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Re-endothelialization of the aortic root in infants with hypoplastic left heart syndrome can be delayed by the use of prostaglandin E1 (PGE1) as a sole right ventricular volume expander. Re-endothelialization is important for the patency of the right ventricular outflow tract and the bidirectional cavopulmonary shunt that connects the right atrium and left atrium. However, re-endothelialization has not been studied in infants with severe pulmonary hypertension. The purpose of this study was to determine whether re-endothelialization of the aortic root occurs in infants with severe pulmonary hypertension as well as in infants with hypoplastic left heart syndrome. For this purpose we evaluated re-endothelialization of the aortic root in infants with hypoplastic left heart syndrome and severe pulmonary hypertension, who had not received PGE1 for at least 2 weeks, compared with that in infants with hypoplastic left heart syndrome who had not received PGE1 for at least 4 weeks. As a control, the endocardial surface of the aortic root was evaluated in infants with normal pulmonary artery pressure and in a group of infants with normal pulmonary pressure who had been intubated for 2 to 8 hours. Samples were obtained from the left ventricular outflow tract and the aortic root in infants with hypoplastic left heart syndrome and severe pulmonary hypertension. 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