Anatomical–pathophysiological classification of congenital systemic-to-pulmonary shunts associated with pulmonary arterial hypertension
| 1 Type |
| 1.1 Simple pre-tricuspid shunts |
| 1.1.1 ASD |
| 1.1.1.1 Ostium secundum |
| 1.1.1.2 Sinus venosus |
| 1.1.1.3 Ostium primum |
| 1.1.2 Total or partial unobstructed anomalous pulmonary venous return |
| 1.2 Simple post-tricuspid shunts |
| 1.2.1 VSD |
| 1.2.2 Patent ductus arteriosus |
| 1.3 Combined shunts |
| Describe combination and define predominant defect |
| 1.4 Complex congenital heart disease |
| 1.4.1 Complete atrioventricular septal defect |
| 1.4.2 Truncus arteriosus |
| 1.4.3 Single ventricle physiology with unobstructed pulmonary blood flow |
| 1.4.4 Transposition of the great arteries with VSD (without pulmonary stenosis) and/or patent ductus arteriosus |
| 1.4.5 Other |
| 2 Dimension (specify for each defect if more than one congenital heart defect exists) |
| 2.1 Haemodynamic (specify Qp/Qs)# |
| 2.1.1 Restrictive (pressure gradient across the defect) |
| 2.1.2 Nonrestrictive |
| 2.2 Anatomical¶ |
| 2.2.1 Small to moderate (ASD ≤2.0 cm and VSD ≤1.0 cm) |
| 2.2.2 Large (ASD >2.0 cm and VSD >1.0 cm) |
| 3 Direction of shunt |
| 3.1 Predominantly systemic-to-pulmonary |
| 3.2 Predominantly pulmonary-to-systemic |
| 3.3 Bidirectional |
| 4 Associated cardiac and extracardiac abnormalities |
| 5 Repair status |
| 5.1 Unoperated |
| 5.2 Palliated (specify type of operation(s), age at surgery) |
| 5.3 Repaired (specify type of operation(s), age at surgery) |
ASD: atrial septal defect; VSD: ventricular septal defect. #: ratio of pulmonary (Qp) to systemic (Qs) blood flow; ¶: the size applies to adult patients. Modified from the Evian-Venice classification 2003 13, with permission from the publisher.