International Registry of Congenital Porto Systemic Shunts

• Research type

  Research Database

• IRAS ID

  268730

• Contact name

  Khalid Sharif

• Contact email

  khalid.sharif1@nhs.net

• Research summary

  International Registry of Congenital Porto-Systemic Shunts

• REC name

  East Midlands - Derby Research Ethics Committee

• REC reference

  21/EM/0146

• Date of REC Opinion

  18 Aug 2021

• REC opinion

  Further Information Favourable Opinion

• Data collection arrangements

  The main goals of the registry are to a) characterize the natural history of cPSS b) develop a clear, consensual nomenclature c) identify subjects at risk of developing complications and d) standardize care of patients with cPSS diagnosis, follow-up and treatment.
  Data to be stored includes:
  Medical History
  Porto systemic shunt patient history
  Symptoms and Clinical exam at diagnosis
  Angiography, brain MRI, scintigraphy
  Liver function blood results at diagnosis
  Genetic workup
  Liver nodules follow up (progression of known liver nodule, new liver nodules, number of nodules)
  Complications occurring since last visit (neurologic, cardio-pulmonary, surgical)
  Indication and type of closure
  Surgical details
  Interventional radiology
  cardio-pulmonary complications

• Research programme

  Patient recruitment will be both retrospective and prospective, as nearly 300 cases are currently followed by the participating centers whose data is important to include. Owing to the rarity of the condition, it is important to emphasize the importance of recording both prevalent and incident cases. The potential implications of abnormal porto-venous shunting and decreased hepatic portal flow are numerous and potentially serious. In children, CPSS are increasingly sought in patients presenting with unexplained neurological or psychiatric abnormalities, severe cardiopulmonary complications such as hepatopulmonary syndrome, portopulmonary hypertension and neonatal high-output heart failure, as well as in primary liver tumours. Other less well described associations include growth retardation or overgrowth, nephropathy and coagulation abnormalities. In adults, CPSS have been reported at all ages, and are often interpreted as incidental findings, although in referral centers they are now sought as part of the work up of pulmonary hypertension.

• Research database title

  International Registry of Congenital Porto-Systemic Shunts

• Establishment organisation

  Birmingham Women's and Children's NHS Foundation Trust

• Establishment organisation address

  Steelhouse Lane

  Birmingham

  B4 6NH