{
  "abstract": "This report details the first UK-based experience of closing congenital portosystemic shunts (CPSS) using intravascularly deployed cardiac closure devices. The procedure, performed by interventional radiologists at a single specialist centre, involved five paediatric patients between January 2019 and September 2024.CPSS are rare vascular malformations resulting in the bypass of hepatic sinusoidal blood flow.1 2 They can be classified as being extra- or intra-hepatic, and further described by the connecting vessels.1 2 Complications are secondary to physiological compounds evading metabolism within the liver.3 4 This can result in significant morbidity including varying degrees of neurocognitive impairment, hepato-pulmonary syndrome, pulmonary hypertension, liver tumours and endocrine abnormalities.3 4 Approaches to closure were traditionally surgical and have progressed to less invasive methods with interventional radiology avoiding morbidity associated with laparotomy.2 In selected cases, CPSS were deemed unsuitable for endovascular closure with standard available vascular devices. Following careful multidisciplinary team discussion, we considered the use of cardiac septal occluder devices that would best suit the anatomy of the CPSS and avoid laparotomy. We made an application for a new interventional procedure for off-label use of cardiac devices in these patients. Patient families were appropriately consented.Four of the patients received an atrial septal defect occluder and one received a ventricular septal defect occluder. The devices ranged from 9 mm to 27 mm in diameter.Table 1 summarising patient data:Abstract OC9 Table 1Age at diagnosis (median) 4 years 6 months Age at device use (median) 13 years 4 months Time between diagnosis and closure (median) 6 years 3 months Clinical presentation of CPSS 3 were incidentally diagnosed1 had conjugated hyperbilirubinaemia1 had a liver tumour Number of shunts All had 1 shunt Shunt anatomy 4 had main portal vein to inferior vena cava (side to side connection)1 had main portal vein to right atrium Liver nodules 4 of 5 had liver nodules (1–5 in number) Cardiac comorbidities Atrial septal defect, cardiomegaly, patent ductus arteriosus, enlarged superior vena cava, mild aortic stenosis and tiny patent foramen ovale Other comorbidities Failure to thrive, poor concentration, attention deficit hyperactivity disorder, developmental delay, premature adrenarche, polycystic ovarian syndrome, hyperandrogenism and insulin resistance Single stage closure 3 of 5 Anticoagulation All patients received subcutaneous heparin post operatively1 case that suffered thrombosis received warfarin for 2 years3 cases stopped heparin by 1 year of follow up Complications 1 patient had device migration, twice1 had thrombosis Outcome 1 patient remains untreated (device migration)4 are off anticoagulation and well. These cases highlight the feasibility of using cardiac closure devices in CPSS with anatomy that may seem unsuitable for endovascular closure. Although there have been challenges in initiating a new technique, overall results have been promising and are improving with increased experience.References Korff S, Mostaguir K, Beghetti M, et al. International registry of congenital porto-systemic shunts: a multi-centre, retrospective and prospective registry of neonates, children and adults with congenital porto-systemic shunts. Orphanet Journal of Rare Diseases 2022;17:284.McLin VA, Franchi-Abella S, Brütsch T, et al. Expert management of congenital portosystemic shunts and their complications. JHEP Reports 2024;6.Sokollik C, Bandsma RHJ, Gana JC, et al. Congenital portosystemic shunt: characterization of a multisystem disease. J Pediatr Gastroenterol Nutr. 2013;56:675–81.Bahadori A, Kuhlmann B, Debray D, et al. Presentation of congenital portosystemic shunts in children. Children 2022;9:243.",
  "authors": [
    {
      "affiliations": [
        "Leeds Children’s Hospital"
      ],
      "name": "David Wood"
    },
    {
      "affiliations": [
        "Leeds Children’s Hospital"
      ],
      "name": "Chloe Ashton"
    },
    {
      "affiliations": [
        "Leeds Children’s Hospital"
      ],
      "name": "Jai Patel"
    },
    {
      "affiliations": [
        "Leeds Children’s Hospital"
      ],
      "name": "Sanjay Rajwal"
    },
    {
      "affiliations": [
        "Leeds Children’s Hospital"
      ],
      "name": "Marumbo Mtegha"
    }
  ],
  "title": "OC9 Closure of hepatic congenital portosystemic shunts using cardiac closure devices: a case series",
  "uid": "054a50f3-9660-508c-91d7-e5f3974be3bf"
}