Prenatal diagnosis of a coronary fistula in a fetus with pulmonary atresia with intact ventricular septum and trisomy 18

Infants with trisomy 18 have severe mental retardation along with anomalies of the nervous, cardiovascular, gastrointestinal, genitourinary, and musculoskeletal systems; survival beyond 1 year of age is rare. Even with successful repair of the cardiac defects, the prognosis would not be altered greatly. The most common cardiac malformations described in association with trisomy 18 are ventricular septal defect, tetralogy of Fallot, double-outlet right ventricle, and abnormalities of the atrioventricular and semilunar valves. Prenatal diagnosis of pulmonary atresia with intact ventricular septum has been reported. We report a case of pulmonary atresia with intact ventricular septum in association with trisomy 18 and a coronary fistula demonstrated by fetal echocardiography.