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Eisenmenger syndrome, a type of congenital heart disease, causes pulmonary hypertension. It's most commonly caused by a large hole in your heart between the two lower heart chambers (ventricles), called a ventricular septal defect. This hole in your heart causes blood to circulate abnormally in your heart.
Definition. 1  Pulmonary hypertension is a rare lung disorder characterized by increased pressure in the pulmonary artery. The pulmonary artery carries oxygen-poor blood from the lower chamber on the right side of the heart (right ventricle) to the lungs where it picks up oxygen.