Electrocardiographic diagnosis of malformations associated with tricuspid atresia: correlation with morphologic features.
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The clinical, radiographic and echocardiographic features of two cases of pulmonic stenosis in cats are presented. A rarely reported combination of pulmonic stenosis and tricuspid valve dysplasia is described.
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OBJECTIVE: Competence of the tricuspid valve is crucial for survival of children with hypoplastic left heart syndrome. We studied the morphology and topology of the valvar and subvalvar structures, trying to identify abnormalities which could impair valvar function. METHODS: A total of 82 specimens with hypoplastic left heart syndrome were examined pathologically. Measurements of valvar dimensions were taken, significant dysplasia of the valvar leaflets was noted and the muscular and tendinous supporting structures determined. The findings were correlated to the subgroups of hypoplastic left heart syndrome. RESULTS: Of the hearts, 10 (12%) showed a bileaflet right atrioventricular valve, 27 (33%) a moderately and 2 (2%) a severely dysplastic tricuspid valve. The majority of the abnormalities was found in hearts with a patent mitral valve. In 79% of the hearts with mitral atresia, the septal surface was concave instead of convex to the right ventricular lumen and the direct tendinous attachments of the septal leaflet replaced by a multitude of freestanding papillary muscles. The number of direct septal attachments was significantly higher in hearts with a patent mitral valve. CONCLUSIONS: The tricuspid valve in hypoplastic left heart syndrome can differ from the valve seen in normal patients. The subvalvar apparatus is different in hearts with mitral atresia, whereas dysplasia of the leaflets occurs more often together with mitral stenosis. These features should be considered in reconstructive operations as well as during diagnostic procedures.
Clinical, hemodynamic, angiographic, and echocardiographic as well as operative findings of a 19 years old girl are presented who had the unique combination of severe pulmonary valvular and infundibular stenosis with Ebstein's anomaly of the tricuspid valve. Pulmonary stenosis was relieved by infundibular resection and valvotomy. The atrial septal defect was closed with plication of atrialized ventricle and insertion of a porcine tricuspid prosthesis. Recatheterization 2 months postoperatively revealed disappearance of characteristic angiographic findings and slight residual gradient.
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A method for quantitative detection of tricuspid regurgitation is proposed. In cases without tricuspid insufficiency or intracardiac shunt, 1/slope (ts-fv) of the earpiece dye-dilution curve after injection of dye into the femoreal vein (fv curve) was significantly correlated with that (ts-pa) of the curve after injection into the pulmonary artery (PA curve) within each range of right heart blood volume (rbv) calculated as a product of cardiac output and mean transit time difference between FV curve and PA curve. The relationship among the 3 parameters was expressed as a statistically significant regression equation; log (tsfv) equals 0.9858 log (ts-pa) plus 0.2980 log (RBV) minus 0.6418 (p small than 0.005, sd of predicted ts-fv equals plus or minus 0.591). The assumption was made that tricuspid regurgitation caused prolongation ot ts-fv without any changes in ts-pa or RBV, being based on the concepts of the indicator dispersion and of the path-length distribution. The ratio of tricuspid regurgitation to cardiac output was calculated from (observed ts-fv-predicted ts-fv)/predicted ts-fv, in which predicted ts-fv is the value calculated from ts-pa and RBV using the equation shown above. In 2 patients who manifested clinical tricuspid insufficiency, large amount of tricuspid regurgitant fraction was determined by this method. It is suggested that the present method utilizing double injections--single sampling dye-dilution technique is useful for clinical quantitation of tricuspid regurgitation.
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A patient in whom infective endocarditis affecting the pulmonary and tricuspid valves developed after percutaneous balloon dilatation of the pulmonary valve is reported.
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The feasibility and results of percutaneous double balloon valvuloplasty were evaluated in 2 patients with stenosis of porcine bioprostheses in the tricuspid valve position. The procedures were performed with a Trefoil 3 x 10 and a 15 mm balloon. Long inflations (4 and 3 minutes) were well tolerated. A significant immediate increase in the valve area, without significant valvular regurgitation, was achieved in both cases, from 0.65 to 1.15 cm2 in case 1 and from 0.9 to 1.65 cm2 in case 2. Both patients required valve replacement during the follow-up, at 14 and 21 months. There was no restenosis, but echocardiography showed right atrial thrombosis in case 1. Progressive restenosis with peripheral edema and increase of the mean doppler gradient occurred in case 2. The procedure is feasible, safe, and well tolerated. It provides significant immediate hemodynamic improvement, but it should be considered as a palliative technique since a normal valve area can not usually be obtained and a restenosis is likely to occur at midterm follow-up.
Sixty patients with pulmonary atresia and intact ventricular septum (PA:IVS) presenting from 1970 to 1980 are reviewed. Three groups of patient are discussed: those with tripartite right ventricles, those with no trabecular portion to the cavity, and those with neither trabecular nor infundibular portions. The decrease in early mortality for neonates with PA:IVS since 1977 (one death in 15 patients) supports our current management policy of preoperative prostaglandin E1 infusion with transpulmonary valvotomy (for patients with an infundibular cavity) combined with a left modified Blalock-Taussig shunt using a Gore-Tex prosthesis. Tricuspid valve growth, estimated by serial angiograms in 12 patients, was greater if right ventricle-to-pulmonary artery continuity was established. Later definitive repair was attempted in nine patients, with two early deaths; five underwent right ventricular outflow tract reconstruction and four had modified Fontan procedures. Neonates with critical pulmonary stenosis are also discussed. Their neonatal mortality (nine deaths in 20 patients) was similar to that of comparable patients with PA:IVS, but their actuarial survival at 5 years (55%) was superior (36% at 5 years).
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