Cardiac CT in a 5-Month-Old Baby with VACTERL Syndrome after Cardiac Surgery

Torel Ogur, MD, Patrick T. Norton, MD, Klaus D. Hagspiel, MD
Department of Radiology and Medical Imaging, University of Virginia, USA

A 5-month-old baby boy with a history of double outlet right ventricle (DORV) with atrial septal defect (ASD), ventricular septal defect (VSD), patent foramen ovale (PFO) and VACTERL syndrome (unilateral renal agenesis, syndactyly, congenital hemivertebrae) was referred for cardiac CT. He underwent surgical repair with an ASD and VSD patch and PFO ligation at ten weeks of age. He was readmitted due to atrial tachycardia and worsening pulmonary hypertension. Cardiac catheterization revealed systemic pulmonary artery (PA) pressures and near atretic left pulmonary veins. The cardiac surgeon requested the CT for a detailed evaluation of the pulmonary veins prior to possible surgical repair.