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

被引:0
作者
Shigefuku, Ryuta [1 ]
Iwasa, Motoh [1 ]
Yoshikawa, Kyoko [1 ]
Tanaka, Hideaki [1 ]
Tamai, Yasuyuki [1 ]
Eguchi, Akiko [1 ]
Sato, Toru [2 ]
Ogihara, Yoshito [2 ]
Dohi, Kaoru [2 ]
Nakagawa, Hayato [1 ]
机构
[1] Mie Univ, Dept Gastroenterol & Hepatol, Grad Sch Med, 2-174 Edobashi, Tsu 5148507, Japan
[2] Mie Univ, Grad Sch Med, Dept Cardiol & Nephrol, Tsu, Mie, Japan
关键词
Idiopathic portal hypertension; Splenectomy; Portopulmonary hypertension; Pulmonary arterial hypertension; Macitentan; PREVALENCE;
D O I
10.1007/s12328-024-02072-0
中图分类号
R57 [消化系及腹部疾病];
学科分类号
摘要
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.
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收藏
页码:130 / 136
页数:7
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