Management of critical aortic stenosis in infancy

Critical aortic valvular stenosis presents in infancy with severe congestive heart failure. Clinical assessment and electrocardiography are of value, but cardiac catheterization with angiography has been considered mandatory prior to surgical treatment. With cross-section echocardiography an accurate diagnosis of aortic stenosis and associated lesions is possible. Over the past 2 years, we have established a protocol according to which, if a clinical diagnosis of critical aortic stenosis is confirmed by cross-sectional echocardiography in the absence of major associated cardiac anomalies, infants are submitted for aortic valvotomy under inflow occlusion without invasive studies. This protocol was used in an effort to decrease the mortality rate by avoiding the preoperative stress of cardiac catheterization and angiography, as well as the hazards of cardiopulmonary bypass in the severely ill infant. Eight infants with critical aortic stenosis have been operated upon, five without prior cardiac catheterization. Ages at operation ranged from 2 days to 7 months, with six children less than 2 weeks of age. The noninvasive diagnosis was confirmed at operation in each case. There was one early postoperative death and one late death. No death has been related to the technique of inflow occlusion. A decision tree for the noninvasive assessment of suspected critical aortic stenosis based on the clinical features and echocardiographic findings is presented.