10 Pulmonary Atresia and Intact Ventricular Septum
A 30-year-old white woman, gravida 2, para 1+0, referred at 21 weeks’ gestation for an abnormal fetal four-chamber view on prenatal ultrasound. The mother has a 2-year-old daughter with a hypoplastic right heart and pulmonary atresia.
d. Cardiac catheterization and angiography are recommended to demonstrate coronary sinusoids (by RV angiogram) and rule out coronary artery anomalies such as stenosis. In many pediatric cardiac centers, radiofrequency perforation of the RV outflow tract (RVOT) is possible at cardiac catheterization, eliminating the need for surgery when the RV can otherwise sustain sufficient pulmonary circulation.