Critical aortic stenosis in early infancy. Anatomic and echocardiographic substrates of successful open valvotomy.

To establish noninvasive criteria by which to manage infants with critical aortic stenosis, we examined 20 heart specimens from infants under 3 months of age and reviewed the clinical course and real time echocardiograms of 20 patients in the same age group. All 20 infants underwent open valvotomy, and in five cases both echocardiographic and postmortem measurements were available for the same heart. The anatomic specimens showed a spectrum of valvular, ventricular, and vascular abnormalities that could be accurately identified by echocardiography. A small left ventricular cavity was usually associated with a narrow ventriculoarterial junction, small ascending aorta, and narrow subaortic region. In these hearts, the mitral valve had a single or grossly hypoplastic papillary muscle with short or "arcuate" tendinous cords. A dilated left ventricular cavity had wider inflow and outflow orifices, and the tension apparatus of the mitral valve was either normal or supported by hypertrophic papillary muscles, at the other end of the spectrum. The survivors (n = 15) and nonsurvivors (n = 5) of open valvotomy showed significant differences in the echocardiographic dimensions of the left ventricle (p less than 0.005), the subaortic region (p less than 0.05), the ventriculoaortic junction (p less than 0.05), the ascending aorta (p less than 0.005), and the mitral valve orifice (p less than 0.001). Moreover, the papillary muscle of the mitral valve was invariably single or hypoplastic with short tendinous cords in the early nonsurvivors. Infants with unfavorable cardiac anatomy tended to present earlier (p less than 0.05) and to have a lower systemic blood pressure (p less than 0.05), and they required prostaglandin E2 to maintain right ventricular support of the circulation through a persistent arterial duct. This study suggests that patients with a small left ventricle (echocardiographic inflow dimension less than 25 mm), a narrow ventriculoaortic junction (less than 5 mm), and a small mitral valve orifice (less than 9 mm) will not achieve a satisfactory surgical result from aortic valvotomy. Such patients should be considered for cardiac transplantation or the Norwood-type of palliation for hypoplastic left heart syndrome.