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J Thorac Cardiovasc Surg 2004;128:315-316
© 2004 The American Association for Thoracic Surgery

Brief communication

Fontan operation for hypoplastic left heart syndrome with absent aortic valve

^a Department of Pediatric Cardiovascular Surgery, Nagano children's Hospital, Nagano, Japan,
^b Department of Cardiology, Nagano Children's Hospital, Nagano, Japan

Received for publication December 5, 2003; accepted for publication December 24, 2003.

^* Address for reprints: Narutoshi Hibino, MD, 3100 Toyoshina, Toyoshina-cho, Minamiazumi-gun, Nagano 399-8288, Japan
nhhibino@uranus.dti.ne.jp

The first 20% of the full text of this article appears below.

Congenital absence of aortic valve leaflets is an extremely uncommon complication of hypoplastic syndrome. We describe the first successful experience with a Fontan operation after the Norwood and bidirectional Glenn procedures for hypoplastic left heart syndrome with congenital aortic valve absence.

Clinical summary

A 6-year-old boy was referred to our hospital after initially having cyanosis and respiratory distress at birth after an uneventful term pregnancy. On first admission, echocardiography showed mitral valve atresia and an absent aortic valve with a small and thick left ventricular cavity wall. Cardiac catheterization performed at 8 days of age had revealed a hypoplastic left ventricle with severe hypokinesis and severe aortic regurgitation. At 9 days of age, the patient underwent the Norwood procedure with a 4-mm polytetrafluoroethylene graft for a Blalock-Taussig shunt.

In the findings of the Norwood operation, the aortic annulus formed a mild narrowing with completely absent aortic valve. The aortic annulus was not . . . [Full Text of this Article]

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