Catheter interventional treatment of congenital portosystemic venous shunts in childhood

Knirsch, Walter; Benz, Dominik C.; Bühr, Patrick; Quandt, Daniel; Weber, Roland; Kellenberger, Christian J.; Kretschmar, Oliver

doi:10.1002/ccd.26362

Cited by 38 publications

(46 citation statements)

References 32 publications

(50 reference statements)

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“…Invasive treatment with shunt closure in patients with CPSS is recommended with regard to the severity of complications and its potential reversibility after shunt closure. 8 On the other hand, if there is severe pulmonary hypertension or right heart failure, treatment either by transcatheter or surgical approach should be considered. 8 .…”

Section: Discussionmentioning

confidence: 99%

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Percutaneous embolization of congenital portosystemic venous shunt in an infant with respiratory distress

et al. 2018

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A 5-month-old boy with progressive respiratory distress was admitted to our hospital. Physical examination revealed mild tachypnea and retraction. The left main bronchus was found as severely collapsed between the right pulmonary artery and the descending aorta, on the bronchoscopic evaluation. Further evaluation revealed persistent ductus venosus (PDV). As in the fetal period the ductus venosus arises from the posterior aspect of the left portal vein, a PDV is considered another type of intrahepatic portosystemic shunt. Therefore, he was diagnosed with congenital portosystemic venous shunt, leading to persistent respiratory distress. The PDV was closed with Amplatzer vascular plug II, and then he had immediate clinical improvement. Congenital portosystemic venous shunts (CPSS) are rare vascular malformations associated with severe complications. Here we presented a case with progressive respiratory distress as a result of CPSS and rapid improvement after embolization.

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Section: Discussionmentioning

confidence: 99%

“…8 On the other hand, if there is severe pulmonary hypertension or right heart failure, treatment either by transcatheter or surgical approach should be considered. 8 . Our patient was at risk for high output cardiac failure due to increased venous return caused by the low resistance hepatic circulation.…”

Section: Discussionmentioning

confidence: 99%

Percutaneous embolization of congenital portosystemic venous shunt in an infant with respiratory distress

et al. 2018

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“…Patients usually present with cyanosis, digital clubbing, and dyspnea on exertion or at rest [ 36 ]. Associated portopulmonary hypertension can affect 13–66% of children with hepatopulmonary syndrome and CPSS and usually appears later in the course of the disease [ 7 , 37 , 38 ]. The histological picture is consistent with obliteration of pulmonary arteries with microthrombi and intimal fibrosis.…”

Section: Clinical Picturementioning

confidence: 99%

Congenital portosystemic venous shunt

2017

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Congenital portosystemic venous shunts are rare developmental anomalies resulting in diversion of portal flow to the systemic circulation and have been divided into extra- and intrahepatic shunts. They occur during liver and systemic venous vascular embryogenesis and are associated with other congenital abnormalities. They carry a higher risk of benign and malignant liver tumors and, if left untreated, can result in significant medical complications including systemic encephalopathy and pulmonary hypertension. Conclusion: This article reviews the various types of congenital portosystemic shunts and their anatomy, pathogenesis, symptomatology, and timing and options of treatment. What is Known: • The natural history and basic management of this rare congenital anomaly are presented. What is New: • This paper is a comprehensive review; highlights important topics in pathogenesis, clinical symptomatology, and treatment options; and proposes an algorithm in the management of congenital portosystemic shunt disease in order to provide a clear idea to a pediatrician. An effort has been made to emphasize the indications for treatment in the children population and link to the adult group by discussing the consequences of lack of treatment or delayed diagnosis.

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“…Catheter intervention of Abernethy malformation is safe and effective. 5 Pre-procedure planning of closure of Abernethy malformation in heterotaxy syndrome requires detailed study Cardiology in the Young of venous anatomy, as these patients may have other venous anomalies as well. The vascular plug has to be meticulously placed to avoid occluding any systemic vein.…”

Section: Case Reportmentioning

confidence: 99%

Haemodynamic consequences following closure of an Abernethy malformation in a patient following a total cavopulmonary shunt

2018

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A 17-year-old girl with situs ambiguous, hypoplastic right ventricle with a large ventricular septal defect, and severe pulmonary stenosis had undergone Kawashima operation 10 years back. She had significant desaturation because of a large Abernethy malformation, with reverse shunting from the inferior caval vein to the portal vein. It was closed with a vascular plug, with improvement in oxygen saturation. She developed extensive inferior caval vein thrombus following the procedure, which was managed conservatively by anti-coagulation.

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Catheter interventional treatment of congenital portosystemic venous shunts in childhood

Cited by 38 publications

References 32 publications

Percutaneous embolization of congenital portosystemic venous shunt in an infant with respiratory distress

Percutaneous embolization of congenital portosystemic venous shunt in an infant with respiratory distress

Congenital portosystemic venous shunt

Haemodynamic consequences following closure of an Abernethy malformation in a patient following a total cavopulmonary shunt

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