Portopulmonary Hypertension Associated with Congenital Absence of the Portal Vein Treated with Bosentan

Hino, Tomoko; Hayashida, Akihiro; Okahashi, Nobuyuki; Wada, Nozomi; Watanabe, Nozomi; Obase, Kikuko; Neishi, Yoji; Kawamoto, Takahiro; Okura, Hiroyuki; Yoshida, Kiyoshi

doi:10.2169/internalmedicine.48.1715

Cited by 17 publications

(8 citation statements)

References 12 publications

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“…CEPS is a rare congenital malformation characterized by the partial or complete absence of the portal vein and subsequent development of an extrahepatic portosystemic shunt ( 1 ). Patients with this malformation experience various symptoms including nausea, fatigue, epigastric pain, anorexia, jaundice, encephalopathy due to hyperammonemia ( 4 ), hepatopulmonary syndrome (HPS) ( 5 ), and PoPH ( 6 - 8 ). The age at the diagnosis ranges from prenatal to 84 years, with 66% of patients diagnosed before 12 years of age ( 9 ).…”

Section: Discussionmentioning

confidence: 99%

An Adult Case of Congenital Extrahepatic Portosystemic Shunt Successfully Treated with Balloon-occluded Retrograde Transvenous Obliteration

et al. 2021

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A 42-year-old woman visited our hospital due to syncope. Contrast-enhanced CT revealed portosystemic shunt, portal vein hypoplasia, and multiple liver nodules. The histological examination of a liver biopsy specimen exhibited portal vein hypoplasia and revealed that the liver tumor was positive for glutamine synthetase. The patient was therefore diagnosed with congenital extrahepatic portosystemic shunt type II, and with focal nodular hyperplasia (FNH)-like nodules. She had the complication of severe portopulmonary hypertension and underwent complete shunt closure by balloon-occluded retrograde transvenous obliteration (B-RTO). The intrahepatic portal vein was well developed at 1 year after B-RTO, and multiple liver nodules completely regressed. Her pulmonary hypertension also improved.

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Section: Discussionmentioning

confidence: 99%

An Adult Case of Congenital Extrahepatic Portosystemic Shunt Successfully Treated with Balloon-occluded Retrograde Transvenous Obliteration

et al. 2021

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“…Pulmonary hypertension has been reported in 30 children at ages ranging from the neonatal period to 15 years (mean: 5 years 4 months) with all anatomic types of shunt. 24,35,42,50,51,58,61,99,101,107,109,110,114,118,127,129,130,133,138 Signs of pulmonary hypertension were the occasion of the diagnosis of CPSS in 19 instances, and consisted of dyspnea, fainting, screening by clinical examination or echocardiography, or rapid right heart failure leading to death. The latter was observed in two children aged 12 and 20 months, who had extrahepatic shunts.…”

Section: Pulmonary Hypertensionmentioning

confidence: 99%

Congenital Portosystemic Shunts in Children: Recognition, Evaluation, and Management

et al. 2013

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Congenital portosystemic shunts are present in one in 30,000 children. Among the associated risks of severe complications are neonatal cholestasis, benign and malignant liver tumors, hepatopulmonary syndrome, portopulmonary hypertension, and encephalopathy. They can be detected on prenatal ultrasonograms, during the investigation of a positive galactosemia screening test in neonates or of a complication, or be found fortuitously on an abdominal ultrasound. Small intrahepatic shunts may resolve spontaneously within one year of age, but other shunts such as extrahepatic, persistent ductus venosus or persisting intrahepatic shunts, must be closed in one or two steps, by interventional radiology techniques or surgically. The plasticity of the intrahepatic portal system allows revascularization of the liver after shunt closure, even when no intrahepatic portal structures can be detected on imaging studies. This leaves little or no place for liver transplantation in the management of these children.

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“…Excessive involution can lead to complete or partial absence of the portal system [ 6 ]. As a result, mesenteric and splenic venous blood drains into renal veins, hepatic veins, or directly into the IVC, resulting in poor perfusion of the liver [ 2 , 7 , 8 ]. CAPV is often associated with hepatic tumors.…”

Section: Discussionmentioning

confidence: 99%

Computed tomography and magnetic resonance imaging of multiple focal nodular hyperplasias of the liver with congenital absence of the portal vein in a Chinese girl: case report and review of the literature

Zhang

Wang

et al. 2014

Eur J Med Res

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BackgroundPatients with congenital absence of the portal vein (CAPV) often suffer from additional medical complications such as hepatic tumors and cardiac malformations.Case presentationCongenital absence of the portal vein (CAPV) is a rare malformation. We present a case of a 16-year-old Chinese girl with CAPV with multiple pathology-proven hepatic focal nodular hyperplasias (FNHs) and ventricular septal defect (VSD). The CT and MRI features of this case are described, and previously reported cases are reviewed.ConclusionsCAPV is a rare congenital anomaly and in such patients, clarifying the site of portosystemic shunts, liver disease, and other anomalies is critical for appropriate treatment selection and accurate prognosis determination. Close follow-up, including laboratory testing and radiologic imaging, is recommended for all CAPV patients.

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Portopulmonary Hypertension Associated with Congenital Absence of the Portal Vein Treated with Bosentan

Cited by 17 publications

References 12 publications

An Adult Case of Congenital Extrahepatic Portosystemic Shunt Successfully Treated with Balloon-occluded Retrograde Transvenous Obliteration

An Adult Case of Congenital Extrahepatic Portosystemic Shunt Successfully Treated with Balloon-occluded Retrograde Transvenous Obliteration

Congenital Portosystemic Shunts in Children: Recognition, Evaluation, and Management

Computed tomography and magnetic resonance imaging of multiple focal nodular hyperplasias of the liver with congenital absence of the portal vein in a Chinese girl: case report and review of the literature

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