Introduction
Alagille syndrome is an autosomal dominant disease caused by pathogenic mutations in the JAG1 and NOTCH genes. Multiple system involvement is seen in Alagille syndrome. The most frequently involved system is the hepatobiliary system and is seen as a result of insufficiency of intrahepatic bile ducts. In addition, abnormalities can be observed in the cardiac, vascular, ocular, renal system and skeletal muscles.
Pulmonary hypertension may be present and it can be associated with congenital defects such as pulmonary valve stenosis or Fallot tetralogy.
In this case report we described aortic valve infective endocarditis in a patient with Alagille syndrome and group 1 pulmonary hypertension without any cardiac defect.