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 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 6-year-old boy who had been found to have hypertrophic obstructive cardiomyopathy presented with severely limited symptoms of heart failure due to progressive left ventricular outflow obstruction. Cardiac catheterization revealed the peak systolic pressure gradient of 87mmHg at left ventricular outflow, and systolic anterior motion of the anterior mitral leaflet with concomitant mitral regurgitation was observed by echocardiography. Transaortic septal myectomy was performed using transesophageal echocardiography guidance before, during and after surgery. Although the patient needed permanent pacemaker implantation for postoperative complete heart block, the procedure reduced the left ventricular outflow obstruction and relieved his symptoms.

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 cyanotic baby boy was given a diagnosis of single right ventricle, double outlet right ventricle, hypoplastic aortic arch, mitral atresia, atrial septal defect and pulmonary-ductus-descending aorta trunk. On day 4, extended aortic arch anastomosis and pulmonary artery banding were undertaken. At age 70 days, severe cyanosis and respiratory distress appeared and advanced rapidly. Angiography revealed critical subaortic stenosis and pulmonary hypertension, and the patient required urgent Damus-Kaye-Stansel anastomosis with concomitant right modified Blalock-Taussig shunt. Patients with single ventricle and hypoplastic aortic arch are a high-risk subgroup of progressive subaortic stenosis after initial pulmonary artery banding, and therefore need careful observation and may require early relief of subaortic stenosis.

There is an impression among cardiothoracic surgeons that the technique of profound hypothermic circulatory arrest (PHCA) is associated with an increased bleeding tendency compared to conventional bypass surgery. In addition to the recognized factors contributing to the hemorrhagic tendency seen in moderate hypothermic cardiopulmonary bypass (CPB), it is likely that the lower temperature utilized in PHCA may exacerbate platelet dysfunction. In this report, platelet counts and functions at the same cardiopulmonary bypass time were compared in human PHCA surgery (hypothermia group, n=16) and moderate hypothermic cardiopulmonary bypass surgery (control group, n=20). Mean platelet count corrected by hematocrit in the hypothermia group at 2h of CPB was significantly lower than in the control group (3.7×10⁴μl vs. 11.4×10⁴/μl, p<0.0001). In the hypothermia group, there were significant increases in the percentage of GMP-140 (P-selectin)-positive platelets (11.8% vs. 8.3%, p=0.0091) at 1h of CPB, and also in microparticles (24.8% vs. 10.5%, p<0.0001) and aggregated platelets (3.4% vs. 1.4%, p=0.0058) at 2h of CPB. Profound hypothermic circulatory arrest used in surgery for aortic arch aneurysm or dissection may cause irreversible platelet dysfunction and contribute to hemorrhagic tendency during the surgery. To minimize platelet dysfunction during CPB, the lowest blood temperature should be maintained above 15°C.

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.

The incidence of ventricular subepicardial aneurysm following myocardial infarction is quite low. We report a case of subepicardial aneurysm that was diagnosed on postoperative pathohistologic examination. A 69-year-old man was admitted to our hospital because of left ventricular aneurysm following myocardial infarction. The patient had left main trunk disease, triple-vessel coronary artery desease and low output syndrome. Under cardiopulmonary bypass with the heart arrested, the aneurysm was resected and the defect was closed. The suture line was reinforced using Teflon felt and GRF glue. A saphenous vein graft was anastmosed to the left anterior descending artery. On pathohistologic examination, the wall of the aneurysm was found to be composed of fibrotic tissue, myocardial fibers, medium-sized pericardial arteries, epicardium and fibrin thrombi. We diagnosed this as subepicardial aneurysm.

Seven patients with congenital heart defects suffered from multiple major hemorrhages from the lung after surgery and 5 of them died at 8 to 54 postoperative days because of respiratory insufficiency. In a patient with tetralogy of Fallot associated with pulmonary atresia, bleeding occured after the second shunt operation, presumably from rupture of bronchial collateral vessels. The clinical diagnoses of the other 6 patients were coarctation of the aorta (CoA) with common atrioventricular canal (CAVC) in 1, triple shunt in 1, persistent truncus arteriosus in 2, total anomalous pulmonary venous connection in 1 and CAVC in 1. Subclavian flap aortoplasty was performed without pulmonary artery banding in the patient with CoA and CAVC, whereas complete repair was performed in the other 5 patients. As these patients were associated with severe pulmonary hypertension preoperatively and 4 of them encountered pulmonary hypertensive crisis, the hemorrhage from the lung may be related to pre and postoperative high pressure of the pulmonary artery. Dilatation and rupture of the pulmonary capillary net was demonstrated in the patient with CoA and CAVC. These findings suggest the hypothesis that bleeding occurred due to rupture of the capillary net as a result of transmission of high pressure. Major bleeding from the lung is a rare but catastrophic complication after repair of congenital heart defects. As the treatment is difficult, early surgical intervention and treatment of postoperative pulmonary hypertension are important in complex lesions with severe pulmonary hypertension.

We performed surgical treatment for 21 patients of airway obstructions associated with congenital heart disease from December 1986 to March 1993. In all patients perioperative bronchoscopy demonstrated the cause and site of airway obstructions. Seven patients with corrective cardiac surgery (7/7), 7 with palliative cardiac surgery (7/10) and 2 with surgery for airway diseases (2/4) could be weaned from respirators following surgical treatment. Five patients died postoperatively. A respirator was required in 16 patients (76%) preoperatively. The suspension of pulmonary artery with intraoperative bronchoscopy was carried out in 6 patients. Five (5/6) were successfully extubated earlier postoperative day (mean 8.4 days), whereas only five in 10 patients without that procedure could be weaned from the respirator at a mean of 2 months. Identification of potential airway obstruction and early extubation is needed to reduce the mortality and morbidity caused by airway obstruction associated with congenital heart disease. Preoperative bronchoscopy is useful for diagnosis of airway obstructions and essential for decision making concerning surgical treatment. To early extubation in patients with marked airway obstructions, we recommend appropriate choice of the surgical procedure combined the suspension of pulmonary artery.