What causes pulmonary hypertension in association with congenital heart disease

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Pulmonary artery hypertension (PAH) is associated with a variety of disease states and can also be idiopathic. When associated with congenital heart disease, PAH is usually the result of high blood flow and pressure in the pulmonary vasculature caused by left-to-right intracardiac shunting at the ventricular level. This situation is typically present with ventricular or atrioventricular septal defects, in which blood flows along a pressure gradient from the high-pressure left ventricle to the lower-pressure right ventricle.

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