Hepatopulmonary syndrome and intrapulmonary vasodilatation in children with chronic liver diseases

  • Dr Mohammad Borzouee, shiraz university of medical sciences, Iran
  • Dr Gholamhosein Ajami, Iran
  • Abstract
    Heart function, pulmonary artery pressure (PAP) and also pulmonary vascular bed status should be assessed before liver transplantation. The hepato-pulmonary syndrome (HPS) is defined as the triad of liver disease, arterial desaturation, and intrapulmonary vascular dilatation (IPVD). The reported prevalence of HPS in adult cirrhotic patients varies between 4% and 19%, and various threshold values defining arterial desaturation have been used and recommended previously. We studied 114 pediatrics patients with chronic liver diseases (£ 18y) in waiting list for liver transplantation. Conventional and contrast enhanced echocardiography was performed for detection of pulmonary vasodilatation and blood gas analysis for hypoxia. Cardiac function and PAP estimation were normal in all. Sixty patients (52%) had positive contrast enhanced echocardiography which means 52% of patients had IPVD. Twenty five of 71 patients which had arterial blood gas analysis revealed positive contrast enhanced echocardiography with Pao2 <70 mmhg which means 35.5% of our patients fulfilling the criteria of HPS. Conclusion: We concluded which the heart function and PAP were in acceptable range. There was no indirect evidence(s) of pulmonary arterial hypertension. Pulmonary venous dilatation and HPS are common findings in children with chronic liver disease independent of causes. The prevalence seems to be higher among children than those reported in adulthood which may hypothesize more vascular responsiveness or angiogenesis in children..
    Keywords: Cirrhosis, pulmonary hypertension, chronic liver disease. contrast enhanced echocardiography.