{
  "abstract": "Hepatopulmonary syndrome (HPS) and Portopulmonary hypertension (PoPH) are both pulmonary complications of advanced liver disease associated with high mortality and poor prognosis. 1 HPS is arterial hypoxaemia (PaO2 <80 kPa) with an increased alveolar-arterial oxygen partial pressure difference (AaPO2 >15) on a background of liver disease2–4 which is a consequence of pulmonary vasodilatation. PoPH is characterised by pulmonary arterial hypertension with co-existing portal hypertension.5 6 The aim of this 20-year retrospective review (2003–2023) is to report our single centre experience to further delineate the clinical presentation, diagnosis, and treatment for children with HPS and PoPH. We identified patients through the database and collected data through evaluation of electronic case records.We identified 7 children (4 female and 3 males; age range 10 months-13 years) with a diagnosis of HPS. Their underlying aetiologies comprised of biliary atresia (2), portal cavernoma (2), veno-occlusive disease (1), non-cirrhotic portal hypertension (1) and peroxisomal biogenesis disorder (1). 4/7 patients presented with dyspnoea and 4/7 showed clinical signs of cyanosis and clubbing at presentation. Oxygen saturations (SpO2) in air ranged from 82–89%. 3/7 patients had congenital cardiac disease. Of the non-cardiac patients, only 1 patient had a normal Chest X-Ray, the others demonstrated coarse bronchovascular markings. For diagnostic confirmation, 6 patients had contrast enhanced echocardiography, which were all positive and demonstrated evidence of shunting. Macroaggregated albumin (MAA) perfusion scanning identified abnormal extra pulmonary shunt fractions (25–73%) in all patients.3 cardiac catheter reports were available for analysis. They demonstrated normal pulmonary artery pressures and pulmonary vascular resistance however, showed significant pulmonary venous desaturation.Of our cohort, 2 patients died prior to being listed for transplant. The remaining 5 patients have undergone successful transplants. They all had resolution of their hypoxaemia before discharge from hospital post-transplant and have remained without supplemental oxygen. Their mean follow-up time is 73 months.We studied 1 female, aged 13 years, with a diagnosis of PoPH. She presented with exertional dyspnoea and syncopal episodes. She had cyanosis on exertion with SpO2 of 96% at rest. The work up for pulmonary hypertension incidentally revealed a portosystemic shunt (Portal vein to Inferior Vena Cava) on ultrasound imaging. Cardiac MRI demonstrated mean pulmonary artery pressure of 43 mmHg at diagnosis which subsequently improved to 22 mmHg with triple combination therapy (oral phosphodiesterase inhibitor, oral endothelin receptor antagonist and inhaled prostacyclin). Currently, she is being consider for portosystemic shunt closure.Transplant remains the only cure for HPS and in this series hypoxaemia resolved in all patients. Patients with the lowest oxygen saturations at presentation had poorer outcomes (i.e death). Further research is required in PoPH to determine efficacious treatment strategies and ascertain the role of liver transplantation in this cohort.HPS and PoPH are rare entities associated with very high levels of mortality if left untreated, hence the need for further research to aid early clinical diagnosis and curative treatment strategies for these patients.References Luo BW, Du ZY. Advances in diagnostic imaging of hepatopulmonary syndrome. Frontiers in Medicine 2022 Jan 10;8:817758.Weinfurtner K, Forde K. Hepatopulmonary syndrome and portopulmonary hypertension: current status and implications for liver transplantation. Current hepatology reports 2020 Jul 11;19(3):174.Anand AC, Mukherjee D, Rao KS, Seth AK. Hepatopulmonary syndrome: prevalence and clinical profile. Indian J Gastroenterol. 2001;20(1):24–7.Rodríguez-Roisin R, Krowka MJ, Hervé Ph, Fallon MB, on behalf of the ERS (European Respiratory society). Highlights of the ERS Task Force on pulmonary-hepatic vascular disorders (PHD). Journal of Hepatology 2005 Jun;42(6):924–7.Jasso-Baltazar EA, Peña-Arellano GA, Aguirre-Valadez J, Ruiz I, Papacristofilou-Riebeling B, Jimenez JV, et al. Portopulmonary hypertension: an updated review. Transplantation Direct 2023 Jul 21;9(8):e1517.Tingo J, Rosenzweig EB, Lobritto S, Krishnan US. Portopulmonary hypertension in children: a rare but potentially lethal and under-recognized disease. Pulmonary Circulation 2017 Jul 31;7(3):712.",
  "authors": [
    {
      "affiliations": [
        "Leeds Children’s Hospital"
      ],
      "name": "Chloe Ashton"
    },
    {
      "affiliations": [
        "Leeds Children’s Hospital"
      ],
      "name": "Kavitha Jayaprakash"
    },
    {
      "affiliations": [
        "Leeds Children’s Hospital"
      ],
      "name": "Marumbo Mtegha"
    },
    {
      "affiliations": [
        "Leeds Children’s Hospital"
      ],
      "name": "Palaniswamy Karthikeyan"
    },
    {
      "affiliations": [
        "Leeds Children’s Hospital"
      ],
      "name": "Sanjay Rajwal"
    }
  ],
  "title": "OC51 Hepatopulmonary syndrome and portopulmonary hypertension in children with portal hypertension: a single-centre experience",
  "uid": "71db33ad-f694-538b-a8cf-7e627902c766"
}