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At least 775 records · Page 43Linked to original sources
PubMed1980

Fontan procedure for tricuspid atresia.

A Fontan procedure has been performed on 29 patients for tricuspid valvular atresia. The age range was 8 months to 33 years (median 10 years), the pulmonary vascular resistance ranged from 1.8-6.1 units . m2 (mean 3.3 units . m2), and the mean pulmonary arterial pressure ranged from 13-45 mm Hg (mean 21 mm Hg). Twenty-nine previous operations had been performed in 23 patients. Fourteen other associated cardiopulmonary anomalies were present in 12 patients. There were four hospital deaths (13.8%), all in patients with complicating features. Among the last 22 consecutive patients who have undergone operation, one died (4.5%). Complete atrioventricular block necessitated pacemaker implantation in one patient. No late deaths occurred. Of the 19 patients followed 3 months or more from the time of operation, eight have no restriction of exercise capacity, nine have only mild restriction, and two have a poor result. The Fontan approach to tricuspid atresia has several theoretical advantages over previously used shunts or pulmonary artery banding, the operative mortality in patients who have suitable anatomy and hemodynamics is low, and the results have been good.

Adolescent↗
PubMed1994

Cardiopathy and ocular abnormalities in Noonan syndrome.

We describe three children with Noonan syndrome with cardiopathy. One female child had cardiopathy and ocular abnormalities. The other two male children had congenital heart disease of which one had uncommon association of tricuspid valve dysplasia with regurgitation associated with endocardial cushion defect. Karyotypes of the female and one of the male children were normal. The growth hormone and thyroid hormone studies in the first and second male children were normal. All the three children were managed conservatively and followed-up.

Cardiomegaly↗
PubMed1982

Detection of tricuspid regurgitation by contrast echocardiography.

Contrast echocardiography with simultaneous M-mode echocardiography was performed in 77 cases of tricuspid regurgitation (TR) and 168 cases having no TR (non-TR). The echocardiographical characteristics of TR were as follows: 1) By the subxiphoid approach, a contrast appeared in the inferior vena cava during systole. In some cases of non-TR, a similar appearance was noted but limited to the atriosystolic or late systolic-to-early diastolic phases. Such confusing cases should be excluded by the simultaneous observation of the M-mode contrast echogram. 2) By apical four-chamber view, a negative contrast echo above the tricuspid valve and subsequent back and forth movements of the contrast across the valve were useful. This movement, however, may be misleading on two-dimensional echo and the M-mode echocardiogram was also necessary to obtain the exact timing and the direction of regurgitant flow. 3) Presence of the dilated inferior vena cava. The sensitivity of this method was extremely high, and the specificity was also excellent for TR.

Adult↗
PubMed1997

Replacement of right ventricle to pulmonary artery and right atrium to right ventricle homograft after a modified Rastelli operation.

The patient, a 17 year old boy, had undergone a Rastelli operation for transposition of the great arteries plus ventricular septal defect and left ventricular outflow tract obstruction at the age of 7 years. Associated tricuspid valve stenosis was treated with insertion of a second homograft shortly after the Rastelli operation. The patient remained symptom-free for 10 years, when he suffered femoral artery embolus. Investigation showed severe obstruction of both conduits and biventricular dysfunction with thrombus in the left ventricle. After embolectomy and stabilisation of his general condition and prolonged treatment with heparin, he underwent uneventful replacement of the two conduits. He remains well, 8 months after the operation.

Adolescent↗
PubMed1992

[Surgical management of tricuspid regurgitation in patients undergoing mitral valve surgery--analysis of the amount of tricuspid valve annular dilatation].

