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1/20. Congenitally corrected transposition with pulmonary atresia and intact ventricular septum.

    We describe a patient with the rare association of the heart in the left chest, congenitally corrected transposition, pulmonary atresia and an intact ventricular septum. There were associated fistulous communications between the morphologically left ventricle and the coronary arteries. Diagnosis was made by echocardiography, and subsequently confirmed by cardiac catheterization.
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2/20. dilatation of a restrictive interatrial communication using a balloon angioplasty catheter.

    Balloon atrioseptostomy is a life-saving procedure palliating certain congenital heart defects like transposition of the great arteries, right or left atrioventricular valve atresia, hypoplastic left heart syndrome, and pulmonary hypertension. Occasionally the Rashkind balloon septostomy technique may be ineffective in creating an adequate interatrial communication. We performed balloon dilatation of a restricted atrial septal defect using a balloon angioplasty catheter in a three-month-old infant.
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3/20. Left ventricle to coronary sinus fistula complicating the outcome of transposition of the great arteries.

    We report a case in which a neonate with transposition of the great arteries and intact ventricular septum with unrestricted atrial communication had persistent hypoxemia probably due to a congenital left ventricle to coronary sinus fistula.
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4/20. nitric oxide in neonatal transposition of the great arteries.

    Three newborn infants with transposition of the great arteries (TGA) and intact ventricular septum (IVS) developed postnatal persistent pulmonary hypertension of the newborn (PPHN) and were successfully treated with inhaled nitric oxide (iNO). Intervention with balloon atrial septostomy (BAS) was performed in two of the infants before the iNO treatment, but they continued to be severely hypoxic with metabolic acidosis. However, the iNO immediately improved oxygenation and the clinical condition. The third neonate had a moderately large atrial communication and echocardiographic signs of PPHN. He received iNO before BAS with dramatic clinical improvement, which therefore postponed BAS. CONCLUSION: early diagnosis of PPHN and treatment with iNO may improve final outcome in neonates with TGA and IVS. In the presence of moderately large atrial communication and PPHN, treatment with iNO might be considered before BAS.
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5/20. phentolamine as a treatment for poor mixing in transposition of the great arteries with adequate intraatrial communication.

    patients with transposition of the great arteries often show poor mixing for different reasons, even after adequate balloon atrial septostomy. We present a patient with such a lesion whose clinical status improved dramatically after phentolamine was applied. We believe this improvement is due to reduction in afterload caused by the alpha(2) blocker and also possibly as a response to a presumptive effect of the drug on the diastolic function of the right ventricle, allowing more left-to-right shunt across the atrial septal defect. Both phenomena can improve cardiac output in such a situation.
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6/20. Pericardial patch enlargement of Blalock-Hanlon atrial septectomy.

    A modified Blalock-Hanlon operation is described, in which both the right and left atriotomies were bridged by a generous pericardial patch. This patch significantly enlarged the interatrial communication and markedly increased arterial blood saturation.
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7/20. coronary sinus septal defect associated with tricuspid atresia.

    coronary sinus septal defects are unusual congenital anomalies that may assume particular clinical importance in patients undergoing a modified fontan procedure. Two of 10 patients with tricuspid atresia who underwent a modified fontan procedure between January, 1984, and December, 1984, were noted to have coronary sinus septal defects in the early postoperative period. A pathologic review of 159 specimens of tricuspid atresia revealed coronary sinus septal defects in four. Although infrequent, coronary sinus septal defects may permit significant persistent right-to-left interatrial shunting after a modified fontan procedure. Ideally, preoperative recognition allows for selective direct closure or closure of the coronary sinus orifice. Alternatively, routine baffling of the coronary sinus into the left atrium obviates this potential source of interatrial communication.
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8/20. An unusual complication of a conduit-mounted homograft: valve leaflet fixation in the open position.

    Surgical management of left ventricular outflow tract obstruction in patients with complete transposition of the great arteries and an intact ventricular septum is difficult. Direct resection of subpulmonary stenosis has not produced satisfactory results. Thus, relief of stenosis has been accomplished with valved conduits from the left ventricle to the pulmonary artery. Initially, conduit tissue valves were porcine in nature. More recently, human-origin conduit tissue valves have been employed. In this report, we describe an unusual complication in a child with complete transposition in whom a homograft valve conduit was placed from the left ventricle to the pulmonary artery to relieve subpulmonary stenosis. The leaflets became fixed in an open position. Thus, the conduit was converted to a functionally nonvalved communication. The subpulmonary stenosis regressed, allowing egress from both the natural outflow tract and the conduit.
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9/20. Assessing the blalock-hanlon atrioseptectomy at cardiac catheterization.

    Blalock-Hanlon atrial septectomy (BH) is performed to increase systemic and pulmonary venous mixing at the atrial level. Failure of clinical improvement may be related to inadequate intraatrial communication. A simple catheterization technique employing a balloon catheter is used to assess the presence or absence of the limbus of the foramen ovale following BH, since persistence of the limbus of the fossa ovalis after BH is associated with significantly smaller percentage of communication (atrial communication area/total atrial area).
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10/20. The location and course of the atrioventricular conduction system in common atrioventricular orifice and in its related anomalies with transposition of the great arteries--A histopathological study of six cases.

    The location and course of the atrioventricular (AV) conduction system were studied histopathologically in 6 hearts by sectioning serially, 4 having common AV orifice (CAVO) with complete or partial transposition of the great arteries (TGA) and 2 having ventricular septal defect of the persistent common AV canal type (VSD-C) with complete TGA. Two cases of CAVO with TGA and asplenia syndrome (Cases 1 and 2) had 2 discrete AV conduction systems, being posterior and anterior to the site of the defect, respectively. In these 2 cases posterior AV conduction system well developed, whereas the anterior one was hypoplastic. In another case of CAVO with TGA and asplenia syndrome (Case 3), only the anterior AV conduction system existed near the base of the great arteries. In the other case of CAVO with TGA and polysplenia syndrome (Case 4), the posterior AV conduction system was found to have a congenital interruption of the AV bundle of his. Two cases of VSD-C with TGA but with no splenic anomaly (Cases 5 and 6) showed the posterior AV conduction system with communication-free accessory bundles. The posterior AV node, bundle of his and left bundle branch inevitably shifted postero-inferiorly, except in Case 3. The bundle branches were always distributed appropriately to their morphologically matched ventricles. The superiorly oriented vector in the mean frontal QRS axis in Cases 1, 5 and 6 seemed to be related to the postero-inferior displacement of the posterior AV conduction system, whereas those of the left-inferior oriented vector in Cases 2 and 3 were thought to be responsible for the excessively unbalanced size of ventricles. The complete AV block in Case 4 was correlated with the interruption of the bundle of his. The morphogenesis of the anterior AV conduction system was discussed in relation to the asplenia syndrome.
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