A. Eisenmenger syndrome | Includes all large intracardiac and extracardiac defects which begin as systemic-to-pulmonary shunts and progress with time to severe elevation of PVR and to reversal (pulmonary-to-systemic) or bidirectional shunting; cyanosis, secondary erythrocytosis and multiple-organ involvement are usually present. |
B. Left-to-right shunts |
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C. PAH with coincidental CHD | 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 is contraindicated. |
D. Postoperative PAH | CHD is repaired but PAH either persists immediately after surgery or recurs/develops months or years after surgery in the absence of significant postoperative haemodynamic lesions. The clinical phenotype is often aggressive. |
Adapted with permission from Simonneau.2
CHD, congenital heart disease; PAH, pulmonary arterial hypertension; PVR, pulmonary vascular resistance.