Table 2.

Dana Point 4^th World Symposium on Pulmonary Hypertension’s Anatomic-Pathophysiologic Classification of Congenital Systemic-to-Pulmonary Shunts Associated With Pulmonary Arterial Hypertension

1 Type

1.1 Simple pre-tricuspid shunts

1.1.1 Atrial septal defect (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 Ventricular septal defect (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 >1 congenital heart defect)

2.1 Hemodynamic Qp/Qs (ratio of pulmonary blood flow to systemic blood flow)

2.1.1 Restrictive (pressure gradient across the defect)

2.1.2 Nonrestrictive

2.2 Anatomic

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)

Reproduced from¹⁸ with permission.