J.Jpn. Surg. Soc.. 102(8): 608-611, 2001

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Feature topic

SURGICAL TREATMENT OF HYPOPLASTIC LEFT HEART SYNDROME

Hypoplastic left heart syndrome (HLHS) was previously uniformaly fatal within the first month of life. The development of the Norwood stage I operation has afforded new hope to the families of newborn infants with this defect. Recent, modification of the Norwood procedure has improved the surgical results. The one-year survival rate after the Norwood procedure is from 50% to 70% in major institutions. The results of second palliation (hemi-Fontan operation or bidirectonal Glenn operation) are nearly acceptable. Recently, fetal echocardiography has allowed early diagnosis of HLHS, and after a prenatal diagnosis of HLHS, couples may be offered termination of the pregnancy. It is necessary to improve the results of the Norwood stage I operation, to save more fetuses and neonates with HLHS.

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