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11/28. A case report of Noonan's syndrome with pulmonary valvar stenosis and coronary aneurysms.

    BACKGROUND: noonan syndrome is a rare disease, mainly presenting with malformations such as dysplasia and stenosis of the pulmonary valve, atrial septal defect and a typical pattern of hypertrophic cardiomyopathy. We report a case of noonan syndrome with giant coronary aneurysms. CASE REPORT: A young woman with the phenotypic characteristics of Noonan's syndrome presented with severe pulmonary stenosis and giant coronary aneurysms. Cross sectional echocardiography showed valvar and subvalvar pulmonary stenosis. The valve itself was thickened and dysplastic, a characteristic that is typical of Noonan's syndrome. In addition to the usual abnormalities of the pulmonary valve and the ventricular myocardium, the patient showed a wide spectrum of previously unreported coronary aneurysms. CONCLUSIONS: These additional findings support the hypothesis that a vasculitic process has been superimposed on the connective tissue defect associated with Noonan's syndrome. Furthermore, since the pathogenesis of the condition remains unclear, this case stresses the need to look carefully for abnormalities co-expressed in Noonan's syndrome.
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ranking = 1
keywords = stenosis
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12/28. Surgical treatment of an unusual atrial septal defect: the vestibular defect.

    A 14-year-old female patient underwent surgical treatment of multiple atrial septal defects associated with unroofed coronary sinus and pulmonary valvar stenosis. One of the defects was that of the superior oval fossa and the other a large ellipsoidal defect positioned inferior to the inferior rim of the oval fossa. The patient underwent primary closure of the defects with a favorable result. To the best of our knowledge, this is the first surgical experience of an unusual atrial septal defect or the vestibular defect.
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ranking = 0.14285714285714
keywords = stenosis
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13/28. 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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ranking = 0.28571428571429
keywords = stenosis
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14/28. Surgical management of infants with isolated supravalvular pulmonary stenosis: case reports.

    Pulmonary stenosis (PS) can be seen from the right ventricular outflow tract to the peripheral pulmonary arteries. Most frequently, the obstruction occurs at the level of the pulmonary valve; however, it occurs less frequently at the infindibular level within the trabecular component of the right ventricle or within the pulmonary arterial pathways. Lesions at any of these levels can occur as part of more congenital cardiac malformations such as tetralogy of fallot, complete transposition of great arteries, or atrial septal defect. Isolated supravalvular pulmonary stenosis (iSPS) is less common than other types of PS. In this study, we present our experience with 4 patients who underwent cardiopulmonary bypass operation for iSPS. In one patient, the circular stenotic area was noted on the touch point of the pulmonary valve. Right ventricular pressures ranged from 70 to 90 mmHg, and the pulmonary artery mean pressures ranged from 14 to 17 mmHg. In all patients, the left ventricular and aortic systolic, diastolic, and mean pressures were moderately increased. pulmonary artery stenosis was treated successfully using a pericardial or Dacron patch on cardiopulmonary bypass. Various techniques such as balloon dilation have been proposed to deal with this problem, but these may often be unsuccessful because of the elasticity and recoil of the pulmonary artery constrictive ring. Even though endovascular stenting and/or balloon angioplasty have been recently proposed as an initial treatment strategy, they may be associated with some severe complications including pulmonary artery thrombosis or stent migration. Our study, even though it consists of a limited number of cases, suggests that open heart surgery using an oval-shaped patch may be a used as the other main choice for the treatment of iSPS.
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ranking = 1
keywords = stenosis
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15/28. Suspension string: a new method of aortic valvuloplasty for aortic insufficiency and ventricular septal defect.

