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1/130. Successful correction of double-outlet right ventricle with a ventricular D-l-malposition of the great arteries, bilateral conus, pulmonary stenosis and subaortic ventricular septal defect.

    The authors present the case of a fifteen-year old girl with double outlet right ventricle with ventricular d-loop and l-malposition of the great arteries, bilateral conus, pulmonary stenosis and subaortic ventricular septal defect, who was operated on successfully. This is the fourth case of double outlet right ventricle with l-position of the aorta that has been surgically corrected. The subaortic position of the interventricular defect favours the creation of the tunnel connecting the left ventricle with the aorta without obstructing the right ventricular outflow tract. The patient was doing well 11 months postoperatively.
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keywords = operative
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2/130. Left ventricle to pulmonary artery conduit in treatment of transposition of great arteries, restrictive ventricular septal defect, and acquired pulmonary atresia.

    Progressive cyanosis after banding of the pulmonary artery in infancy occurred in a child with transposition of the great arteries and a ventricular septal defect, and a Blalock-Taussig shunt operation had to be performed. At the time of correction a segment of pulmonary artery between the left ventricle and the band was found to be completely occluded so that continuity between the left ventricle and the pulmonary artery could not be restored. A Rastelli type of operation was not feasible as the ventricular septal defect was sited low in the muscular septum. Therefore, in addition to Mustard's operation, a Dacron conduit was inserted from the left ventricle to the main pulmonary artery to relieve the obstruction. Postoperative cardiac catheterization with angiocardiography indicated a satisfactory haemodynamic result. The patient remains well 11 months after the operation. This operation, a left ventricle to pulmonary artery conduit, may be used as an alternative procedure in patients with transposition of the great arteries, intact interventricular septum, and obstruction to the left ventricular outflow, if the obstruction cannot be adequately relieved.
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keywords = operative
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3/130. dna polymorphism analysis in transfusion-associated graft-versus-host disease.

    During cardiac surgery for transposition of the great arteries at age 7 weeks, a female infant received blood, fresh frozen plasma and platelet transfusions. Eleven days postoperatively, she developed bloody diarrhoea, fever, an erythematous macular rash, hepatomegaly, seizures and pancytopaenia. A clinical diagnosis of transfusion related graft-versus-host disease (GVHD) was supported by skin histopathology. dna polymorphism studies confirmed that circulating lymphocytes in peripheral blood and infiltrating cells in the skin were foreign in origin and were derived from transfused blood cells. No underlying immunodeficiency was identified. Treatment with steroids cyclosporin and antithymocyte globulin was unsuccessful and death occurred 2 months after surgery. The features of fever, rash, diarrhoea, liver dysfunction and pancytopaenia which characterize GVHD may mimic drug reactions or viral infection. In addition to histological features on skin biopsy. dna polymorphism studies on skin and blood samples provide a unique and sensitive method to confirm GVHD. Irradiation of blood products should be considered for acutely compromised infants requiring urgent cardiac surgery.
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ranking = 1
keywords = operative
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4/130. Subaortic obstruction after the use of an intracardiac baffle to tunnel the left ventricle to the aorta.

    Postoperative hemodynamic studies in five patients document subaortic obstruction after surgical repair utilizing an intracardiac baffle to establish continuity between the left ventricle and the aorta. Four of the patients had a Rastelli procedure for D-transposition of the great arteries with a ventricular septal defect and pulmonary stenosis; one patient had repair of double outlet right ventricle with a ventricular septal defect and pulmonary stenosis. The left ventricular outflow was shown to be a long narrow tunnel by angiography in four of five patients and by echocardiography in one patient. Resting aortic peak systolic pressure gradient ranged from 10 to 42 mm Hg (mean 24). The obstruction was localized to the proximal end of the left ventricule to aorta tunnel (i.e., at the site of ventricular septal defect) in five patients. One patient with a gradient of 42 mm Hg has angina and decreased exercise tolerance. Subaortic obstruction is a newly described sequelae after the Rastelli procedure for transposition or repair of double outlet right ventricle. The obstruction may be hemodynamically significant and should be searched for at postoperative cardiac catheterization.
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ranking = 2
keywords = operative
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5/130. Management of postoperative chylothorax with nitric oxide: a case report.

