Moyamoya in children with sickle cell anemia and cerebrovascular occlusion

Abstract

similar to that seen in Prinzmetal variant anginay These ECG changes lead them to postulate that prolonged coronary spasm caused the infarctions. They further theorized that this spasm might be a reflex phenomenon secondary to papillary muscle injury, associated with excessive papillary muscle traction due to the prolapsing valve. Another possible explanation is that myocardial ischemia is the primary event, injuring the papillary muscle and causing the mitral valve prolapse. Prolapsing mitral valves are sometimes associated with atherosclerotic coronary artery disease. TM In patients such as ours, who have angiographically normal coronaries, some mechanism such as coronary spasm would then have to be postulated to explain the myocardial infarction and papillary ischemia. In summary, our experience with this patient, as well as that of others with six previously reported patients, suggests an association between myocardial infarction with angiographically normal coronaries and the prolapsing mitral valve syndrome. Although the etiology of the infarctions in these patients is unsettled, coronary spasm is suggested by the ECG findings? Physicians should know of the existence of this potential rare complication of the prolapsing mitral valve syndrome.