Risk factors for myocardial ischemia and early mortality in infants with Pulmonary Atresia with Intact Ventricular Septum: a scoping review

Infant mortality for Pulmonary Atresia with Intact Ventricular Septum (PAIVS) remains high (up to 20-30%), often in relation to development of myocardial ischemia in the presence of a diminutive right ventricle (RV) and single ventricle physiology. Although coronary artery anatomy is recognized as a major predictor of poor outcomes, its predictive value varies widely among studies and does not comprehensively determine mortality risk.

This scoping review aims to characterize the breadth and strength of association between anatomical and pathophysiological risk factors and myocardial ischemia, use of mechanical circulatory support, and early mortality in infants with Pulmonary Atresia with Intact Ventricular Septum (PAIVS).