Skip to main content
    • Aa
    • Aa

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

  • Matthew J. Strickland (a1) (a2), Tiffany J. Riehle-Colarusso (a1), Jeffrey P. Jacobs (a3), Mark D. Reller (a4), William T. Mahle (a5), Lorenzo D. Botto (a6), Paige E. Tolbert (a2), Marshall L. Jacobs (a7), Francois G. Lacour-Gayet (a8), Christo I. Tchervenkov (a9), Constantine Mavroudis (a10) and Adolfo Correa (a1)...

Administrative databases are often used for congenital cardiac disease research and evaluation, with little validation of the accuracy of the diagnostic codes.


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.


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.


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.

Corresponding author
Correspondence to: Matthew J. Strickland, Centers for Disease Control and Prevention, 1600 Clifton Rd. NE, Mailstop E-86 Atlanta, GA 30333, United States of America. Tel: 404-421-3183; Fax: 404-498-3040; E-mail:
Linked references
Hide All

This list contains references from the content that can be linked to their source. For a full set of references and notes please see the PDF or HTML where available.

1. JG Berry , CG Cowley , CJ Hoff , R Srivastava . In-hospital mortality for children with hypoplastic left heart syndrome after stage I surgical palliation: teaching versus nonteaching hospitals. Pediatrics 2006; 117: 13071313.

2. JG Berry , TA Lieu , PW Forbes , DA Goldmann . Hospital volumes for common pediatric specialty operations. Arch Pediatr Adolesc Med 2007; 161: 3843.

4. OJ Benavidez , K Gauvreau , KJ Jenkins . Racial and ethnic disparities in mortality following congenital heart surgery. Pediatr Cardiol 2006; 27: 321328.

5. JA Connor , K Gauvreau , KJ Jenkins . Factors associated with increased resource utilization for congenital heart disease. Pediatrics 2005; 116: 689695.

6. RK Chang , S Rodriguez , M Lee , TS Klitzner . Risk factors for deaths occurring within 30 days and 1 year after hospital discharge for cardiac surgery among pediatric patients. Am Heart J 2006; 152: 386393.

7. AJ Marelli , AS Mackie , R Ionescu-Ittu , E Rahme , L Pilote . Congenital heart disease in the general population: changing prevalence and age distribution. Circulation 2007; 115: 163172.

8. MZ Gurvitz , M Inkelas , M Lee , K Stout , J Escarce , RK Chang . Changes in hospitalization patterns among patients with congenital heart disease during the transition from adolescence to adulthood. J Am Coll Cardiol 2007; 49: 875882.

10. CE Cronk , ME Malloy , AN Pelech , Completeness of state administrative databases for surveillance of congenital heart disease. Birth Defects Res A Clin Mol Teratol 2003; 67: 597603.

11. BK Frohnert , RC Lussky , MA Alms , NJ Mendelsohn , DM Symonik , MC Falken . Validity of hospital discharge data for identifying infants with cardiac defects. J Perinatol 2005; 25: 737742.

12. C Mavroudis , JP Jacobs . Congenital Heart Surgery Nomenclature and Database Project: overview and minimum dataset. Ann Thorac Surg 2000; 69 (Suppl 4): S217.

16. JP Jacobs , C Mavroudis , ML Jacobs , Lessons learned from the data analysis of the second harvest (1998–2001) of the Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database. Eur J Cardiothorac Surg 2004; 26: 1837.

17. JP Jacobs , FG Lacour-Gayet , ML Jacobs , Initial application in the STS congenital database of complexity adjustment to evaluate surgical case mix and results. Ann Thorac Surg 2005; 79: 16351649.

18. JP Jacobs , ML Jacobs , B Maruszewski , Current status of the European Association for Cardio-Thoracic Surgery and the Society of Thoracic Surgeons Congenital Heart Surgery Database. Ann Thorac Surg 2005; 80: 22782283.

25. J Stark , S Gallivan , J Lovegrove , Mortality rates after surgery for congenital heart defects in children and surgeons. Lancet 2000; 355: 10041007.

26. JF Stark , S Gallivan , K Davis , Assessment of mortality rates for congenital heart defects and surgeons’ performance. Ann Thorac Surg 2001; 72: 169174.

32. JW Brown , M Ruzmetov , Y Okada , P Vijay , MW Turrentine . Surgical results in patients with double outlet right ventricle: a 20-year experience. Ann Thorac Surg 2001; 72: 16301635.

33. K Takeuchi , FX McGowan Jr, Analysis of surgical outcome in complex double-outlet right ventricle with heterotaxy syndrome or complete atrioventricular canal defect. Ann Thorac Surg 2006; 82: 146152.

34. MS Cohen , AH Schultz , ZY Tian , Heterotaxy syndrome with functional single ventricle: does prenatal diagnosis improve survival? Ann Thorac Surg 2006; 82: 16291636.

38. JP Jacobs , G Wernovsky , MJ Elliott . Analysis of outcomes for congenital cardiac disease: can we do better? Cardiol Young 2007; 17 (Suppl 2): 145158.

42. JP Jacobs , RH Anderson , P Weinberg , The nomenclature, definition and classification of cardiac structures in the setting of heterotaxy. Cardiol Young 2007; 17 (Suppl 2): 128.

Recommend this journal

Email your librarian or administrator to recommend adding this journal to your organisation's collection.

Cardiology in the Young
  • ISSN: 1047-9511
  • EISSN: 1467-1107
  • URL: /core/journals/cardiology-in-the-young
Please enter your name
Please enter a valid email address
Who would you like to send this to? *



Altmetric attention score

Full text views

Total number of HTML views: 1
Total number of PDF views: 10 *
Loading metrics...

Abstract views

Total abstract views: 125 *
Loading metrics...

* Views captured on Cambridge Core between September 2016 - 27th May 2017. This data will be updated every 24 hours.