[Differential diagnosis of relative stenosis of the tricuspid valve in combined rheumatic heart diseases].
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Two perinatal autopsy cases are reported where a rare congenital anomaly, namely pulmonary atresia with intact ventricular septum in association with tricuspid stenosis and a hypoplastic right ventricle was encountered.
UNLABELLED: The clinical utility of visualization of blood flow patterns across the tricuspid valve (TV) and pulmonary valve (PV) by M-mode contrast echocardiography was studied in 38 patients with documented valvular or congenital heart disease and in 15 controls. Diastolic turbulence was observed in the TV outflow side in three patients with tricuspid stenosis. Turbulent flow was also detected during systole in patients with septal defects (atrial septal defect in four patients and ventricular septal defect in one patient) in the right ventricular outflow tract and in three atrial septal defect patients also in the inflow tract, presumably because of increased volume of flow. Tricuspid regurgitation (11 patients) was characterized by retrograde laminar jet throughout systole, while in pulmonary regurgitation (six patients) retrograde flow was initially laminar although later on it might become turbulent. In pulmonary hypertension (29 patients) antegrade flow occurred approximately to mid-systole and then was interrupted by retrograde laminar flow which contributed to the mid-systolic closure of the pulmonary valve and pulmonary regurgitation in some cases. CONCLUSIONS: contrast echocardiography is a useful technique to analyse right heart blood flow patterns and may aid the diagnosis of various right heart abnormalities.
Tricuspid valve, superior vena cava (SVC), and hepatic vein Doppler patterns may be abnormal in right heart anomalies and have been used to predict high central venous pressure (CVP) in adults. The purpose of this study was to evaluate the relationship of these systemic venous flow indices to CVP in children. Children undergoing cardiac catheterization were studied prospectively using simultaneous recordings of mean CVP with pulsed-Doppler tracings of SVC, hepatic vein, and tricuspid valve flow. Systemic venous Doppler measurements included peak velocities and velocity time integrals for ventricular systole (S), ventricular diastole (D), and ventricular systole (B), and atrial systole (A). Tricuspid inflow Doppler E and A waves were recorded also. Patients with significant tricuspid stenosis or regurgitation, systemic venous obstruction, and nonsinus rhythm were excluded. The 42 patients ranged in age from 0.2 to 21.0 years and in weight from 3.0 to 68.0 kg. Mean CVPs ranged from 1 to 17 mmHg. Catheterization indications included hemodynamic evaluation (25 patients), transplant biopsy, (11 patients), and interventional procedures (6 patients). No SVC or tricuspid valve Doppler measurement correlated with CVP. Hepatic vein peak D, peak B, and peak A significantly correlated with CVP (r = 0.34 - 0.55; P < 0.05, linear regression). For all correlations, the r values were low with significant overlap among patients. Thus, in children, only hepatic vein peak velocities correlate with CVP. Because of the low r values and significant overlap among patients, the currently used Doppler indices have a low sensitivity for predicting CVP in this age group.
Right heart catheterization was carried out on 14 patients with pulmonic regurgitation using intracardiac phonocardiography. All the patients showed pulmonic regurgitant murmur in the right ventricular outflow tract. In addition, seven out of the 14 patients showed mid-diastolic and presystolic murmurs maximally in the outflow tract of the right ventricle. Furthermore, inspiration increased the loudness of these diastolic murmurs in four patients. These findings were compatible with those of right-sided Austin Flint murmur due to functional tricuspid stenosis in pulmonic incompetence. Ten out of the 14 patients had pulmonary hypertension and all the subjects with a rightsided Austin Flint murmur showed elevated pulmonary arterial pressure. Thus, pulmonic regurgitation with pulmonary hypertension is thought to be closely related to the right-sided Austin Flint murmur and the turbulence resulting from antegrade flow across a closing tricuspid valve may be responsible for the genesis of the murmur.
Case history and necropsy findings of a 5-month-old infant with a unique heart defect with features of truncus arteriosus communis and aortopulmonary defect in combination with severe tricuspid stenosis are presented. There is a wide spectrum of remarkable heart defects between truncus arteriosus communis and aortopulmonary septal defect.
Eight patients with Ebstein's anomaly, corrected by the Carpentier technique between 1984 and 1987, were evaluated by echocardiography and by conventional Doppler and color-flow Doppler mapping. Because this technique aims at restoring not only tricuspid valve function but also right ventricular size and geometry, the following parameters were studied: 1) right ventricular size, by measuring the apex-annulus distance compared with that of a group of 30 normal volunteers; and 2) tricuspid valve motion and function. The apex-annulus distance ranged from 5.8 to 8 cm (mean, 6.4 cm) in the Ebstein group and 3.8 to 5.1 cm (mean, 4.3 cm) in the control group. There was no tricuspid stenosis. Right ventricular filling was normal as documented by color-flow mapping. Tricuspid regurgitation was trivial in three patients, mild in four, and severe in only one, in whom a significantly restricted motion of the leaflets was demonstrated.
