Background

The international nomenclature of Congenital Heart Diseases (CHD) remains challenging. Classifications have been proposed such as the International Pediatric and Congenital Cardiac Code (IPCCC) and the ACC-CHD (Anatomic and Clinical Classification).

Methods

We retrospectively included all consecutive fetal echocardiograms (1 cardiologist) over 6 years. Reports were independently coded with 1 single code (the most precise) by 3 pediatric cardiologists with increasing experience (junior [J], senior I [SI] and II [SII]). Discordances between doctors were compared to a gold standard code secondary fixed by SI and SII, with focus on coding difficulties and effects of professional experience using IPCCC and ACC-CHD.

Results

Among 299 scans, 7 were excluded (doubts). Coding was always possible with IPCCCC, but not achieved in 112 cases with ACC-CHD. One hundred and eighty hearts were selected. Using either IPCCC or ACC-CHD, coding with 1 item was difficult for SI and SII in 15% of cases (ACC-CHD categories 6, 9, 8). IPCCC was too exhaustive for its simple use leading to discordance. ACC-CHD was also difficult to use (learning curve, use of 1 code, complex CHD). Coding discordance using ACC-CHD main categories was higher for junior compared to seniors (J-SI, P = 0.04; J-SII, P = 0.02), without difference between seniors. Compared to the gold standard for ACC-CHD (main, sub) categories, junior concordance was lower (73.3%, 71.1%) than SI (90%, 83.3%, P < 0.005) and SII (88.3%, 87.2%, P < 0.0001). Senior concordance was stronger (75%) with ACC-CHD sub categories compared to IPCCC (65%, P = 0.028).

Conclusion

IPCCC and ACC-CHD remain difficult for their use in clinical practice. Many functional abnormalities are not listed in the ACC-CHD but could be updated with a few more sub groups. The use of 1 code appears restrictive to well classify some complex CHD and limits our study. However, we believe that some ACC-CHD categories allow simplification with prognosis issues for further studies.