SHAPE of Portal Hypertension in Children

Early diagnosis of portal hypertension is difficult as symptoms rarely manifest until the later stages of liver disease. Both cirrhotic and non-cirrhotic portal hypertension can result in life-threatening complications, the most frequent of which is bleeding from esophageal varices. In children, variceal bleeds are associated with mortality rates of 1-3 %, while life-threatening complications have been reported in up to 20 % of children with cirrhosis. Despite the high incidence of portal hypertension in children with liver disease, a noninvasive modality to monitor disease progression and risk of complications is currently lacking. Hence, this trial will investigate the safety and efficacy of subharmonic aided pressure estimation (SHAPE) as a noninvasive ultrasound technique for diagnosing portal hypertension in children.

Inclusion Criteria: * Patients with a diagnosis of chronic liver disease without portal hypertension. * Patients with a diagnosis of chronic liver disease with portal hypertension. Exclusion Criteria: * Subjects who are pregnant. * Patients with known or suspected hypersensitivity to egg phosphatidyl serine or with a history of anaphylactic allergy to eggs or egg products. * Subjects with allergy to egg products or other components of the ultrasound contrast agents will be excluded. * History of allergic reaction to Lumason®, sulfur hexafluoride, sulfur hexafluoride lipid microsphere components, or other ingredients in Lumason (polyethylene glycol, distearoylphosphatidylcholine (DSPC), dipalmitoylphosphatidylglycerol sodium (DPPG-Na), palmitic acid) * History of allergic reaction to Sonazoid * Patients with biliary atresia with asplenia or polysplenia. * Patients with prior liver transplant. * Patients with cystic fibrosis. * Patients with chronic lung disease. * Patients with portal vein thrombosis, cavernous transformation of the portal vein or absent portal vein. * Adults not competent/impaired. * Patients with significant heart disease or severe congenital heart disease