The importance of nomenclature for congenital cardiac disease

implications for research and evaluation.

Matthew J. Strickland, Tiffany J. Riehle-Colarusso, Jeffrey P. Jacobs, Mark Reller, William T. Mahle, Lorenzo D. Botto, Paige E. Tolbert, Marshall L. Jacobs, Francois G. Lacour-Gayet, Christo I. Tchervenkov, Constantine Mavroudis, Adolfo Correa

Research output: Contribution to journalArticle

95 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)92-100
Number of pages9
JournalCardiology in the Young
Volume18 Suppl 2
StatePublished - Dec 2008
Externally publishedYes

Fingerprint

Hypoplastic Left Heart Syndrome
Terminology
International Classification of Diseases
Heart Diseases
Pediatrics
Transposition of Great Vessels
Tetralogy of Fallot
Fetus
Databases

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Pediatrics, Perinatology, and Child Health

Cite this

Strickland, M. J., Riehle-Colarusso, T. J., Jacobs, J. P., Reller, M., Mahle, W. T., Botto, L. D., ... Correa, A. (2008). The importance of nomenclature for congenital cardiac disease: implications for research and evaluation. Cardiology in the Young, 18 Suppl 2, 92-100.

The importance of nomenclature for congenital cardiac disease : implications for research and evaluation. / Strickland, Matthew J.; Riehle-Colarusso, Tiffany J.; Jacobs, Jeffrey P.; Reller, Mark; Mahle, William T.; Botto, Lorenzo D.; Tolbert, Paige E.; Jacobs, Marshall L.; Lacour-Gayet, Francois G.; Tchervenkov, Christo I.; Mavroudis, Constantine; Correa, Adolfo.

In: Cardiology in the Young, Vol. 18 Suppl 2, 12.2008, p. 92-100.

Research output: Contribution to journalArticle

Strickland, MJ, Riehle-Colarusso, TJ, Jacobs, JP, Reller, M, Mahle, WT, Botto, LD, Tolbert, PE, Jacobs, ML, Lacour-Gayet, FG, Tchervenkov, CI, Mavroudis, C & Correa, A 2008, 'The importance of nomenclature for congenital cardiac disease: implications for research and evaluation.', Cardiology in the Young, vol. 18 Suppl 2, pp. 92-100.
Strickland MJ, Riehle-Colarusso TJ, Jacobs JP, Reller M, Mahle WT, Botto LD et al. The importance of nomenclature for congenital cardiac disease: implications for research and evaluation. Cardiology in the Young. 2008 Dec;18 Suppl 2:92-100.
Strickland, Matthew J. ; Riehle-Colarusso, Tiffany J. ; Jacobs, Jeffrey P. ; Reller, Mark ; Mahle, William T. ; Botto, Lorenzo D. ; Tolbert, Paige E. ; Jacobs, Marshall L. ; Lacour-Gayet, Francois G. ; Tchervenkov, Christo I. ; Mavroudis, Constantine ; Correa, Adolfo. / The importance of nomenclature for congenital cardiac disease : implications for research and evaluation. In: Cardiology in the Young. 2008 ; Vol. 18 Suppl 2. pp. 92-100.
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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.",
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AU - Mahle, William T.

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