Hypoplastic Left Heart Syndrome

http://radiographics.rsna.org/content/21/3/705.full
Dianna M. E. Bardo, MD ; David G. Frankel, MD ; Kimberly E. Applegate, MD ; Daniel J. Murphy, MD ; Russel P. Saneto, DO, PhD

1 From the Departments of Radiology (D.M.E.B., D.G.F.), Pediatric Cardiology (D.J.M.), and Pediatric Neurology (R.P.S.), Cleveland Clinic Foundation and Children’s Hospital, 9500 Euclid Ave, Cleveland, OH 44195; and the Department of Radiology, University Hospital, Cleveland, Ohio (K.E.A.). Recipient of a Certificate of Merit award for a scientific exhibit at the 1998 RSNA scientific assembly. Received March 19, 1999; revision requested May 18 and final revision received August 24; accepted August 24.

Hypoplastic left heart syndrome (HLHS) is a complex combination of cardiac malformations that probably results from multiple developmental errors in the early stages of cardiogenesis and that, if left untreated, invariably proves fatal. A variety of chest radiographic findings are seen in patients with HLHS, including an enlarged cardiac silhouette (notably a prominent right atrium), pulmonary venous hypertension, an atrial septal defect, and valvular stenosis or atresia. The recent evolution of palliative surgical procedures (modified Norwood procedure, bidirectional cavopulmonary shunt, modified Fontan procedure, aortic valvuloplasty, heart transplantation) has increased the survival rate in children with HLHS. Echocardiography allows accurate assessment of the size and location of the ductus arteriosus, the hemodynamics of the aortic root, the patency and size of the foramen ovale or atrial septal defect, and the presence of a ventricular septal defect to help determine whether surgical intervention is appropriate and, if so, to facilitate planning. Pediatric radiologists now view radiologic images obtained in patients with HLHS before surgical intervention and at important intervals during treatment. Familiarity with the malformations that characterize HLHS and the surgical procedures used to enhance postnatal survival will help pediatric radiologists provide better care for patients with this relatively common pathologic condition.