Characterization and natural history of congenital intrahepatic portosystemic shunts

Cytter-Kuint, Ruth; Slae, Mordechai; Kvyat, Karina; Shteyer, Eyal

doi:10.1007/s00431-021-03949-9

Cited by 13 publications

(21 citation statements)

References 15 publications

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“…In their cases, except for one foetus in which the left portal vein could not be identified, the starting position of the shunt in all other cases was located in the left portal vein. Cytter-Kuint et al studied IHPSS in 15 children, of which 14 shunts were located between the LPV and hepatic vein, and only one case was located between the RPV and RHV [ 14 ]. However, none of these studies have discussed this feature further.…”

Section: Discussionmentioning

confidence: 99%

Prenatal ultrasonographic features and follow-up outcomes of 19 cases of congenital intrahepatic portosystemic venous shunts diagnosed during the foetal period

Zhu

Xia

et al. 2022

Insights Imaging

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Background To investigate the prenatal ultrasonographic features and case characteristics of the congenital intrahepatic portosystemic venous shunt (IHPSS) diagnosed during the foetal period and analyse its prognosis. Methods We conducted a retrospective cohort study of patients diagnosed with IHPSS between 2016 and 2021. IHPSS was defined as an abnormal connection between the foetal intrahepatic portal and the hepatic veins. Results In this study, 19 foetuses were identified, including 12 cases of single shunt and 7 cases of multiple shunts, with a gestational age of 33.8 ± 4.5 (range 25–40) weeks at diagnosis. In the single-shunt group, the origin position of the shunts was all from the left branch of the portal vein (LPV), whereas in the multiple-shunt group, the origin position of the shunts was from the LPV in six cases. Common concomitant intrauterine abnormalities of IHPSS include foetal growth restriction (47.4%) and foetal cardiac enlargement (21.1%). The postnatal manifestations of IHPSS include biochemical abnormalities (increased gamma-glutamyl transferase and bilirubin levels), neonatal hypoglycaemia, neonatal hyperammonaemia, pulmonary hypertension, multiple intrahepatic hyperechoic nodules, and cutaneous haemangiomas. Spontaneous closure of shunts occurred in ten cases, and the mean time to shunt closure was 8.1 months (1–28 months). Conclusions Most IHPSS found during the foetal period is located in the left branch of the portal vein, and the gestational age at diagnosis is usually in the late second or third trimester. Spontaneous closure of shunts can occur in most live births, and the prognosis is good.

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Section: Discussionmentioning

confidence: 99%

Prenatal ultrasonographic features and follow-up outcomes of 19 cases of congenital intrahepatic portosystemic venous shunts diagnosed during the foetal period

Zhu

Xia

et al. 2022

Insights Imaging

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“…The cause of CPSVS remains unclear; however, it may arise from incomplete vascular remodeling between the fetal hepatic and perihepatic circulation during gestation. 6 , 7 The potential complications caused by long-term shunting can be serious, including hepatic encephalopathy (HE), hepatopulmonary syndrome, and portopulmonary hypertension. 4 …”

Section: Discussionmentioning

confidence: 99%

Balloon-occluded retrograde transvenous obliteration for treatment of congenital intrahepatic portosystemic venous shunt: A case report

Lin

Fan

et al. 2023

Journal of Interventional Medicine

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“…A congenital portal-systemic venous shunt can accelerate the circulation time of the portal and hepatic veins and shorten the HVAT, HA-HVAT, and PV-HVTT. [ 3 , 4 ] Some patients can be diagnosed with a congenital portal-systemic shunt through prolongation of the hepatic vein imaging phase when no clear connection lesions are seen. [ 14 ] Multimodal imaging techniques helped perform a detailed analysis of the case, which led to an accurate diagnosis of the disease.…”

Section: Discussionmentioning

confidence: 99%

“…[ 3 ] The characteristics and natural course of intrahepatic malformations vary in these patients. [ 4 ] A spontaneous intrahepatic portosystemic venous shunt is also rare, with an extremely low incidence in adults. [ 5 ] Herein, we report a patient with PVA and intrahepatic portosystemic venous shunt, diagnosed using multimodal imaging techniques.…”

Section: Introductionmentioning

confidence: 99%

Portal vein aneurysm combined with intrahepatic portosystemic venous shunt diagnosed by multimodal imaging techniques: A case report

Liu

Liu²,

Guo

2022

Medicine

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Introduction: Portal vein aneurysms (PVA) and intrahepatic portosystemic venous shunts are rarely diagnosed clinically. Patient Concerns: A 75-year-old female was admitted to our hospital for evaluation of significant weight loss, diabetes, and an irregularly shaped cystic lesion in the left lateral lobe of the liver. Diagnosis: The patient was diagnosed with a portal vein aneurysm combined with an intrahepatic portosystemic venous shunt using multiple imaging techniques. Interventions: The patient had no relevant clinical symptoms of PVA with concurrent intrahepatic portosystemic venous shunt; hence, no interventions were performed. Ultrasonography was suggested to be performed every 3 months. Outcomes: The patient did not visit the hospital after discharge; however, 4 telephonic follow-up evaluations showed that the patient was well. Lessons: Multimodal imaging techniques should be used to evaluate the source of blood flow, presence or absence of shunts, and the course, number, and location of the shunts to prevent misdiagnosis of this disease.

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Characterization and natural history of congenital intrahepatic portosystemic shunts

Cited by 13 publications

References 15 publications

Prenatal ultrasonographic features and follow-up outcomes of 19 cases of congenital intrahepatic portosystemic venous shunts diagnosed during the foetal period

Prenatal ultrasonographic features and follow-up outcomes of 19 cases of congenital intrahepatic portosystemic venous shunts diagnosed during the foetal period

Balloon-occluded retrograde transvenous obliteration for treatment of congenital intrahepatic portosystemic venous shunt: A case report

Portal vein aneurysm combined with intrahepatic portosystemic venous shunt diagnosed by multimodal imaging techniques: A case report

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