We have experienced 3 cases of valvulectomy without prosthetic replacement. All patients had active infective endocarditis. Clinical states of the patient who received pulmonary valvulectomy and of the other who received partial tricuspid valvulectomy are stable in the late phase. They have no cardiomegaly. The patient who received total tricuspid valvulectomy has progressive cardiomegaly. It seems that the volume overload due to the regurgetant flow in the tricuspid position causes the dilatation of the right heart. Tricuspid valvuloplasty with remained tissue or with pericardial patch may be more recommendable.

A 3-month-old girl of univentricular heart of left ventricular type with atresia of left atrioventricular valve (LAVV) and coarctation of the aorta (Co/AO) is presented. UCG and angiography revealed concordant AV connection with straddling RAVV with transposed great arteries [SDDT]. The following pressures (in mmHg) were noted on catheterization: RA mean 1 (a=3, v=1), LA mean 12 (a=17, v=14), LV 84/0/8, Ao 81/41, and PA 74/39. Patent foramen ovale (PFO) was restrictive and balloon atrioseptostomy was not feasible. Blalock-Hanlon atrial septectomy (8×6mm), subclavian flap aortoplasy (SFA) and pulmonary arterial banding were performed simultaneously under bilateral thoracotomy. Acute renal failure occurred after surgery and the girl required peritoneal dialysis for 5 days. At 6 months after surgery, girl is doing well. There will be a predictable fall in pulmonary vascular resistance after atrial septectomy and SFA with a ligation of PDA may result transient increase in systemic resistance. Therefore, atrial septectomy and SFA in conjunction with pulmonary arterial banding should be done simultaneously.

A 2-year-old girl who had been followed because of pulmonary valve stenosis since birth, underwent unsuccessful balloon valvuloplasty twice. The angiographic study showed thin cusps and remarkably thick and immobile filling defects in the main pulmonary artery, which indicated supravalvular PS or pulmonary valve dysplasia. Operation was done under conditions of a beating heart and total cardiopulmonary bypass. Pulmonary valve dysplasia was localized on the edge of all three cusps. After resection of the dysplastic lesion, the stenosis was released and slight regurgitation was observed by ultrasonography study.

We report a very rare case of annuloaortic ectasia with an etiology of both aortitis syndrome and Marfan's syndrome. A 25-year-old woman showed AAE and AR. Her mother had died of SLE, but there was no Marfan's syndrome in her family. Her eyes were normal but her finger was long enough to show wrist sign and thumb sign. Urgent operation was performed because of her progressive heart failure. The ascending aorta was enlarged and Valsalva sinuses showed asymmetrical dilatation. The Cabrol operation was done with a composite graft of 23mm Medtronic Hall valve and 26mm Gelseal graft. The valve was sutured to the graft for 5mm from the end of graft to minimize the tension for annulus because of the high invidence of valve detachment and leakage in aortitis syndrome. Pathological study showed findings of both aortitis syndrome and Marfan's syndrome. Postoperative aortography showed good valvular function, and the patient is doing well now at 6 months after operation.