SHAPE of Portal Hypertension in Children

Noninvasive Subharmonic Aided Pressure Estimation 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