HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Full Text Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Osman O. Al-Radi Ian Adatia Christopher A. Caldarone John G. Coles William G. Williams Glen S. Van Arsdell Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Yun, T.-J. Articles by Van Arsdell, G. S. Search for Related Content PubMed PubMed Citation Articles by Yun, T.-J. Articles by Van Arsdell, G. S.

J Thorac Cardiovasc Surg 2006;131:1108-1113
© 2006 The American Association for Thoracic Surgery

Surgery for Congenital Heart Disease

Contemporary management of right atrial isomerism: Effect of evolving therapeutic strategies

^a Division of Pediatric Cardiac Surgery, Asan Medical Center, University of Ulsan, Ulsan, Republic of Korea
^b The Cardiac Program, Hospital for Sick Children, Toronto, Canada
^c Division of Pediatric Cardiology, University of California, San Francisco, Calif

Presented at 31st Annual Meeting of the Western Thoracic Surgical Association, Victoria, British Columbia, June 22-25, 2005.

Received for publication June 16, 2005; revisions received November 10, 2005; accepted for publication November 16, 2005.

^_* Address for reprints: Glen S. Van Arsdell, MD, Division of Cardiovascular Surgery, The Hospital for Sick Children, 555 University Ave, Toronto, Ontario, Canada, M5G 1X8 (Email: glen.vanarsdell{at}sickkids.ca).

OBJECTIVES: Infants with right atrial isomerism have poor outcomes because of a complex combination of cardiac anomalies. Aggressive management of total anomalous pulmonary venous drainage might have a positive effect on the prognosis.

METHODS: Outcomes of all children with right atrial isomerism from 1994 to the present were reviewed. Management of total anomalous pulmonary venous drainage evolved from no repair or conventional surgical technique to primary sutureless repair on initial palliation. Cox survival models were used to identify variables associated with reduced survival.

RESULTS: There were 55 children enrolled in the study. The median age at the initial visit was 2 days. Fifty-one patients had total anomalous pulmonary venous drainage (obstructive in 22 patients). Withdrawal of treatment occurred in 11 (20%) of 55 patients during an interval of institutional bias toward no treatment. Thirteen (24%) of 55 patients had palliations without total anomalous pulmonary venous drainage repair, and 3 (23%) of 13 survived. Thirty-one (56%) of 55 patients had operations that included total anomalous pulmonary venous drainage repair, of whom 13 (42%) of 31 underwent primary sutureless repair for total anomalous pulmonary venous drainage. Sixteen (52%) of 31 survived, and their current status 1 to 10 years (median, 5.8 years) after repair is post-Fontan (7/16 [44%]), postbidirectional Glenn (6/16 [38%]), and others (3 [20%]). In patients who underwent total anomalous pulmonary venous drainage repair (n = 31), 2 risk factors of decreased survival were identified: drainage site obstruction and infracardiac or mixed-type total anomalous pulmonary venous drainage. After adjustment, sutureless repair appeared to be associated with improved survival (hazard ratio, 0.43), but this beneficial effect did not reach significance (P = .19).

CONCLUSIONS: Mortality continues to be high; however, aggressive total anomalous pulmonary venous drainage repair for right atrial isomerism has resulted in improved survival. The role of primary sutureless repair for total anomalous pulmonary venous drainage remains to be defined.

This article has been cited by other articles:

				T. Nakata, Y. Fujimoto, K. Hirose, M. Osaki, Y. Tosaka, Y. Ide, M. Tachi, and K. Sakamoto Functional single ventricle with extracardiac total anomalous pulmonary venous connection Eur. J. Cardiothorac. Surg., July 1, 2009; 36(1): 49 - 56. [Abstract] [Full Text] [PDF]

				H.G. Lim, E.A. Bacha, G.R. Marx, A. Marshall, F. Fynn-Thompson, J.E. Mayer, P. Del Nido, and F.A. Pigula Biventricular repair in patients with heterotaxy syndrome. J. Thorac. Cardiovasc. Surg., February 1, 2009; 137(2): 371 - 379.e3. [Abstract] [Full Text] [PDF]

				T. Nakata, Y. Fujimoto, K. Hirose, and K. Sakamoto Right atrial isomerism with infracardiac total anomalous pulmonary venous connection complicated by hiatal hernia. Eur. J. Cardiothorac. Surg., August 1, 2008; 34(2): 460 - 462. [Abstract] [Full Text] [PDF]

				W.-K. Jhang, Y.-J. Chang, C.-S. Park, Y.-M. Oh, Y.-H. Kim, and T.-J. Yun Hybrid palliation for right atrial isomerism associated with obstructive total anomalous pulmonary venous drainage Interactive CardioVascular and Thoracic Surgery, April 1, 2008; 7(2): 282 - 284. [Abstract] [Full Text] [PDF]

				U. K. Chowdhury, B. Airan, A. Malhotra, A. K. Bisoi, A. Saxena, S. S. Kothari, M. Kalaivani, and P. Venugopal Mixed total anomalous pulmonary venous connection: Anatomic variations, surgical approach, techniques, and results J. Thorac. Cardiovasc. Surg., January 1, 2008; 135(1): 106 - 116. [Abstract] [Full Text] [PDF]