• A congenital heart lesion that increases pulmonary arterial blood flow
• Results in left-to-right shunt, results in lung infection, pulmonary vascular congestion, pulmonary artery (PA) hypertension, right heart failure, pulmonary vasoconstriction, pulmonary vascular obstructive disease
• Eisenmenger syndrome: Increased pulmonary hypertension such that left-to-right shunt ceases and shunt becomes right-to-left, requiring heart-lung transplant
• Inhaled nitric oxide, oxygen, or IV tolazoline reverses PA vasoconstriction
• PA band is palliative and can reduce PA flow to alleviate RV failure and progression of pulmonary hypertension
• Defects occur in 4 anatomic positions
-Perimembranous septum (85%), anterior to crista supraventricularis, beneath leaflet of tricuspid valve, or muscular septum
• Often associated with more complex defects: Truncus arteriosus, AV canal defect, tetralogy of Fallot, transposition of great arteries
• Isolated perimembranous defects may have associated patent ductus arteriosus (PDA), aortic coarctation
• Supracristal defects may have aortic regurgitation