HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Figures Only Full Text Full Text (PDF) CME Test (opens in a new window) Submit a response Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Bardo, D. M. E. Articles by Saneto, R. P. Search for Related Content PubMed PubMed Citation Articles by Bardo, D. M. E. Articles by Saneto, R. P. Related Collections Pediatric Radiology Cardiac Radiology

Hypoplastic Left Heart Syndrome¹

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. Address correspondence to D.G.F.

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.

Index Terms: Heart, abnormalities, 51.1715 • Heart, anatomy, 51.92 • Heart, failure, 51.71 • Heart, flow dynamics, 51.92 • Heart, surgery, 51.451

This article has been cited by other articles:

				A. M. Gaca, J. J. Jaggers, L. T. Dudley, and G. S. Bisset III Repair of Congenital Heart Disease: A Primer-Part 1 Radiology, June 1, 2008; 247(3): 617 - 631. [Abstract] [Full Text] [PDF]

				A. M. Ilbawi, D. E. Spicer, S. Bharati, A. Cook, and R. H. Anderson Morphologic study of the ascending aorta and aortic arch in hypoplastic left hearts: Surgical implications J. Thorac. Cardiovasc. Surg., July 1, 2007; 134(1): 99 - 105. [Abstract] [Full Text] [PDF]

				V. Muthurangu, A. M. Taylor, S. R. Hegde, R. Johnson, R. Tulloh, J. M. Simpson, S. Qureshi, E. Rosenthal, E. Baker, D. Anderson, et al. Cardiac Magnetic Resonance Imaging After Stage I Norwood Operation for Hypoplastic Left Heart Syndrome Circulation, November 22, 2005; 112(21): 3256 - 3263. [Abstract] [Full Text] [PDF]