|1. Eisenmenger syndrome|
Includes all large intra- and extra-cardiac defects which begin as systemic-to-pulmonary shunts and progress with time to severe elevation of pulmonary vascular resistance (PVR) and to reversal (pulmonary-to-systemic) or bidirectional shunting; cyanosis, secondary erythrocytosis and multiple organ involvement are usually present.
|2. Left-to-right shunts|
Include moderate to large defects; PVR is mildly to moderately increased systemic-to-pulmonary shunting is still prevalent, whereas cyanosis is not a feature.
|3. Pulmonary arterial hypertension (PAH) with coincidental congenital heart disease|
Marked elevation in PVR in the presence of small cardiac defects, which themselves do not account for the development of elevated PVR; the clinical picture is very similar to idiopathic PAH. To close the defects in contraindicated.
|4. Post-operative PAH|
Congenital heart disease is repaired but PAH either persists immediately after surgery or recurs/develops months or years after surgery in the absence of significant postoperative hemodynamic lesions. The clinical phenotype is often aggressive.
↵∗ Nice 2013.