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1/52. 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 = catheterization
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2/52. 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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keywords = catheterization
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3/52. Retrograde catheterization of the pulmonary veins following surgical correction of transposition of the great vessels.

    Prior to a Mustard repair for transposition of the great vessels, complete physiologic data of the pulmonary bed can be obtained by catheterization of the pulmonary artery with a "J" formed wire, and direct catheterization of the pulmonary veins via an atrial septal defect. Following "physiologic" repair ready access to the pulmonary veins is denied. Evaluation of the pulmonary venous pressure is of great importance, however, in differentiating pulmonary vascular disease from pulmonary hypertension secondary to patch obstruction of pulmonary venous return. This paper describes a technique of catheterization of the pulmonary veins following a Mustard repair for TGV.
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keywords = catheterization
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4/52. Congenitally corrected transposition of the great arteries (L-TGA) with situs inversus totalis in adulthood: findings with magnetic resonance imaging.

    diagnosis of congenitally corrected transposition of the great arteries (L-TGA) with situs inversus totalis in two adult patients was made by magnetic resonance imaging (MRI). Visualization of the complete anatomy and quantification of ventricular function was possible. Relevant concomitant disease such as perimembraneous ventricular septal defect, atrial secundum septal defect, tricuspid regurgitation, valvular pulmonic stenosis, and pulmonary artery dilatation were clearly depicted by MRI using standard spin-echo and gradient-echo techniques. Findings were confirmed by cardiac catheterization in both patients. In this rare and complex congenital cardiac anomaly, MRI is an excellent imaging modality as echocardiography may be difficult to interpret due to restricted imaging windows. MRI may help in the decision about the necessity to undergo further invasive evaluation and may help to make cardiac catheterization a straightforward procedure.
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keywords = catheterization
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5/52. 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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keywords = catheterization
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6/52. Pulmonary venous obstruction in patients with transposition of the great arteries.

    We report two cases of complete transposition of the great arteries with pulmonary venous obstruction which was diagnosed by intraoperative transesophageal echocardiography, but was not detected by transthoracic echocardiography and cardiac catheterization. The pulmonary venous obstruction was relieved simultaneously with arterial switch operation. The intraoperative transesophageal echocardiography has great contribution for thorough evaluation of the pulmonary venous return of the patients.
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ranking = 1
keywords = catheterization
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7/52. [Echocardiographic diagnosis of transposition of the great arteries associated with anomalous pulmonary venous connection]

    We report 2 cases of transposition of the great arteries associated with anomalous pulmonary venous connection emphasizing the clinical findings, the diagnosis, and the evolution of the association. One of the patients had the anomalous pulmonary venous connection in its total infradiaphragmatic form, in the portal system, and the other patient had a partial form, in which an anomalous connection of the left superior lobar vein with the innominate vein existed. At the time of hospital admission, the patients had cyanosis and respiratory distress with clinical findings suggesting transposition of the great arteries. The diagnosis in 1 of the cases, in which the anomalous connection was partial, was established only with echocardiography, without invasive procedures that would represent risk for the patient; in the other case, in which the anomalous connection was total, the malformation was only evidenced with catheterization. The patients underwent surgery for anatomical correction of the heart disease. Only 1 patient had a good outcome.
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ranking = 1
keywords = catheterization
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8/52. Right atrial tunnel to the left atrial appendage: a danger during balloon septostomy.

    Right atrial tunnel to the left atrial appendage is a very rare anomaly not previously described. Per se, it has no physiological significance but is a source of potential disaster during balloon atrial septostomy. The precise anatomy is demonstrated and ways are proposed to avoid tearing the atrial wall during therapeutic cardiac catheterization.
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keywords = catheterization
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9/52. Successful arterial switch operation for post-Mustard pulmonary venous obstruction and secondary pulmonary hypertension.

    A 16-year-old girl presented with dyspnea 15 years after the Mustard operation for transposition of the great arteries with intact ventricular septum. An echocardiogram revealed secondary pulmonary hypertension due to pulmonary venous obstruction. cardiac catheterization showed the left (pulmonary) ventricular pressure was over the systemic level. We performed a successful one-stage switch conversion. The patient is doing well 1 year after the switch conversion.
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ranking = 1
keywords = catheterization
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10/52. Neoaortic root dilation associated with left coronary artery stenosis following arterial switch procedure.

    We describe a patient who was diagnosed with d-transposition of the great arteries, with intact ventricular septum, who underwent arterial switch procedure on day 5 of life. Over the subsequent years, he developed progressive neoaortic root dilation with a Z score of up to 7.2. At 5 years of age, he presented with myocardial infarction. cardiac catheterization demonstrated a markedly dilated aortic root with kinking and stenosis of the left main coronary artery into the left anterior descending coronary artery. He underwent emergency left internal mammary artery bypass grafting to the left anterior descending coronary artery. Although he required left ventricular assist device (LVAD) support in the early post-operative period, he recovered with a left ventricular ejection fraction of 52% on the most recent follow-up.
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ranking = 1
keywords = catheterization
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