revealed the abnormalities of chest X-ray and ECG which necessitated further evaluation. Right heart catheterization led to a diagnosis of idiopathic PAH, although the diagnostic process at that time could not be fully followed. He had taken beraprost 10 µg since then, and a daily 1.0 mg dose of riociguat was added 2 years ago. He had neither relevant family history nor other medical history. His condition corresponded to World Health Organization functional classification class II. The right heart catheterization study a decade ago revealed a high cardiac output of 10.3 L/min with elevated mean pulmonary arterial pressure of 33 mm Hg. He was admitted for the reassessment of hyperdynamic circulatory state. On admission, clubbing of toes was present (Figure S1) and arterial blood gas analysis showed mild hypoxemia with increased alveolar-arterial oxygen pressure difference (Table 1). An ECG showed high amplitude of R wave in leads V1 to V2, and a chest X-ray revealed enlarged bilateral pulmonary arteries (Figure S2). Echocardiography showed normal biventricular function and volumes, whereas tricuspid regurgitation pressure gradient was slightly elevated to 29.4 mm Hg. Blood analysis revealed hypoalbuminemia, thrombocytopenia, and reduced levels of coagulation factors indicating liver damage, accompanied by ammonemia and a decreased Fisher ratio (Table 1). Although flapping tremor was not found, slowness of speech and bradykinesia were observed. Slowing of electroencephalography background activity corresponding to theta waves supported a clinical presentation of hepatic encephalopathy. Hepatic impairment with Child-Pugh score B was investigated by abdominal ultrasonography, which demonstrated the main trunk of portal vein draining into the inferior vena cava and compensatory dilation of the hepatic arteries (Figure S3). Computed tomography scan demonstrated a portocaval shunt and absence of the intrahepatic portal veins. A single extrahepatic vessel connecting the main branch of the portal vein to inferior vena cava led to the diagnosis of type 1b congenital portosystemic venous shunt (CPVS; Figure 1). Head magnetic resonance imaging revealed mild cerebral atrophy for his age and high signal intensity in the globus pallidum on T1-weighted images presumably attributed to manganese deposition resulting from the portal-systemic shunting (Figure S4). Right heart catheterization revealed that mean pulmonary arterial pressure and pulmonary capillary wedge pressure were 33 mm Hg and 12 mm Hg, respectively. The cardiac output measured by Fick method was 8.1 L/min, and pulmonary and systemic vascular resistance was 2.6 and 10.0 wood units, respectively (Table 2). Technetium-99 m macroaggregated albumin lung perfusion scan demonstrated