Pediatria e Terapêutica
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Pulmonary Atresia with Intact Ventricular Septum: Management Options and Decision-making

Henry Burkholder and Duraisamy Balaguru

Pulmonary atresia with intact ventricular septum (PA-IVS) is a complex congenital heart malformation with a diverse set of anatomical and clinical findings. The incidence is 4.1 per 100,000 live births and is less than 1% of all congenital heart disease. During embryogenesis, PA-IVS is postulated to occur after development of ventricular septum which is later than the development of PA with ventricular septal defect. Every case of PA-IVS poses a considerable challenge to the pediatric cardiologist and cardiovascular surgeon. Although echocardiography is often the first line tool in cardiac imaging, cardiac catheterization is the gold standard for diagnosing PA-IVS and describing the important anatomical features that determine the plan of treatment. This article will focus on the management options and decision making from the interventional cardiologists point of view.