Table 1.
ICD-10 codes for moderate and severe congenital heart disease, as defined by the 2018 Adult Congenital Heart Disease guidelines
| Moderate congenital heart disease | ICD-10 codes |
|---|---|
| Partial or total anomalous pulmonary venous return | Q26.2, Q26.3, Q26.4 |
| Anomalous left coronary artery from the pulmonary artery | Q24.5 |
| Atrioventricular septal defect | Q21.2 |
| Aortic stenosis (congenital) | Q23.0 |
| Mitral stenosis (congenital) | Q23.2 |
| Coarctation of the aorta | Q25.1, Q25.42 |
| Cor triatriatum | Q24.2 |
| Ebstein anomaly | Q22.5 |
| Subpulmonary stenosis | Q24.3 |
| Patent ductus arteriosus | Q21.4, Q25.0 |
| Pulmonary regurgitation | Q22.2 |
| Pulmonary stenosis | Q22.1 |
| Peripheral pulmonary stenosis | Q25.6, Q25.7, Q25.71 |
| Sinus of Valsalva fistula | Q25.4 |
| Subaortic stenosis | Q24.4 |
| Supravalvar aortic stenosis | Q25.3 |
| Tetralogy of Fallot | Q21.3 |
| Double aortic arch | Q25.45 |
| Complex congenital heart disease | ICD-10 codes |
|---|---|
| Double outlet right ventricle | Q20.1 |
| Interrupted aortic arch | Q25.21 |
| Single ventricle (DILV, TA, HLHS, etc.) | Q20.4, Q22.4, Q22.6, Q23.4, Q25.2, Q25.29, Q25.41 |
| Pulmonary atresia | Q22.0, Q25.5 |
| Transposition of the great arteries (d-, l- or congenitally corrected) | Q20.3, Q20.5 |
| Truncus arteriosus | Q20.0 |
| Criss cross heart, heterotaxy | Q20.6 |
| Eisenmenger | I27.83 |
| Double outlet left ventricle | Q20.2 |
DILV double inlet left ventricle; HLHS hypoplastic left heart syndrome; TA tricuspid atresia