In patients with intractable ascites, careful consideration should be given to the patient's past history with emphasis on cardiac, liver and renal disorders. Ascitic fluid cytology indicating the presence of malignant cells should be unequivocal; cytology should be repeated before embarking on potentially toxic anti-cancer treatment in ambiguous cases. Elevated serum CA-125 levels should not be relied upon to make the diagnosis of ovarian/peritoneal carcinoma. We report a case of tricuspid stenosis with severe ascites in an elderly woman who was initially mis-diagnosed and treated as peritoneal/ovarian carcinoma.
A case is discussed of a 65-year-old woman who presented with tricuspid stenosis. She was evaluated with multiple imaging techniques including gated radionuclide angiography, cardiac sector scanning, and abdominal CT imaging. A review of the literature of the entity known as intravenous leiomyomatosis is presented.
Surgical treatment of a right atrial myxoma with a right-to-left shunt and coronary artery disease was successfully performed in a 61-year-old man. The interatrial shunt occurred through a patent foramen ovale and was the result of a high central venous pressure due to tricuspid stenosis and regurgitation. Instead of right heart catheterization and cardioangiography, we chose an intraoperative blood-gas study to diagnose the intracardiac shunt. Preoperative polycythemia and hypoxemia were improved after removal of the tumor and closure of the patent foramen ovale.
Surgical pathologic features of the tricuspid valve were reviewed in 363 patients who had undergone tricuspid valve replacement at our institution during the period 1963 through 1987. Valves were purely regurgitant in 74%, stenotic and regurgitant in 23%, and purely stenotic in 2%; two valves were neither stenotic nor regurgitant. Among 269 purely insufficient tricuspid valves, the four most common causes were postinflammatory disease (41%), congenital disorder (32%), pulmonary venous hypertension (21%), and infective endocarditis (4%). Of 92 cases of tricuspid stenosis, with or without regurgitation, postinflammatory disease was observed in 92%. Female patients accounted for 66% of the 363 cases, including 84% of those with postinflammatory disease and 64% of those with pulmonary venous hypertension. In contrast, male patients accounted for 73% of cases with endocarditis and 61% with congenital heart disease. Although postinflammatory disease accounted for 53% of the 363 cases, its relative frequency diminished from 79% during 1963 through 1967 to only 24% during 1983 through 1987. This trend may reflect the decreasing incidence of acute rheumatic fever reported in Western countries. During the same time interval, the relative frequency of congenital heart disease as a cause of tricuspid dysfunction increased from 7% to 53%, and it is currently the most common cause in our surgical population. This finding apparently reflects changes in patient referral practices and the development of new operative procedures.
An eight-year-old girl with partial atrioventricular canal defect and hypoplastic right ventricle was treated successfully by a palliative open-heart surgery. The preoperative right ventricular pressure was 58/7 mmHg (RVP/LVP = 0.67) and morphology of the right ventricle showed severe tricuspid stenosis and small outflow portion. The preoperative RVEDVI was 31 (41% of normal and tricuspid annulus was 17 mm (47% of normal). The right ventricular outflow was reconstructed with insertion of MVOP and the ASD was partially left open (the amplitude of the interatrial communication was 7 mm). Angiocardiogram two and half years after the operation demonstrated significant right ventricular growth with no right to left shunt through interatrial communication. The RVEDVI was 46 (56% of normal) and tricuspid annulus was 36 mm (90% of normal). This technique can be a procedure of choice in patient with right ventricular hypoplasia, who is not candidate for simple right ventricular reconstruction nor Fontan procedure, as the growth of the right ventricule is expected in the future.
