Landzberg et al listed risk factors associated with the development of pulmonary arterial hypertension (PAH) in a patient with congenital heart disease. The author is from Children's Hospital and Harvard University in Boston.


Risk factors for development of PAH:

(1) type of the congenital heart defect

(2) large defect

(3) high flow rate through and/or high pressure across the shunt


Risk of PAH

truncus arteriosus

high risk

atrioventricular canal defect

high risk

ventricular septal defect (VSD)

moderate risk

atrial septal defect (ASD)

low risk


A patient at increased risk for PAH is also more likely to experience an earlier age of onset.


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