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1/182. 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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2/182. 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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ranking = 1.1428571428571
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3/182. 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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4/182. Arterial switch with internal pulmonary artery banding. A new palliation for TGA and VSD in complex cases.

    In most cases, one stage repair by arterial switch operation (ASO) is the optimal treatment for neonates with transposition of the great arteries (TGA). Nevertheless, a ventricular septal defect (VSD) associated with TGA remains a major risk factor for early death and reoperation after complete repair in neonates with complex anatomy. A new palliative approach for such specific cases is proposed. An internal pulmonary artery banding (IPAB), as that already used to palliate other cardiac malformations, is performed in association with ASO instead of VSD closure. At the end of ASO, a circular polytetrafluorethylene (PTFE) patch with a 4-mm central hole is oversewn into the neo-pulmonary trunk. We adopted this method in a 17-day-old boy with TGA, VSD, hypoplastic tricuspid valve and diminutive right ventricle. After the operation the child thrived and was doing well without medication. Satisfactory growth of the right ventricle and tricuspid valve was observed by echocardiography during the following months. The patient successfully underwent VSD closure and IPAB removal 2 years after the first procedure. ASO with IPAB could be appropriate in all forms of TGA and VSD in which VSD closure appears too challenging in the neonatal period and in patients with uncertain suitability for biventricular repair. We preferred to use IPAB instead of classic PAB in order to reduce the risk of pulmonary valve damage, pulmonary artery distortion, and above all pulmonary artery dilatation and related coronary compression. In the presented case the strategy as well as IPAB worked according to our expectations.
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ranking = 0.28571428571429
keywords = ventricle
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5/182. Post-Mustard procedure pulmonary venous obstruction: An opportunity for anatomic correction with a one-stage arterial switch.

    A 14-year-old boy after a Mustard procedure for transposition of the great arteries developed pulmonary hypertension secondary to baffle obstruction. This occurred over several years without apparent significant symptomatology. Systemic-level pressure prevailed in the left (pulmonary) ventricle and provided an opportunity to perform a successful one-stage arterial switch.
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ranking = 0.14285714285714
keywords = ventricle
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6/182. 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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ranking = 0.14285714285714
keywords = ventricle
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7/182. Rapid two-stage repair of S,L,L, ventricular septal defect, pulmonary atresia, ebstein anomaly of the tricuspid valve, and stenotic pulmonary arteries.

    The repair of a corrected transposition of the great arteries, ventricular septal defect, and pulmonary atresia is presented. An ebstein anomaly of the tricuspid valve, dextrocardia, and severe distortion of the pulmonary arteries complicated the surgical procedure, which was performed in two stages. Reconstruction of the pulmonary arteries and a bidirectional cavopulmonary anastomosis were performed first; Rastelli and hemi-Mustard procedures completed the correction. The rationale and the possible indications of this "one and a half ventricle" repair are discussed.
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ranking = 0.14285714285714
keywords = ventricle
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8/182. Changes of management in a patient with double outlet left ventricle.

    Double outlet left ventricle is an extremely rare anomaly. Until recently, the diagnosis was usually established by angiography or at postmortem. There are only a few reports describing the echocardiographic findings in this lesion, and as far as we know, no report showing the anatomy as well as the velocity and pattern of flow by color Doppler echocardiography. The patient reported here underwent surgery at the age of four years, when an aortic homograft was placed between the right ventricle and the pulmonary trunk. This biventricular repair had to be changed into a Fontan-type procedure, 15 years later since the hypoplastic right ventricle did not grow adequately.
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9/182. Double outlet left ventricle with subpulmonary ventricular septal defect and pulmonary hypertension.

    A two-month old male infant with the rare occurrence of double outlet left ventricle, subpulmonary ventricular septal defect and pulmonary hypertension is presented. The infant was managed temporarily with banding of the pulmonary trunk, with a favorable result, and is scheduled for definitive intraventricular repair.
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ranking = 0.71428571428571
keywords = ventricle
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10/182. Transposition of the great arteries, pulmonary atresia, and multiple ventricular septal defects associated with multiple cardiac rhabdomyomas in a case of tuberous sclerosis.

    Multiple cardiac rhabdomyomas are frequently associated with tuberous sclerosis. However, very few cardiac malformations have been described with tuberous sclerosis. We report an unusual case of tuberous sclerosis with cerebral and renal lesions associated with dextrotransposition of the great arteries, pulmonary atresia, patent ductus arteriosus, multiple ventricular septal defects, and parachute mitral valve deformity. After reviewing other cases of congenital heart diseases associated with tuberous sclerosis, emphasis is made on the potential influence of multiple rhabdomyomas developing very early during fetal life on final cardiac structures.
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ranking = 8.9585544089713E-6
keywords = cerebral
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