Tetralogy of Fallot with right pulmonary artery origin from the ascending aorta: noninvasive diagnosis and surgical correction in a 7-week-old infant

Anomalous origin of one pulmonary artery from the ascending aorta is a rare anomaly which is almost always associated with hypertension in the contralateral pulmonary artery originating from the right ventricle. We report echocardiographic evaluation of an infant with tetralogy of Fallot and aortic origin of the right pulmonary artery. Since all relevant information regarding anatomy and hemodynamics could be obtained by echocardiography, cardiac catheterization and angiography were unnecessary. Surgical correction including transatrial patch closure of the ventricular septal defect, resection of the infundibular stenosis and direct reimplantation of the right pulmonary artery was performed successfully at the age of 7 weeks. The postoperative course was uneventful and persistent pulmonary hypertension was excluded by echocardiography. Our case shows that complete echocardiographic evaluation is possible in infants with this complex cardiac malformation. We recommend reserving cardiac catheterization for those patients with echocardiographic evidence of significant elevation of pulmonary vascular resistance. In order to avoid irreversible pulmonary vascular changes early surgical correction within the first 3 months of life should be performed in all children with aortic origin of a pulmonary artery including those with associated tetralogy of Fallot.