    OBJECTIVE: To describe the use of inhaled nitric oxide in the management of refractory postoperative chylothorax. DESIGN: Case report. SETTING: A pediatric intensive care unit of a tertiary care children's hospital. PATIENT: A neonate with refractory chylothoraces complicated by moderate pulmonary hypertension after a complicated arterial switch operation. INTERVENTIONS: Administration of inhaled nitric oxide through a ventilator circuit. MEASUREMENTS AND MAIN RESULTS: The institution of inhaled nitric oxide at 20 ppm resulted in a marked reduction in chest tube drainage and a decrease in echocardiographically estimated pulmonary artery pressure from 50%-75% systemic to 30%-50% systemic. Chest tube drainage doubled when the nitric oxide was decreased to 10 ppm and, again, dramatically decreased after raising nitric oxide back to 20 ppm. After 8 days of nitric oxide therapy, the chest tube drainage ceased. nitric oxide therapy was successfully discontinued 19 days after initiation, with no recurrence of chylothorax. There was no effect of nitric oxide on systemic blood pressure. methemoglobin levels while on NO remained <1.7%. CONCLUSION: Consideration may be given to the use of inhaled nitric oxide in the therapy of refractory chylothoraces complicated by central venous hypertension.
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ranking = 5
keywords = operative
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6/130. Double-switch operation for congenitally corrected transposition and Ebstein's malformation.

    An infant is described with congenitally corrected transposition and Ebstein's malformation. Banding of the pulmonary trunk had been previously performed because of a muscular ventricular septal defect. The patient underwent the double-switch procedure with the intention of unloading the morphologically right ventricle and the malformed tricuspid valve. This resulted in prompt postoperative functional and haemodynamic improvement.
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keywords = operative
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7/130. Aortopulmonary collateral artery embolization during postoperative extracorporeal membrane oxygenation after arterial switch procedure.

    Aortopulmonary collateral arteries sometimes complicate cyanotic congenital heart defects. Combined with a relevant left-right shunt, this could result in massive airway bleeding during and after corrective surgery. A preoperatively diagnosed 1.2 mm small aortopulmonary collateral artery in a newborn suffering from transposition of the great arteries caused life-threatening airway bleeding during surgery. Postoperative extracorporeal membrane oxygenation (ECMO) was necessary, and coil embolization was performed on ECMO to terminate pulmonary bleeding.
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ranking = 6
keywords = operative
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8/130. Intraatrial mitral valve insertion with native valve preservation in children.

    Two patients underwent intraatrial mitral valve insertion for an unsuccessful valvotomy for severe mitral stenosis and left-sided atrioventricular valve insufficiency associated with corrected transposition utilizing a porcine valve from a valved conduit with preservation of the native valve. The valves were inserted using continuous suture distally at the mitral annulus and proximally at the pulled atrial wall distal to the pulmonary veins. Both patients had uneventful hospital course and are doing well at up to 6 months postoperatively. This approach provides a viable option for congenital mitral stenosis or insufficiency in children.
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ranking = 1
keywords = operative
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9/130. Intraoperative myocardial ischemia recognized by transesophageal echocardiography monitoring in the pediatric population: a report of 3 cases.

    We used continuous intraoperative transesophageal echocardiography (TEE) monitoring to detect intraoperative myocardial ischemia in children after they had been weaned from cardiopulmonary bypass for cardiac surgery. Three pediatric patients are described here to illustrate the usefulness of such TEE monitoring in surgical procedures involving coronary arteries. The indications for intraoperative TEE monitoring and a simplified scheme for immediate qualitative interpretation are discussed.
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ranking = 7
keywords = operative
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10/130. Third-degree burns due to intraoperative use of a Bair Hugger warming device.

    We report the case of a 3-year-old boy who underwent correction of transposition of the great arteries who developed burns from use of a patient warming device. His repair had been delayed because he was from a developing country, and he was offered surgery as part of a humanitarian effort. Postoperatively he was noted to have second- and third-degree burns from use of a Bair Hugger (Augustine Medical, Eden Prairie, MN) warming system after cardiopulmonary bypass.
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ranking = 5
keywords = operative
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