Table 3

Updated Clinical Classification of Pulmonary Arterial Hypertension Associated With Congenital Heart Disease

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
 • Correctable†
 • Noncorrectable
 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.