A baby girl with a low birth weight was given a diagnosis of congenital bicuspid aortic stenosis and mitral valve prolapse. At the age of 40 days, she underwent balloon aortic valvotomy, but significant aortic regurgitation appeared afterwards. Another surgical intervention became necessary by the age of 20 months (weight, 5.7 kg), because of intractable heart failure mostly caused by exacerbated mitral regurgitation. We performed a leaflet extension valvuloplasty for the small bicuspid aortic valve using an autologous pericardium treated by glutaraldehyde. The mitral valve was replaced with an ATS-16AP valve. Although her postoperative course was complicated with mitral paravalvular leakage and poor left ventricular function, she was discharged from hospital 6 months post operatevely. Leaflet extension valvuloplasty is a surgical option for infants with a small aortic annulus, but the procedure could be the only solution in cases when Konno or Ross techniques are not suitable.

A 22-year-old man was hospitalized due to severe back pain having being diagnosed as Stanford type A aortic dissection, AAE, mitral regurgitation and pectus excavatum associated with Marfan's syndrome. A single staged operation including ascending aortic replacement, mitral valve replacement and sternal turnover with a rectus muscle pedicle was carried out in order to keep the blood supply to the plastron to reduce the risk of infection during such a long operation. By this approach, it was found that the operative field was excellent and postoperative hemodynamics were stable. However, frail plastron occurred because of difficulties in keeping the patient stabilized because of severe pain thus re-fixation was required. The necessity of strong pain control after such an operation was also recognized.

Lung hemorrhage associated with pulmonary reperfusion injury is a rare but lethal condition. We presented a case salvaged by non-heparinized extracorporeal life support for massive lung hemorrhage after pulmonary thromboembolectomy. Sub-acute pulmonary thromboembolism with a floating right atrial thrombus was diagnosed in 63-year-old woman by computed tomography and echocardiography. An emergency pulmonary thromboembolectomy was performed using cardiopulmonary bypass and moderate hypothermia. Immediately after reperfusion, extraordinary lung hemorrhage occurred and continued. We decided to take over the standard cardiopulmonary bypass with a non-heparinized extracorporeal life support system. Fortunately, hemostasis of the lung hemorrhage was completely secured within 12h, and the extracorporeal life support was terminated at 20h after the surgery. The patient was extubated at 48h after the surgery, and was discharged after the insertion of an inferior vena cava filter for a floating deep venous thrombus. Although the necessity, efficacy and risk of the non-heparinized extracorporeal life support should be clarified, we conclude that it could be the treatment of choice for life threatening lung hemorrhage associated with pulmonary reperfusion injury.

Supravalvular aortic stenosis is a rare obstructive lesion of the left ventricular outflow tract localized at the level of sinotubular junction. It has been recognized that supravalvular stenosis may occur as a part of Williams syndrome and is sometimes complicated by obstruction of the left main coronary artery. We successfully performed single patch augmentation for supravalvular aortic stenosis and left coronary ostial stenosis with concomitant aortic valvotomy in a child without Williams syndrome. The patient had been followed as congenital bicuspid aortic valvular and supravalvular stenosis. At the age of 3 years, cardiac catheterization revealed an increased pressure gradient of 90mmHg at the left ventricular outflow and newly developed ostial stenosis of the left coronary artery. An oblique incision on the ascending aorta was made above the sinotubular junction and extended leftward onto the left main coronary artery, and this incision opened the fibrous ridge at the left coronary artery. After commissurotomy for the bicuspid valve, both the supravalvular and ostial stenosis were augmented with a single autologous pericardial patch treated by glutaraldehyde. The pressure gradient was significantly reduced and the ischemic left ventricular dysfunction was eliminated.

A 61-year-old man was referred to our hospital for treatment of hemolytic anemia after ascending aortic replacement aortic dissection. Cine mode magnetic resonance imaging (MRI) showed stenosis at the proximal anastomostic site of a Teflon strip. We diagnosed hemolytic anemia induced by collision of red blood cells on the inverted felt strip. Conservative therapy with Sarpogrelate and β-blockers was effective to treat his hemolytic anemia. However, 7 years later he was re-admitted because of infective endocarditis at the aortic valve, and underwent aortic root replacement. Intraoperative findings showed a stiff and inverted Teflon felt strip causing stenosis of the proximal anastomosis. Hemolytic anemia should be considered a rare complication of using a Teflon felt strip to reinforce anastomosis for acute aortic dissection.

A premature boy was born after 35 weeks gestation (1,561 g in weight) with a diagnosis of anomalous origin of the right pulmonary artery from the ascending aorta (AORPA) and perimembranous ventricular septal defect (VSD). The fourteenth day after birth, banding of the right pulmonary artery was performed as a palliative operation. At the age of 5 months (3.9 kg in weight), the right pulmonary artery branched from the ascending aorta, and was anastomosed to a flap made by the lateral wall of the main pulmonary artery with pericardial patch augmentation. VSD patch closure was performed concomitantly. Severe stenosis of the right pulmonary artery compressed by the ascending aorta and left pulmonary hypertension were revealed 3 weeks after the repair. At the age of 11 months, a surgical relief of the right pulmonary artery stenosis was performed. Transection of the ascending aorta provided an excellent exposure of the right posterior pulmonary artery. After patch plasty of the stenotic pulmonary artery, the divided ascending aorta was restored using a strip form patch on 4/5 circle of its posterior wall to extend the aorta and widen the space for the right pulmonary artery. This technique preserves growth potential of the ascending aorta. There are few reports of surgical repair of AORPA with VSD in low birth weight infants. We presented here a case with surgical relief of post-operative right pulmonary artery stenosis. Long term observation of repaired right pulmonary artery, and requiring residual slight hypertension of the left pulmonary artery.