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1/12. 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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ranking = 1
keywords = valve
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2/12. Primitive ventricle with acquired subpulmonary stenosis.

    The course and natural history of two children aged 1 yr and 5 yr with primitive ventricle with outlet chamber and normally related great arteries are described. They initially presented as ventricular septal defect with increased pulmonary blood flow and subsequently developed 'cyanotic attacks', chronic cyanosis and diminished pulmonary flow by acquiring subpulmonary obstruction of the bulboventricular foramen. Both had successful surgical treatment utilizing their own normal pulmonary valves by connecting the right atrium to the right ventricular outflow, with a Dacron conduit in one, and in the other by detaching the pulmonary artery and valve from the rudimentary outflow chamber and anastomosing it directly to the right atrial appendage. Changing morphology and its effect on function are discussed.
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ranking = 148.45000582223
keywords = pulmonary valve, valve
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3/12. Dacron conduit with a stented porcine xenograft valve in the anatomic correction of transposition of the great arteries and subpulmonary stenosis.

    A stented porcine xenograft valve, sutured into a woven Dacron tubular graft, has been used in the anatomic correction of D-loop, D-transposition of the great arteries, and subpulmonary stenosis. The main advantage of this technique is its simplicity and practicality, as both Dacron tubular grafts and stented porcine xenograft valves are commercially available.
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ranking = 6
keywords = valve
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4/12. 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 = 295.90001164447
keywords = pulmonary valve, valve
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5/12. 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 = 294.90001164447
keywords = pulmonary valve, valve
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6/12. 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 = 294.90001164447
keywords = pulmonary valve, valve
(Clic here for more details about this article)

7/12. 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 = 147.45000582223
keywords = pulmonary valve, valve
(Clic here for more details about this article)

8/12. 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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ranking = 303.90001164447
keywords = pulmonary valve, valve
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9/12. Familial pulmonary valve stenosis, atrial septal defect, and unique electrocardiogram abnormalities.

    The familial association of pulmonary stenosis, atrial septal defect, and unique electrocardiographic abnormalities involving a mother and two children is reported. Familial pulmonary stenosis not occurring as part of a named syndrome or without associated multiple congenital abnormalities is rare. The constellation of pulmonary stenosis, atrial septal defect, and the particular electrocardiogram abnormalities present here is to our knowledge previously unreported. The pattern of inheritance is consistent with an autosomal dominant mode of transmission.
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ranking = 589.80002328893
keywords = pulmonary valve, valve
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10/12. The transmitral approach to left ventricular outflow tract obstruction.

    Left ventricular outflow tract obstruction is a particularly vexing problem in patients with complete transposition (atrioventricular concordance and ventriculoarterial discordance) and intact ventricular septum. Previous techniques, such as resecting the obstructive lesion through the pulmonary valve or bypassing the obstruction, have not given entirely satisfactory results. An alternative approach of resection through the left atrium and the retracted mitral valve has recently been proposed. We have used this new technique to achieve excellent relief of this condition in three patients with different types of obstruction.
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ranking = 148.45000582223
keywords = pulmonary valve, valve
(Clic here for more details about this article)
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