    In a 4-year-old boy with ventricular septal defect, severe aortic insufficiency, and mild infundibular stenosis, a new method was used to reconstruct the prolapsed aortic cusp. Two ends of a pledged stitch were passed through the aorta at each side of the right, noncoronary commissure and then through another pledget, and were then tied repeatedly in a row. The length of the row of knots was equal to that of the free edge of left coronary or noncoronary leaflet. The remainder of the stitch was passed through a pledget and then the aortic wall at each side of the left and right coronary commissure to the extraaortic wall pledget and were tied. A suspension string was formed by the row of knots and supported by a Teflon (Dupont Teflon, Wilmington, DE) felt pledget sandwich at each of two commissures. The free margin of the prolapsed cusp was attached to the suspension string by a continuous suture. The concomitant anomalies were corrected. The result was satisfactory.
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ranking = 727.29093312109
keywords = infundibular stenosis, stenosis
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16/28. Percutaneous transcatheter myectomy of subvalvar pulmonary stenosis in tetralogy of Fallot: a new palliative technique with an atherectomy catheter.

    A male infant with tetralogy of fallot was given a modified left Blalock-Taussig shunt at 11 days of age because of duct dependence. Twenty months later, because of increasing cyanosis and polycythaemia, he was given palliative treatment with balloon dilatation of the right ventricular outflow tract. Seven months later a right ventricular angiogram showed further progression of the infundibular stenosis with poor anterograde opacification of the pulmonary arteries. He underwent percutaneous transcatheter myectomy of the infundibulum with an atherectomy catheter. One month later he was well and less cyanosed.
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ranking = 727.86236169252
keywords = infundibular stenosis, stenosis
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17/28. Perforation of the heart in a newborn with critical valvar pulmonary stenosis during balloon valvoplasty.

    We describe the perforation of the right ventricular outflow tract with a 0.021 inch wire in a newborn infant with critical pulmonary valvar stenosis in an attempt to balloon dilatation of the pulmonary valve. The complication was diagnosed by contrast injection into the pericardium. The infant was followed with sector scan echocardiography and recovered completely from that injury. The dilatation was successfully repeated a few days later with the use of a soft tipped wire to cross the pulmonary valve.
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ranking = 0.71428571428571
keywords = stenosis
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18/28. Use of propranolol for severe dynamic infundibular obstruction prior to balloon pulmonary valvuloplasty (a brief communication).

    A case of severe pulmonary valvar stenosis and infundibular obstruction has been reported. Infundibular obstruction was so severe that no catheter could be advanced into the pulmonary artery. propranolol, 0.5 mg given intravenously, reduced the obstruction and allowed the balloon dilatation of the pulmonary valve to be carried out without complication. Subsequently oral propranolol helped to remove the infundibular obstruction. We strongly recommend the use of propranolol when infundibular obstruction is present prior or after the balloon pulmonary valvuloplasty.
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ranking = 0.14285714285714
keywords = stenosis
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19/28. Esmolol (Brevibloc) to assess dynamic subpulmonary stenosis in a child after repair of complete transposition: a case report.

    Esmolol (Brevibloc), a new, ultra-short acting, cardioselective beta-adrenergic blocking agent with half-life of 9.2 min following i.v. administration was given to a 4-year-old child with known dynamic and fixed sub-pulmonary stenosis post Senning repair for complete transposition of the great vessels. The left ventricular systolic pressure increased from 48 to 100 mmHg, heart rate showed an increase from 65 to 140 bpm, the right femoral arterial pressures decreased from 115/58 to 77/35 mmHg following an infusion of Isoprel. Infusion of esmolol partially relieved the dynamic sub-pulmonary stenosis. There were no adverse effects and esmolol was tolerated well by the child. Esmolol might thus play a role in the pediatric catheterization laboratory during investigational procedures, electrophysiological studies and in the control of rapid supraventricular tachycardia, especially in adolescents with WPW pre-excitation. Esmolol would also be beneficial in emergency treatment of epinephrine or isoproterenol overdosage.
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ranking = 0.85714285714286
keywords = stenosis
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20/28. Surgical relief of subpulmonary stenosis after previous Senning (or Mustard) operation for complete transposition.

    A technique to overcome acquired left ventricular outflow obstruction after previous Senning (Mustard) atrial redirection operations is described.
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ranking = 0.57142857142857
keywords = stenosis
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