As a means to determine whether correction for tricuspid regurgitation (TR) in mitral valve surgery is necessary, pulsed Doppler echocardiography was used to study 61 patients (age 49.5 +/- 9.5 years) who underwent mitral valve surgery. Early postoperative tricuspid regurgitation (average 9 +/- 3 postoperative days) was evaluated by a comparison with tricuspid valve annular dilatation and systolic annular shortening in preoperative right ventriculography. Kishimoto's method was used to measure the angiographic maximal early systolic (TVD) and minimal end-systolic diameters where as the shortening of the tricuspid annulus (STA) was expressed as a percent reduction in the maximal diameter by Ubago's methods. Patients were categorized into two groups, i.e., a group having had tricuspid annuloplasty (TAP group n = 23), and a NON-TAP group (n = 38). Preoperative right ventricular volume and hemodynamic indicator were studied with respect to both the TVD and the STA. Results are as follows: 1) The TVD significantly correlated with the end-diastolic right ventricular volume index (EDVI), regurgitant fraction of the tricuspid valve (RF), end-systolic right ventricular volume index (ESVI), pulmonary vascular resistance (PVR), mean pulmonary artery pressure (PAm), mean right atrial pressure (RAm), and right ventricular end-diastolic pressure (RVEDP) (p less than 0.01). 2) The STA was significantly correlated with EDVI, RF, ESVI, RAm and RVEDP (p less than 0.01). In the NON-TAP group, the TVD was significantly larger in patients with residual TR (average 32.5 mm/m2) than in patients having postoperative disappearance of TR (average 25.7 mm/m2) (p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1977

Congenital papillary tumor of the tricuspid valve. An unusual cause of right ventricular outflow obstruction in a neonate with trisomy E.

A trisomy E neonate had congenital cardiac anomalies that included a ventricular septal defect, a bicuspid aortic valve, and a congenital papillary tumor of the tricuspid valve. The large papillary tumor was responsible for severe intermittent pulmonary outflow obstruction. The cause and pathogenesis of these rare papillary tumors are unknown, but they are probably related to the more sessile varieties of congenital valvular dysplasia. The life-threatening obstruction to blood flow caused by the congenital valvular tumor probably can be alleviated by simple surgical resection. This type of tumor should be considered in the differential diagnosis of atypical forms of outflow tract obstruction.

Abnormalities, Multiple↗
PubMed1996

Combined aortic and mitral valve replacement in an adult with mucopolysaccharidosis (Maroteaux-Lamy syndrome).

A 41-year-old man with aortic stenosis, mitral stenosis, and tricuspid regurgitation due to Maroteaux-Lamy syndrome underwent aortic and mitral valve replacement with tricuspid annuloplasty. The annular diameter of the aortic and mitral valves was extremely small. The valve prostheses were 19 mm in diameter in the aortic position and 25 mm in the mitral position. Histologically, the valves showed thickening and hyalinization of the collagen fibers, and the presence of foamy cells that contained a large quantity of pure acid mucopolysaccharide. On an echocardiographic examination performed 2 years after the surgery, the peak systolic gradient of the trans-aortic valve was 18 mmHg at rest, and 26 mmHg during exercise. Careful observation of the residual pressure gradient will be needed. The patient's present New York Heart Association (NYHA) status is grade I and he has returned to work.

Adult↗
PubMed1983

Modified Fontan procedure in patients with previous ascending aorta-pulmonary artery anastomosis.

The Fontan procedure has proved useful in the surgical treatment of patients with tricuspid atresia, univentricular heart, and other complicated cardiac anomalies. Previous palliation of these patients with ascending aorta-right pulmonary artery anastomosis (Waterston shunt) may impose severe restrictions in the operability of these patients because of severe distortion, narrowing, and scarring of the proximal right pulmonary artery. A modification of the Fontan operation is described in which repair is facilitated by transecting the right pulmonary artery at the site of anastomosis, trimming away the distorted narrowed segment, and anastomosing the resulting cuff to the lateral aspect of the superior vena cava. The right atrial appendage is anastomosed to the rudimentary right ventricle or the main pulmonary artery directly or via a tubular Dacron graft. The technique has been applied successfully in four consecutive cases.

Adolescent↗
PubMed1975

Aortic sinus of Valsalva aneurysms simulating primary right-sided valvular heart disease.