Despite recent renewed interest in the detection of tricuspid valve regurgitation by echocardiographic and Doppler techniques, little morphologic information is available on dysfunctioning tricuspid valves. This report describes 45 necropsy patients with clinical and morphologic evidence of pure (no element of stenosis) tricuspid regurgitation and provides morphometric observations (anular circumference, leaflet area) of the tricuspid valve useful in determining the etiology of pure tricuspid regurgitation. Of 45 patients, 24 (53%) had pure tricuspid regurgitation resulting from an anatomically abnormal valve (prolapse in 7, papillary muscle dysfunction in 6, rheumatic disease in 5, Ebstein's anomaly in 3, infective endocarditis in 2, carcinoid tumor in 1), and 21 (47%) had an anatomically normal valve with systolic pulmonary artery hypertension (cor pulmonale in 12, mitral stenosis in 9). Anular circumference was dilated (greater than 12 cm) in patients with various causes of pulmonary hypertension, floppy valve and Ebstein's tricuspid anomaly. Leaflet area was increased in floppy valve and Ebstein's anomaly. Of the 45 patients, 24 had pulmonary systolic artery pressure measurements available for correlation with tricuspid valve morphology. Pulmonary artery pressures accurately predicted morphologically normal from abnormal valves in 16 patients (89%). Morphologic overlap occurred in six patients with pulmonary pressures of 41 to 54 mm Hg. Of these six, the additional knowledge of normal or dilated anular circumference correctly separated valves with normal and abnormal leaflets.
The authors describe a 37-year-old African American female patient with congenital tricuspid stenosis, a secundum atrial septal defect, a ventricular septal defect, and subpulmonic stenosis whose only surgical intervention was a Glenn shunt procedure at the age of 11. She had been in reasonably good health for 26 years, but developed congestive heart failure. Her deteriorating clinical course was difficult to manage with only medical treatment, and cardiopulmonary transplantation is now being considered for the management of her complicated clinical condition.
A male newborn expired 5 days after birth, because of multiple congenital cardiac anomalies. The postmortem examination demonstrated tricuspid stenosis, pulmonary atresia, hypoplastic right ventricle, persistent venous valve, and so on. In addition, he had coronary arterio-ventricular fistulous connection, associated with absence of the proximal connection of the coronary artery to the aorta or the pulmonary artery. As the result, the coronary blood was totally supplied via the right ventricular-coronary channels. Histologic examination revealed that these arterio-luminal channels were characterized by sinusoidal mural structure.
A 56-year-old man was admitted complaining of throat discomfort and dyspnea. He was given a diagnosis of diffuse large B-cell lymphoma on the basis of findings from tumor biopsy specimens of his left pharynx. MRI tomograms and ultrasonic cardiograms revealed a right atrial tumor causing tricuspid stenosis. Although chemotherapy rendered the cardiac tumor indistinct on MRI and UCG images, gallium-67 scintigraphy still demonstrated abnormal cardiac uptake. After 6 courses of CHOP therapy, sick sinus syndrome with syncope suddenly developed in the patient. A cardiac pacemaker was immediately implanted, and radiotherapy was started. The patient's sinus rhythm returned to normal shortly afterward, and the gallium-67 uptake eventually disappeared. In this case gallium-67 scintigraphy was the only diagnostic procedure capable of detecting evidence of residual disease.
In two cases of tricuspid atresia and one of severe tricuspid stenosis the Glenn and Fontan operation was performed. These patients were evaluated post-operatively by studying the distribution of the ventilation with Xe-133 on a gamma camera; the pulmonary circulation was evaluated by injection through antecubital and pedal veins of TC99mMAA and angiography was performed by "in vivo" tagging of red blood cells with TC99m04 preceded by the administration of stannous pyrophosphate. The scintigraphic findings in two cases consisted of decreased ventilation of the left lung and grossly normal perfusion of both lungs. In one case there was abundant collateral because of partial occlusion of the anastomosis between the superior vena cava and right pulmonary artery. The patients also had angiocardiography with contrast medium; the correlation between the scintigraphic and angiocardiographic findings was excellent.
CHF1/Hey2 null mice generated in different laboratories have discrepant cardiovascular phenotypes. To determine the effect of genetic background on phenotype, we backcrossed our knockout strain more than eight generations to the inbred strains BALB/c and C57BL/6. Knockout mice on these backgrounds showed disparate phenotypes. Mice on both backgrounds demonstrated ventricular septal defects (VSDs), tricuspid stenosis and mitral valve thickening, but at varying frequencies, suggesting a general defect in endocardial cushion remodeling. Additional defects seen exclusively on the C57BL/6 background included biventricular wall thinning and left ventricular enlargement, implying a more severe myocardial defect than previously observed. In addition, aortas and pulmonary arteries from these null mice had thinner walls. Intercrossing of the CHF1/Hey2 null mice on a C57BL/6 background with a C57BL/6 MLC2v-CHF1/Hey2 transgenic line overexpressing CHF1/Hey2 in the atrial and ventricular myocardium also rescued the VSD and myocardial phenotypes, but did not affect vascular wall thickness. Our results indicate that CHF1/Hey2 provides an important myocardial signal to the endocardial cushion for proper septation and valve formation and also plays an important role in maturation of the myocardium and vasculature.