Congenital Aortic Valvular Stenosis: report of a case

선천성 대동맥판막 협착증 치험 1례

The incidence of congenital aortic valvular stenosis has been known rare, and approximately 3-6% of congenital heart diseases. Recently, we experienced 1 case of congenital aortic valvular stenosis, and which was corrected surgically under extracorporeal circulation successfully. A 11 years old male pt. was admitted to N.M.C. because of dyspnea, dizziness, chest pain and episode of syncope. An auscultation, harsh systolic murmur [Gr. IV/VI] was noted at aortic area and also palpable strong thrill. ECG showed LVH c strain pattern and suspicious LVH finding in simple chest P-A film. In Lt. cardiac catheterization, abrupt pressure change [110mmHg] between LV & Aorta was noted across the aortic valve. And aortic insufficiency was absent, well visualized both coronary arteries and suspicious bicuspid aortic valve in aortography. Valve form was bicuspid, large one was noncoronary cusp and another cusp was Rt. & Lt. coronary cusp which was interpositioned rudimentary commissure. Central aortic orifice was about 5ram in diameter. Valvulotomy was done along the fusioned commissure between noncoronary cusp and Rt. & Lt.coronary cusp, and then short incision was added between Rt. coronary cusp & Lt. coronary cusp. Immediate postoperative course smooth but unknown cardiac arrest was noted in POD second day. Complete recovery was done without sequelae by resuscitation. After operation, clinical symptoms were subsided but systolic murmur [Gr. II/VI] was audible at aortic area, diastolic murmur was absent. ECG showed still remained LVH but much decreased R wave voltage in Lt. precordial leads. Simple chest P-A showed no interval changes compared to preop film. Control Lt. heart catheterization revealed still remained pressure gradient [40ramrig] between LV & Aorta. But much decreased pressure gradient compared to preop pressure gradient [110mmHg].