Table 7—

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 Ostium secundum Sinus venosus 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.