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1/7. 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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2/7. Giant pulmonary artery aneurysm with right ventricular outflow tract obstruction.

    aneurysm of the main pulmonary artery is rare. Its natural history is not well understood and there are no clear guidelines regarding its optimal treatment. We present a case of a huge saccular aneurysm of the main pulmonary artery which was associated with infundibular and valvular pulmonary stenosis. It was repaired using a pericardial patch with concomitant pulmonary valvotomy and infundibular resection. Postoperative recovery was uneventful and the patient is doing well. Follow-up echocardiogram revealed good repair.
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3/7. Surgical correction of unusual double-outlet right ventricle.

    This paper presents the case history of an 8-year-old girl who had total situs inversus and double-outlet right ventricle with pulmonary stenosis and severe tricuspid insufficiency in the presence of dextrocardia with ventricular discordance. A successful repair was performed using the Rastelli technique in conjunction with replacement of the tricuspid valve with a Bjork-Shiley prosthesis. The postoperative course was uneventful, and follow-up catheterization revealed a good operative result. However, the patient died suddenly during an emotionally upsetting period about two months after the operation. Postmortem examination revealed only signs of moderately severe cardiac decompensation. Some anatomical and embryological comments are made.
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keywords = operative
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4/7. Late distortion of the original Palmaz stent implanted in postoperative lesions associated with congenital heart disease.

    The objective of this study was to report late distortion of a Palmaz stent. Late distortion of an original Palmaz stent, implanted in an extracardiac lesion, is rare. We completed a 1-year follow-up of 54 patients who had been implanted with 80 Palmaz stents in extracardiac lesions. Distortion of two stents was detected in two patients. For case 1, we implanted a P188 stent for supravalvar pulmonary stenosis complicating an arterial switch operation in a 14-year-old girl. Seven months later, we found compression of the stent. Although we implanted two P308 stents anterior to the distorted stent, distortion of both stents developed after 1 month. Two more P308 stents placed inside each stent were gradually recompressed. A CAT scan showed compression of the stent by a dilated sinus of valsalva. For case 2, we implanted a P308 stent for stenosis of the superior vena cava after Williams operation in an 11-year-old boy. A chest X-ray documented longitudinal compression of the stent 27 months after implantation and a CAT scan showed the ascending aorta was in contact with the stent. A Palmaz stent may be distorted when implanted in a lesion adjacent to a pulsating aorta.
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5/7. heart transplantation in repaired transposition of the great arteries.

    Cardiac transplantation was carried out in a 40-year-old man with the diagnosis of repaired transposition of the great arteries and right-sided aortic arch who had end-stage cardiac failure due to myopathy of the ventricles. Because of several previous surgical repairs and the orientation of the great vessels, the operation presented some technical problems. Therefore, modifications of operative procedures were used, including recipient hypothermia, circulatory arrest, and changes in the donor heart implantation. The removal of the donor heart was modified in such a way that the graft included the aortic arch and both pulmonary arteries. With the extra length of ascending aorta and transverse arch, the innominate, left carotid, and left subclavian vessels were excised as a button, thereby leaving the distal orifice of the aorta in the superior portion of the transverse arch. For the recipient, the operation was performed using hypothermic total circulatory arrest to dissect free the huge pulmonary artery and the short right-sided aortic arch to place the clamp. Implantation of the donor heart was modified accordingly. The technical results were confirmed one and a half months later on a control digital angiogram. Thirty-five days postoperatively the patient was discharged. Six months after operation, the patient is doing better than ever before in his life. Our findings suggest that a complicated conotruncal development does not preclude cardiac transplantation.
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6/7. Accessory valve cusp as a cause of outflow tract obstruction in atrio-ventricular and ventriculo-arterial discordance.

    Congenital corrected transposition of the great arteries in which there is both an atrio-ventricular and a ventriculo-arterial discordance has surgical significance only in consequence of the associated cardiac anomalies. Their surgical correction is subject to multifarious problems. An exceedingly rare cause of a left-ventricular outflow tract obstruction erroneously interpreted preoperatively in an eight-year-old boy is reported. In an l-transposition with inversion of both ventricles, the diagnosis "valvular pulmonary stenosis" was made preoperatively. However, a normally arranged tricuspid pulmonary valve without stenosis was revealed intraoperatively. On the other hand, dystopic valvular tissue in the form of a monocuspid atrioventricular valve which was attached to an accessory papillary muscle with several tiny tendons was found below the pulmonary valve in the morphological left ventricle. The accessory valve cusp, which was the cause of the hemodynamically relevant subvalvular pulmonary stenosis, had no connection with the mitral valve. The abnormal valve cusp was resected without injuring the normal structures. The angiographic follow-up after one year no longer revealed any pressure gradient. This case report points out the possibility of an outflow tract obstruction caused by a complete accesory valve cusp.
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7/7. Surgical repair of pulmonary stenosis with intact ventricular septum in a 68-year-old woman.

    patients with mild pulmonary stenosis after infancy rarely have symptoms or develop increasing obstruction. We experienced a 68-year-old woman with severe pulmonary valvar and infundibular stenosis (peak to peak pressure gradient = 80 mmHg). She had been pointed out heart disease at the age of six. endocarditis at the age of 17 might induce calcification of valve and affect the progression of pulmonary stenosis, and moreover, which might gradually develop severe subvalvar obstruction and poststenotic aneurysm of pulmonary trunk. She refused operative intervention because of mild clinical symptoms (NYHA class II), but we recommended surgical repair due to repeated transient ischemic attacks, which were suspected paradoxical embolism through persistent foramen ovale. She underwent pulmonary valvotomy and infundibular resection and is doing well.
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