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 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): Laszlo Kiraly Robert H. Anderson Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Kiraly, L. Articles by Anderson, R. H. Search for Related Content PubMed PubMed Citation Articles by Kiraly, L. Articles by Anderson, R. H. Related Collections Congenital - cyanotic Valve disease

J Thorac Cardiovasc Surg 2007;133:229-234
© 2007 The American Association for Thoracic Surgery

Surgery for Congenital Heart Disease

Morphologic features of the uniatrial but biventricular atrioventricular connection

^a Gottsegen Hungarian Institute of Cardiology, Pediatric Cardiac Centre, Budapest, Hungary
^b Department of Forensic Pathology, Semmelweis University, Budapest, Hungary
^c Institute of Child Health, Cardiac Unit, University College London, United Kingdom
^d National Heart and Lung Institute Imperial College School of Medicine, London, United Kingdom.

Received for publication June 26, 2006; revisions received August 11, 2006; accepted for publication August 29, 2006.

^_* Address for reprints: Laszlo Kiraly, MD, FETCS, Pediatric Cardiac Surgeon-In-Chief, Gottsegen Hungarian Institute of Cardiology, Pediatric Cardiac Centre, H-1450 Budapest, Pf. 88. H-1096 IX. Haller u. 29. Hungary. (Email: kiraly{at}kardio.hu; kiralyl{at}hu.inter.net).

OBJECTIVES: Hearts with an absent atrioventricular connection and a straddling of the solitary atrioventricular valve are rare but significant lesions. They are suitable only for Fontan-like palliation, in which atrioventricular valvar abnormalities play a significant role in determining the outcome. We studied the segmental arrangements in such lesions and clarified the valvar morphology, particularly its surgical implications.

METHODS: We made a macroscopic review of all specimens with an absent atrioventricular connection and a straddling atrioventricular valve that were held in the collections of 3 institutes. We included only those specimens with the straddling valve supported exclusively by either the right-sided or left-sided atrioventricular junction and excluded those with a common atrioventricular junction.

RESULTS: We found 11 hearts with an absent right atrioventricular connection and a straddling left atrioventricular valve, and 3 with an absent left atrioventricular connection and a straddling right atrioventricular valve. Most had right-hand ventricular topology and discordant ventriculoarterial connections. We found multiple valvar abnormalities, including dysplastic leaflets, short cords, abnormal attachments, and abnormal papillary muscles. The most consistent features were a line of maximal coaptation between the bridging leaflets always perpendicular to the plane of the ventricular septum and a free-floating bridging anterosuperior leaflet.

CONCLUSIONS: Straddling of a solitary atrioventricular valve with an absent atrioventricular connection produces a uniatrial but biventricular connection. In this setting, the valve guarding the abnormal solitary atrioventricular junction cannot be classified morphologically as mitral or tricuspid. The markedly variable valvar morphology likely makes these valves prone to insufficiency in the long term.

This article has been cited by other articles:

				R. J. Sommer, Z. M. Hijazi, and J. F. Rhodes Jr Pathophysiology of Congenital Heart Disease in the Adult: Part I: Shunt Lesions Circulation, February 26, 2008; 117(8): 1090 - 1099. [Full Text] [PDF]