Table 2.

Anatomic-pathophysiologic classification of congenital systemic-to-pulmonary shunts associated with pulmonary arterial hypertension.

(1) Type
(1.1) Simple pretricuspid shunts
(1.2) Simple posttricuspid shunts
(1.3) Combined shunts
(1.4) Complex congenital heart disease
(2) Dimension (specify for each defect if >1 congenital heart
defect)
(2.1) Hemodynamic (specify Qp/Qs)∗: restrictive or
nonrestrictive
(2.2) Anatomic defect size: small, moderate or large
(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
(5.3) Repaired