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Right arrow Congenital - cyanotic

Ann Thorac Surg 2004;78:650-657
© 2004 The Society of Thoracic Surgeons

Original article: cardiovascular

Mid-Term results for double inlet left ventricle and similar morphologies: timing of Damus-Kaye-Stansel

Andrew J. B. Clarke, MBBS, FRACSa, Shingo Kasahara, MDa, David R. Andrews, MBBS FRACSa, Stephen G. Cooper, MBChB, FRACPa, Ian A. Nicholson, MBBS, FRACSa, Richard B. Chard, MBBS, FRACSa, Graham R. Nunn, MBBS, FRACSa, David S. Winlaw, MBBS, MDa*

a The Children's Hospital at Westmead, Adolph Basser Cardiac Institute, Westmead NSW, Australia

Accepted for publication January 7, 2004.

* Address reprint requests to Dr Winlaw, Cardiac Surgery Research, The Children's Hospital at Westmead, Locked Bag 4001, Westmead NSW 2145, Australia
e-mail: davidw{at}chw.edu.au

BACKGROUND: Patients with double inlet left ventricle/l-transposition and similar morphologies have their systemic outflow traverse a bulboventricular foramen (BVF), which has a propensity to narrow over time. A Norwood procedure may be performed as the initial palliation. We prefer aortic arch repair and pulmonary artery banding, delaying Damus-Kaye-Stansel (DKS) or BVF resection until the second palliation. The aims of this study were to compare our results with those reported for Norwood strategy and examine the development of systemic outflow obstruction.

METHODS: Retrospective study of patients with double inlet left ventricle, L-TGA or similar morphology presenting between 1990 and 2000. Follow-up with clinical assessment, echocardiography and catheter studies.

RESULTS: Twenty-five patients had initial palliation with pulmonary artery banding with repair of any associated arch obstruction. Twelve patients had DKS performed as part of their second stage procedure, and 3 had DKS performed later for recurrent stenosis after prior enlargement of BVF. Six patients had BVF resection without later restenosis and 4 patients did not develop BVF stenosis. There was one early death (4%) and two late (8%). Fontan completion was achieved in 20 of the 22 survivors. There were no cases of DKS obstruction, no pulmonary valve had more than mild regurgitation.

CONCLUSIONS: Our approach achieves low operative mortality and morbidity and compares favorably with reported results for Norwood palliation. The significant rate of systemic outflow obstruction in those who did not undergo DKS at the second stage confirms the utility of early DKS in children with this morphology.






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