(RadioGraphics. 1983;3:679-709.)
© RSNA, 1983

Real-time ultrasonic diagnosis of valvular aortic atresia and other hypoplastic left heart syndromes

¹ From the Department of Radiology, The Children's Medical Center, Dayton, Ohio.
² From the Department of Radiology, Grandview Hospital, Dayton, Ohio.

Seven infants with aortic valve atresia, seen since real-time echocardiography became available at Dayton Children's Medical Center in 1979, have had similar two-dimensional ultrasonograms. All have had mitral stenosis or atresia, diminutive, thick walled left ventricles, minute aortic valve rings, and ascending aortas with internal diameters of less than 4 mm extending into the suprasternal region. A large right atrium, right ventricle, and large pulmonary arteries were present in all cases. Table III summarizes our approach to the neonate with congestive heart failure and diminished pulses. When aortic valve atresia is demonstrated, medical management is instituted. Infants with abnormal real-time echocardiograms who do not have the characteristic findings of aortic valve atresia are referred for further cardiac evaluation and catheterization. When a normal real-time study is seen, immediate work-up for sepsis, central nervous system disease, and other medical abnormalities is initiated.

Two-dimensional, real-time echocardiography gives important anatomic information pertinent to surgical decisions in the less severe forms of hypoplastic left heart syndrome. It can also provide information critical to surgical decisions in the management of aortic atresia, a more severe form of the anomaly, the repair of which has recently met with limited success (Norwood). Experience and successful results are needed, however, before these neonates are routinely subjected to surgical therapy. In conclusion, real-time echocardiography, as a bedside technique, makes a major contribution to the diagnostic evaluation of neonates suspected of having hypoplastic left heart syndrome.