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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
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Counts of Target Development Levels for diseases known to be associated with this disease. If the disease has a valid DOID, targets known to be associated with all child diseases are aggregated. Click "Explore Associated Targets" to view more facets and details for the target list.
Description from Mondo Disease Ontology.
DataSources which have contributed target associations to this disease, and the identifiers by which the disease is referenced.
EFO:0009054
Orphanet:275803
SCTID:697905000
UMLS:C3697119
UMLS:CN243982
MONDO:0017152
High level summary of knowledge for a disease, including descriptions and datasource references.