| A. Eisenmenger’s syndrome |
1. Reversed (pulmonary-to-systemic) or bidirectional shunt |
|
2. Cyanosis, erythrocytosis, and multiple organ involvement are present |
| B. Pulmonary arterial hypertension associated with systemic-to-pulmonary shunts |
1. Moderate to large defects with increased PVR (mild to moderate) |
|
2. No cyanosis is present at rest |
| C. Pulmonary arterial hypertension with small defects |
1. Small defects (usually ventricular septal defects ,1 cm and atrial septal defects, 2 cm of effective diameter assessed by echocardiography) |
|
2. The clinical picture is very similar to idiopathic PAH |
| D. Pulmonary arterial hypertension after corrective cardiac surgery |
1. Congenital heart disease has been corrected but PAH is either still present immediately after surgery or has recurred several months or years after surgery |
|
2. Absence of significant post-operative residual congenital lesions or defects that originate as a sequela to previous surgery |
