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1/16. Asymmetrically short tendinous cords causing congenital tricuspid regurgitation: improved understanding of tricuspid valvar dysplasia in the era of color flow echocardiography.

    BACKGROUND: Tricuspid regurgitation as a manifestation of an isolated congenital anomaly of the tricuspid valve is rare. Cross-sectional and color Doppler echocardiography allow improved evaluation of tricuspid valvar function. As a result, the heterogeneous category of congenital tricuspid valvar dysplasia may be better understood from a functional point of view. We are reporting a distinct entity in which tricuspid valvar regurgitation results from failure of coaptation due to short tendinous cords tethering the septal leaflet. patients AND RESULTS: Three children with significant primary tricuspid regurgitation were evaluated, treated, and followed. On echocardiographic evaluation, a central regurgitant jet of moderate or severe degree was directed toward the atrial septum through poorly coapting tricuspid valvar leaflets, which did not approximate due to tethering of the septal leaflet by abnormally short cords. In one patient, the tricuspid valve was otherwise normal; in the other two the leaflets and cords were also thickened. Two patients underwent surgery at 9 and 11 years of age. The cords tethering the septal leaflet were augmented by interposing appropriate lengths of expanded polytetrafluoroethylene suture and performing commissural annuloplasty. Both patients are asymptomatic 33 and 42 months postoperatively, with mild residual tricuspid regurgitation that has not changed since surgery. The other patient, an 8 month-old infant, has not yet undergone surgery. CONCLUSIONS: Asymmetric tendinous cords of the tricuspid valve causing tethering of the septal leaflet is a distinct cause of tricuspid regurgitation that can be recognized with echocardiography. Although rare, the importance of recognizing this lesion lies in its being readily amenable to surgical repair.
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ranking = 1
keywords = dysplasia
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2/16. prenatal diagnosis of severe tricuspid insufficiency in Ebstein's anomaly with pulmonary atresia and intact ventricular septum: a case report.

    pulmonary atresia with intact ventricular septum is an uncommon congenital cardiac anomaly which very often present varying degrees of downward displacement and dysplasia of the tricuspid valve. A 23-year-old woman was referred at 23 weeks' gestation because of fetal cardiomegaly detected by routine ultrasonography. Ebstein's anomaly with pulmonary atresia was diagnosed by fetal echocardiography. On pulsed Doppler examination, a severe tricuspid regurgitation was detected. pulmonary atresia was suspected, as forward flow was not detected into pulmonary artery. The fetus was terminated at 25 weeks. At the postmorterm examination Ebstein's anomaly with pulmonary atresia and intact ventricular septum were confirmed. The finding of right atrial enlargement on an early sonogram should prompt the search for tricuspid valve abnormalities with tricuspid regurgitation and the presence or development of pulmonary atresia or stenosis. With the information provided, early detection of tricuspid valve disease should be possible so that the option of therapeutic abortion can be offered.
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ranking = 0.2
keywords = dysplasia
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3/16. Persistent atrial standstill due to atrial inexcitability. An electrophysiological and histological study.

    Electrophysiological and histopathological examinations were carried out in 2 patients with persistent atrial standstill. Intracardiac studies revealed that atrial standstill was due to atrial inexcitability. It was demonstrated in both patients that prolongation of the H-V interval and the duration of H deflection was associated with complete right bundle branch block and that the escape rhythm was an A-V junctional origin. Histologically, marked fibrosis of the right atrium and fibrous strands in the right ventricle were demonstrated in a 69-year-old man with idiopathic cardiomyopathy. In another female patient with peripartum heart disease aged 40 years, there was moderate degeneration of the myocardium in the right ventricle. The results of electron microscopic observations are also presented. The importance of the examination of His bundle electrography in persistent atrial standstill and the diagnostic criteria and treatment of this lesion are discussed.
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ranking = 0.48330400690627
keywords = cardiomyopathy
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4/16. Functional pulmonary atresia in a newborn with normal intracardiac anatomy.

