A rare case of idiopathic portal hypertension with portopulmonary hypertension occurred following splenectomy with a change in portal hemodynamics.

IF 0.8 Q4 GASTROENTEROLOGY & HEPATOLOGY
Ryuta Shigefuku, Motoh Iwasa, Kyoko Yoshikawa, Hideaki Tanaka, Yasuyuki Tamai, Akiko Eguchi, Toru Sato, Yoshito Ogihara, Kaoru Dohi, Hayato Nakagawa
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引用次数: 0

Abstract

A 22-year-old female was referred to our hospital due to thrombocytopenia and esophagogastric varices (EGV) [LmF2CbRC1, Lg-c,F1RC0], therefore we performed endoscopic variceal ligation. Dynamic abdominal computed tomography showed giant portosystemic shunts (PSSs) from the left gastric vein to the superior vena cava and splenomegaly despite normal hepatic contour. Blood tests showed thrombocytopenia and hypoalbuminemia, but there were no abnormalities in hepatic function. Retrograde hepatic venography and transjugular liver biopsy were subsequently performed in order to further examine liver pathology. These examinations revealed anastomosis between the right and middle hepatic veins, with no features to suggest cirrhosis, therefore diagnosed as idiopathic portal hypertension. Splenectomy was performed for the treatment of hypersplenism with thrombocytopenia. Nine months after undergoing a splenectomy, the patient consulted a cardiologist due to exertional dyspnea with WHO functional class II. Echocardiography revealed a mild dilatated right ventricle (RV) with an estimated systolic pressure of 55 mmHg, consistent with pulmonary hypertension. Right heart catheterization determined an increased mean pulmonary arterial pressure of 40 mmHg and pulmonary vascular resistance of 7.5 wood units, but a normal pulmonary capillary wedge pressure value of 7 mmHg, resulting in the diagnosis of portopulmonary hypertension (PoPH). Administration of oral macitentan 5 mg/day was initiated. Exertional dyspnea and the findings from right heart catheterization were improved with macitentan 10 mg/day. No report exists of PoPH occurring within one year after splenectomy, however we report here a very rare case in which a splenectomy brought about the onset of PoPH.

一名22岁女性因血小板减少和食管胃静脉曲张(EGV)[LmF2CbRC1, Lg-c,F1RC0] 转诊至我院,因此我们为其实施了内镜下静脉曲张结扎术。腹部动态计算机断层扫描显示,尽管肝脏轮廓正常,但从左胃静脉到上腔静脉存在巨大的门静脉分流(PSS)和脾脏肿大。血液检查显示血小板减少和低白蛋白血症,但肝功能未见异常。为了进一步检查肝脏病理,随后对患者进行了逆行肝静脉造影和经颈静脉肝活检。这些检查发现肝右静脉和肝中静脉之间有吻合口,但没有肝硬化的特征,因此被诊断为特发性门静脉高压症。为了治疗脾功能亢进伴血小板减少症,他接受了脾切除术。接受脾脏切除术九个月后,患者因劳力性呼吸困难(WHO功能分级为II级)就诊于心脏病专家。超声心动图显示右心室(RV)轻度扩张,估计收缩压为 55 mmHg,与肺动脉高压一致。右心导管检查确定平均肺动脉压升高至 40 mmHg,肺血管阻力为 7.5 wood 单位,但肺毛细血管楔压值正常,为 7 mmHg,因此诊断为门静脉肺动脉高压(PoPH)。开始口服马西替坦 5 毫克/天。每天服用 10 毫克马西替坦后,劳累性呼吸困难和右心导管检查结果均有所改善。目前还没有关于脾切除术后一年内出现 PoPH 的报告,但我们在此报告了一个非常罕见的病例,即脾切除术后出现 PoPH。
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来源期刊
Clinical Journal of Gastroenterology
Clinical Journal of Gastroenterology GASTROENTEROLOGY & HEPATOLOGY-
CiteScore
2.00
自引率
0.00%
发文量
182
期刊介绍: The journal publishes Case Reports and Clinical Reviews on all aspects of the digestive tract, liver, biliary tract, and pancreas. Critical Case Reports that show originality or have educational implications for diagnosis and treatment are especially encouraged for submission. Personal reviews of clinical gastroenterology are also welcomed. The journal aims for quick publication of such critical Case Reports and Clinical Reviews.
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