Introduction Pulmonary atresia with intact ventricular septum is a congenital anomaly with total interruption of blood flow between the right ventricle and the pulmonary trunk, and in general it is not accompanied by associated defects, except for interatrial septal defect, present in 20%, with predominance of patent foramen ovale. Valve atresia results from the lack of embryological development of this fibrous structure, which can be located at the valve level (fibrous membrane) but also at the infundibular level (blind fundus). […]