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 Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Tchervenkov, C. I. Articles by Tahta, S. A. Search for Related Content PubMed PubMed Citation Articles by Tchervenkov, C. I. Articles by Tahta, S. A.

Ann Thorac Surg 2000;69:S170-S179
© 2000 The Society of Thoracic Surgeons

Congenital Heart Surgery Nomenclature and Database Project: hypoplastic left heart syndrome

^a Division of Cardiovascular Surgery, The Montreal Children’s Hospital, McGill University, Montréal, Québec, Canada
^b Department of Surgery, St. Christopher’s Hospital for Children, Philadelphia, Pennsylvania, USA

Address reprint requests to Dr Tchervenkov, Division of Cardiovascular Surgery, The Montreal Children’s Hospital, 2300 Tupper St, Room C-828, Montreal, PQ, Canada H3H 1P3
e-mail: ctchcvt{at}mch.mcgill.ca

Presented at the International Nomenclature and Database Conferences for Pediatric Cardiac Surgery, 1998–1999.

Abstract

Hypoplastic left heart syndrome (HLHS) encompasses a spectrum of structural cardiac malformations that are characterized by severe underdevelopment of the structures in the left heart-aorta complex, including the left ventricular cavity and mass. The severe end of the spectrum consists of aortic atresia and mitral atresia with a nonexistent left ventricle, whereas at the mild end patients have aortic valve and mitral valve hypoplasia without intrinsic valve stenosis, and milder degrees of left ventricular hypoplasia, recently described as hypoplastic left heart complex (HLHC). Although the overwhelming majority of the patients can only have a univentricular repair, a small minority of patients with HLHS, particularly those that are described as having HLHC, may be candidates for biventricular repair.

In this paper, the extant nomenclature for HLHS is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Nomenclature and Database Committee and representatives from the European Association for Cardiothoracic Surgery. Efforts were made to include all relevant nomenclature categories using synonyms where appropriate. A comprehensive database set is presented, which is based on a hierarchical scheme. Data are entered at various levels of complexity and detail that can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented which will allow for data sharing, and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.

This article has been cited by other articles:

				K. Gambetta, M. K. Al-Ahdab, M. N. Ilbawi, N. Hassaniya, and M. Gupta Transcription repression and blocks in cell cycle progression in hypoplastic left heart syndrome Am J Physiol Heart Circ Physiol, May 1, 2008; 294(5): H2268 - H2275. [Abstract] [Full Text] [PDF]

				A. deAlmeida, T. McQuinn, and D. Sedmera Increased Ventricular Preload Is Compensated by Myocyte Proliferation in Normal and Hypoplastic Fetal Chick Left Ventricle Circ. Res., May 11, 2007; 100(9): 1363 - 1370. [Abstract] [Full Text] [PDF]

				P. Khairy, N. Poirier, and L.-A. Mercier Univentricular Heart Circulation, February 13, 2007; 115(6): 800 - 812. [Abstract] [Full Text] [PDF]

				J. Photiadis, A. E. Urban, N. Sinzobahamvya, C. Fink, E. Schindler, M. Schneider, A. M. Brecher, and B. Asfour Restrictive left atrial outflow adversely affects outcome after the modified Norwood procedure Eur. J. Cardiothorac. Surg., June 1, 2005; 27(6): 962 - 967. [Abstract] [Full Text] [PDF]

				A. F. Corno Borderline left ventricle Eur. J. Cardiothorac. Surg., January 1, 2005; 27(1): 67 - 73. [Abstract] [Full Text] [PDF]

				D. C. Drinkwater Jr, A. S. Aharon, S. V. Quisling, D. Dodd, V. S. Reddy, A. Kavanaugh-McHugh, T. Doyle, N. R. Patel, F. E. Barr, J. K. Kambam, et al. Modified Norwood operation for hypoplastic left heart syndrome Ann. Thorac. Surg., December 1, 2001; 72(6): 2081 - 2087. [Abstract] [Full Text] [PDF]