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pulmonary arterial hypertension associated with congenital heart disease

Disease Summary
Associated Targets ()

Mondo Description Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is a form of pulmonary arterial hypertension (PAH), characterized by elevated pulmonary arterial resistance leading to right heart failure occurring as a common complication of congenital heart malformations with left to right cardiac shunts. Eisenmenger syndrome is the most advanced form of PAH-CHD and is defined as the complete or partial reversal of an initial left-to-right shunt to a right-to-left shunt, causing cyanosis and limited exercise capacity. PAH-CHD also includes mild to moderate systemic-to-pulmonary shunts with no cyanosis at rest, patients with small defects, and those with residual PAH following corrective cardiac surgery.
Mondo Term and Equivalent IDs
MONDO:0017152:  pulmonary arterial hypertension associated with congenital heart disease
EFO:0009054: 
Orphanet:275803: 
SCTID:697905000: 
UMLS:C3697119: 
UMLS:CN243982: