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J Thorac Cardiovasc Surg 2005;130:1503-1510
© 2005 The American Association for Thoracic Surgery

Surgery for Congenital Heart Disease

Matching procedure to morphology improves outcomes in neonates with tricuspid atresia

^a Division of Cardiology and Cardiovascular Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada,
^b Department of General Surgery, Wake Forest University School of Medicine, Winston-Salem, NC,
^c Division of Cardiothoracic Surgery and Biostatistics and Epidemiology, Cleveland Clinic Foundation, Cleveland, Ohio,
^d Division of Cardiovascular Surgery, Children's Hospital National Medical Center, Washington, DC,
^e Division of Cardiothoracic Surgery, St Christopher's Hospital for Children, Philadelphia, Pa,
^f the Department of Cardiothoracic Surgery, Doernbecher Children's Hospital, Oregon Health and Science University, Portland, Ore.

Read at the Eighty-fifth Annual Meeting of The American Association for Thoracic Surgery, San Francisco, Calif, April 10-13, 2005.

Received for publication April 4, 2005; revisions received June 21, 2005; accepted for publication July 23, 2005.

^_* Address for reprints: Brian W. McCrindle, MD, The Hospital for Sick Children, 555 University Ave, Toronto, Ontario, Canada M5G 1X8 (Email: brian.mccrindle{at}sickkids.ca).

OBJECTIVE: This study was undertaken to characterize morphologic substrate of tricuspid atresia with ventriculoarterial concordance and discriminate management strategies that lead to successful definitive repair.

METHODS: From 1999 to 2004, a total of 150 babies with type I tricuspid atresia were enrolled from first diagnosis at 26 institutions. Antegrade pulmonary blood flow was absent in 19%, restricted in 54%, and unrestricted in 28%. Competing-risk methodology determined the time-related prevalence and risk factors for death versus cavopulmonary anastomosis and subsequent death versus Fontan completion.

RESULTS: Overall 5-year survival was 86%. Initial palliation included systemic-pulmonary arterial shunt in 64%, pulmonary artery banding in 11%, and cavopulmonary anastomosis in 24%. Median age at cavopulmonary anastomosis was 6 months, with 83% undergoing bidirectional Glenn shunt and 17% undergoing hemi-Fontan procedure. By the age of 2 years, 89% had cavopulmonary anastomosis, 6% were dead, and 4% remained alive without cavopulmonary anastomosis. Risk factors for death without cavopulmonary anastomosis included presence of mitral regurgitation (P = .03) and palliation with systemic-pulmonary arterial shunts not originating from the innominate artery (P = .04). Factors associated with decreased transition rate to cavopulmonary connection included patient variables (younger admission age to a participating institution, noncardiac anomalies) and procedural variables (larger systemic-pulmonary arterial shunt diameter, previous palliation). Of patients undergoing cavopulmonary anastomosis, 75% had undergone a Fontan operation within 3 years.

CONCLUSION: Smaller shunt size and decreased pulmonary blood flow decrease mortality after initial palliation and increase the rate of successful transition to cavopulmonary anastomosis. Outcomes can be improved by placing smaller shunts from the innominate artery, especially in patients with any mitral regurgitation.

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