The importance of nomenclature for congenital cardiac disease: implications for research and evaluation

• Type of Material: Article
• Authors: Matthew Strickland, Emory University; Tiffany J Riehle-Colarusso, Centers for Disease Control and Prevention; Jeffrey P Jacobs, University of South Florida; Mark D Reller, Oregon Health & Science University; William Mahle, Emory University; Lorenzo D Botto, University of Utah; Paige Tolbert, Emory University; Marshall L Jacobs, Drexel University; Francois G Lacour-Gayet, University of Colorado; Christo I Tchervenkov, McGill University; Constantine Mavroudis, Northwestern University; Adolfo Correa, Emory University
• Language: English
• Date: 2008-12-01
• Publisher: Cambridge University Press (CUP): STM Journals
• Publication Version: Author Accepted Manuscript (After Peer Review)
• Copyright Statement: COPYRIGHT: © Cambridge University Press 2008
• Final Published Version (URL): http://dx.doi.org/10.1017/S1047951108002515
• Title of Journal or Parent Work: Cardiology in the Young
• ISSN: 1047-9511
• Volume: 18
• Issue: S2
• Start Page: 92
• End Page: 100
• Grant/Funding Information: This study was funded in part by U.S. National Institute of Environmental Health Sciences (NIEHS) grant R01-ES012967-01A1; and by support from the Environmental Public Health Tracking Program, National Center for Environmental Health, US Centers for Disease Control and Prevention.
• Keywords: Pediatrics; CHILDREN; SOCIETY; Tetralogy of Fallot; HETEROTAXY SYNDROME; SURGEONS PERFORMANCE; DEFECTS; DATABASE; OUTLET RIGHT VENTRICLE; transposition of the arteries; Science & Technology; FUNCTIONALLY UNIVENTRICULAR HEART; Cardiovascular System & Cardiology; Cardiac & Cardiovascular Systems; Life Sciences & Biomedicine; CLASSIFICATION; MORTALITY-RATES; hypoplastic left heart syndrome
• Research Categories: Health Sciences, Public Health; Health Sciences, Medicine and Surgery; Health Sciences, Human Development

Abstract

BACKGROUND: Administrative databases are often used for congenital cardiac disease research and evaluation, with little validation of the accuracy of the diagnostic codes. METHODS: Metropolitan Atlanta Congenital Defects Program surveillance records were reviewed and classified using a version of the International Pediatric and Congenital Cardiac Code. Using this clinical nomenclature as the referent, we report the sensitivity and false positive fraction (1 - positive predictive value) of the International Classification of Diseases, Ninth Revision, Clinical Modification diagnosis codes for tetralogy of Fallot, transposition of the great arteries, and hypoplastic left heart syndrome. RESULTS: We identified 4918 infants and foetuses with congenital cardiac disease from the surveillance records. Using only the International Classification of Diseases diagnosis codes, there were 280 records with tetralogy, 317 records with transposition, and 192 records with hypoplastic left heart syndrome. Based on the International Pediatric and Congenital Cardiac Code, 330 records were classified as tetralogy, 163 records as transposition, and 179 records as hypoplastic left heart syndrome. The sensitivity of International Classification of Diseases diagnosis codes was 83% for tetralogy, 100% for transposition, and 95% for hypoplastic left heart syndrome. The false positive fraction was 2% for tetralogy, 49% for transposition, and 11% for hypoplastic left heart syndrome. CONCLUSIONS: Analyses based on International Classification of Diseases diagnosis codes may have substantial misclassification of congenital heart disease. Isolating the major defect is difficult, and certain codes do not differentiate between variants that are clinically and developmentally different.