Objective. We describe two infants aged two days and 1.5 months with congenital supravalvar aortic stenosis and myocardial ischemia.
Methods. Case reports.
Results. Both infants had supravalvar aortic stenosis with mean gradients by Doppler of 60 and 76 mmHg respectively, and also mild supravalvar pulmonary stenosis and pulmonary branch stenoses. Both had normal coronaries by 2D echocardiography. The older infant was confirmed to have Williams syndrome by chromosomal analysis. The newborn baby was operated by resection of the supravalvar stenosis at the age of two days, with a good result. However, coming off the by-pass evident ischemia was seen on the ECG, and also poor left ventricular function with low systemic and high pulmonary pressure, as well as significant mitral regurgitation. After two days of ECMO complete recovery was seen with good cardiac function and a normal ECG. The other infant became bradycardic with low blood pressure requiring resuscitation for half an hour at the beginning of a diagnostic cardiac catheterization. Emergency surgery was performed with a Doty patch plasty with a good result and an uneventful recovery.
Conclusion. In both cases the coronary arteries were exposed to a high blood pressure during a relatively short time and since the recovery of the ischemia was complete and fast there is no indication of coronary artery changes. The ischemia was likely due to poor perfusion of the hypertrophied left ventricular muscle at the time of compromised blood pressure after bypass and during anesthesia at the catheterization in the second case.