    Functional pulmonary atresia is a relatively rare clinical condition usually associated with Ebstein's malformation, tricuspid valve dysplasia, Uhl's anomaly, or transient myocardial ischemia with severe tricuspid regurgitation. The occurrence of functional pulmonary atresia associated with transient tricuspid regurgitation in a newborn with an anatomically normal heart is even more uncommon. We describe a case in which color Doppler flow mapping played an essential role in diagnosis and follow-up of this clinical condition in a newborn who had normal intracardiac anatomy.
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ranking = 0.2
keywords = dysplasia
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5/16. Surgical treatment of tricuspid valve dysplasia in the neonatal period.

    tricuspid valve dysplasia, other than Ebstein's anomaly, is a very rare congenital heart defect. During the prenatal and/or the neonatal periods the clinical picture is very critical. We here report on a newborn infant with severe tricuspid valve dysplasia and 4/4 tricuspid regurgitation, giant right atriomegaly, functional pulmonary atresia with ductal-dependent pulmonary blood flow. The child was successfully submitted to implantation of a 15 mm pulmonary stentless heterograft valve using the top-hat technique.
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ranking = 1.2
keywords = dysplasia
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6/16. Application of papillary muscle sling concept in an infant as a biological bridge to transplantation.

    We successfully applied the papillary muscle sling concept in mitral valve reconstruction of an infant with dilated cardiomyopathy. This new method of banding the two papillary muscles together can shorten the distance of the base between the two papillary muscles, reduce the severity of mitral regurgitation, and remodel the dilated left ventricle without any mitral stenosis. The infant survived after this bridge-to-transplantation procedure and was successfully transplanted.
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ranking = 0.48330400690627
keywords = cardiomyopathy
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7/16. Improved delineation of morphological features of arrhythmogenic right ventricular cardiomyopathy with the use of contrast-enhanced echocardiography.

    A case of an already diagnosed arrhythmogenic right ventricular cardiomyopathy in which the use of contrast improved the echocardiographic characterization of morphologic abnormalities of the right ventricle is reported.
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ranking = 7684.3219079934
keywords = arrhythmogenic right ventricular cardiomyopathy, right ventricular cardiomyopathy, ventricular cardiomyopathy, arrhythmogenic, arrhythmogenic right, cardiomyopathy
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8/16. Cardiac sarcoidosis with primary involvement of the tricuspid valve.

    Sarcoid granulomas usually involve the myocardium with rare focal extensions into the pericardium and endocardium with resultant conduction defects, ventricular arrhythmias, and ventricular systolic and diastolic dysfunction. Primary involvement of valvular leaflets resulting in valvular regurgitation or stenosis is not known. We present a case of a wastewater consultant who developed tricuspid regurgitation and symptomatic atrioventricular block secondary to infiltration of tricuspid leaflets and conduction system from sarcoid granulomas. The patient later developed severe dilated cardiomyopathy as a result of extensive cardiac sarcoidosis necessitating cardiac transplantation. Valvular regurgitation should be included as one of the presenting manifestations of cardiac sarcoidosis.
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ranking = 0.48330400690627
keywords = cardiomyopathy
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9/16. tricuspid valve replacement for gross tricuspid insufficiency due to dysplastic valve--a case report.

    A case report is presented of a child in whom there was gross tricuspid insufficiency due to marked dysplasia of the tricuspid valve. This patient received prosthetic valve replacement in tricuspid position with excellent post-operative improvement. To the best of our knowledge, this is the first case report of a tricuspid valve replacement for a severely dysplastic tricuspid valve.
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ranking = 0.2
keywords = dysplasia
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10/16. Ventricularization of right atrial wave form in amyloid restrictive cardiomyopathy.

    Two patients with biopsy-proven amyloid restrictive cardiomyopathy were presented. Both cases showed ventricularization of an elevated right atrial pressure wave form in absence of tricuspid regurgitation. Possible explanations for this finding as well as its clinical implications are discussed. This observation indicates that ventricularization of right atrial pressure wave form could be a useful hemodynamic sign in amyloid restrictive cardiomyopathy in absence of tricuspid regurgitation. Furthermore, such a finding does not seem to be specific for tricuspid regurgitation.
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ranking = 2.8998240414376
keywords = cardiomyopathy
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