Aspergillus infective endocarditis (ASIE) is a very rare disease that carries an extremely poor prognosis. We report a case of ASIE successfully treated by a tricuspid valve replacement and administration of an antifungal drugs. The patient was a 69-year-old man who was taking steroids for an autoimmune disease and was admitted to our hospital because of a persistent fever of 39°C. As chest CT showed infiltrative shadows in both lung fields and the aspergillus antigen was detected in the blood, we diagnosed invasive pulmonary aspergillosis (IPA), and initiated administration of micafungin sodium (MCFG). Later, the patient's heart failure worsened, and echocardiography revealed vegetation measuring 8 mm in diameter in the anterior cusp of the tricuspid valve. As this indicated a definitive diagnosis of ASIE, administration of voriconazole (VRCZ) was initiated. However, the vegetation grew into a movable wart measuring 20 mm in diameter within a week, based on which a diagnosis of drug-resistant ASIE was made, and surgery was considered indicated. Very large vegetations were found in the anterior cusp of the tricuspid valve, anterior papillary muscle, the tendinous chord of the medial papillary muscle and the trabeculae carneae of the right ventricle. Based on the findings, it was judged that tricuspid annuloplasty was impossible and tricuspid valve replacement was performed using a biological valve. As to the antifungal medication, long-term administration of VRCZ and MCFG was continued. The patient followed a favorable course and was discharged from the hospital on the 220th day. The patient aking lifelong VRCZ and has shown no evidence of recurrence of the ASIE. To improve the prognosis of ASIE, rapid and radical surgical resection of the vegetations and appropriate administration of antifungal drugs are important.

Four patients with d-TGA after atrial switch operation were treated with transvenous DDD pacemaker implantation for their postoperative dysrhythmia; complete atrioventricular block (CAVB) in two, sinus bradycardia with grade I atrioventricular block in one after the Mustard procedure and CAVB with sick sinus syndrome after the Senning procedure in one. From an anatomical point of view, the left atrial appendage was the only suitable anchoring site for the atrial lead. The patency of this cavity should be assessed by echocardiography and/or angiography before implantation. As for the ventricular lead, active fixation is recommended because of the relatively smooth endocardial surface of morphological left ventricle. Otherwise, transvenous DDD pacemaker implantation for patients after atrial switch operation was safely performed without any technical difficulties and with few complications.

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.

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.

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.

Under scheduled anticoagulation therapy, surgery for abdominal aortic aneurysm was performed in 4 patients who had undergone heart valve surgery and implantation of a mechanical prosthesis. Warfarin and antiplatelet agents were prescribed in all cases preoperatively. Antiplatelet agents were discontinued from seven to 10 days before operation. Warfarin was stopped from two to three days before operation and heparin (200IU/kg/day) was administered by continuous intravenous infusion to produce an activated clotting time of around 150 seconds. Bolus intravenous heparin of 3, 000 IU was added before aortic crossclamp. Oral anticoagulants were resumed from the beginning of oral intake, and heparin was stopped when the prothrombin time reached therapeutic levels (% PT=40%). In three patients perioperative courses were uneventful. Intraperitoneal hemorrhage occurred in one patient who simultaneously underwent cholecystectomy and aneurysmectomy with Y-grafting. He required blood transfusion and interruption of anticoagulation. Brain thromboembolism occurred in this patient 26 days after the operation. We believe that scheduled anticoagulation for the operation of abdominal aortic aneurysm is safe and useful in patients with prior prosthetic heart valve surgery. However, the coexistence of coagulopathy requires more intensive anticoagulation therapy.

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 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.