While acute regurgitation is the most common valvular lesion associated with aortic sinus of Valsalva aneurysms, this report describes the pathological findings in five patients in whom primary right-sided valve dysfunction was simulated by sinus of Valsalva aneurysms. In two patients aneurysms of the noncoronary sinus projected into the right atrium at the level of the tricuspid valve and caused tricuspid incomptence. In three patients aneurysms of the right sinus of Valsalva projected into the right ventricle immediately below the pulmonic valve and caused pulmonary outflow tract obstruction. In only one of these patients was the aneurysm perforated at necropsy. Although valvular dysfunction was evident clinically, in none was there a clinical suspicion of a sinus of Valsalva aneurysm. In addition to valvular dysfunction, two patients had conduction disturbances and two, right coronary arterial occlusions found to be the result of aneurysms. The risks of valve dysfunction, arrythmias, aneurysm rupture, and sudden death and the ability to correct this lesion surgically make it especially important to consider aortic sinus of Valsalva aneurysm as a cause of obscure right-sided valvular disease.

Adolescent↗
PubMed1985

[Pathophysiology of tricuspid insufficiency: analysis of the motion of the tricuspid valve annulus using 2-dimensional echocardiography].

We investigated tricuspid annular motion in patients with pulmonary hypertension and in normal controls to determine the greatest minimal diameter and percentage shortening of the tricuspid annulus required for functional tricuspid regurgitation. 73 patients were studied by 2-dimensional echocardiography: a control group of 30 patients (group I); 43 patients had pulmonary hypertension, 9 of whom were still in sinus rhythm (group II), the other 34 patients had atrial fibrillation. 19 of these showed competent tricuspid valve with contrast echocardiography (group III), whereas the 15 remaining patients had functional tricuspid regurgitation (group IV). An analysis of shape and position changes of tricuspid annulus during the heart cycle was performed. The maximal diameter (mm/m2) in the apical 4 chamber view was in group I 17.5 +/- 1.4, in group II 20.7 +/- 3.2 (vs. group I p less than 0.05), in group III 19.0 +/- 3.4 (vs. group II NS) and in group IV 25.7 +/- 6.0 (vs. group III p less than 0.001). The values for the minimal annular diameter (mm/m2) were in group I 13.7 +/- 1.2, in group II 17.4 +/- 3.5 (vs. group I p less than 0.01), in group III 16.6 +/- 3.3 (vs. group II NS) and in group IV 23.6 +/- 5.7 (vs. group p less than 0.001). The percent decrease (%) in group I was 21.5 +/- 3.3, in group II 17.0 +/- 6.9 (vs. group I p less than 0.05), in group III 12.8 +/- 4.7 (vs. group II p less than 0.05) and in group IV 7.9 +/- 3.4 (vs. group III p less than 0.001).(ABSTRACT TRUNCATED AT 250 WORDS)

Echocardiography↗
PubMed1990

Advantage of cine-MR imaging for the evaluation of valvular regurgitation.

It is well known that the regurgitant blood flow of valvular disease shows a low intensity signal (LS) in cine-MR imaging (cMRI). To determine the significance of cMRI in evaluation of valvular regurgitation, 104 patients who had been assessed by cineangiography also underwent oblique cMRI for mitral regurgitation (MR) and aortic regurgitation (AR), and transverse cMRI for tricuspid regurgitation (TR). In evaluating the accuracy of diagnosis of valvular regurgitation on the basis of the presence of LS in the recipient chamber, sensitivity was found to be 93.5% for MR, 100% for AR and 85.7% for TR; specificity was 76.9% for MR and 100% for AR. The length and area of LS were planimetered in any view with the largest area, and its ratios to the length and area of the recipient chamber of view were calculated. Each index was significantly higher than the degree calculated by cineangiography. The best correlation with angiography was the ratio of the area of LS to the recipient chamber, and a semiquantitative classification by the index was coincident with angiographic severity in 38 of 43 (88.4%) patients undergoing MR, and in 28 of 30 (93.3%) patients undergoing AR. Thus, cMRI is a clinically useful noninvasive method in the identification and semiquantitative assessment of regurgitation severity.

Aortic Valve Insufficiency↗
PubMed1977

Atresia of the right atrioventricular orifice.

The results are reported of a study of 83 necropsied hearts with atresia of the right atrioventricular orifice. It is emphasised that right atrial or atrioventricular orificial atresia is a better term to describe this anomaly than "tricuspid atresia". Use of the latter term can be confusing when the morphologically tricuspid valve is located beneath the left atrium. It is accepted that the definition employed may include cases in which the mitral valve may be atretic, blocking normal exit from the right atrium, but it is argued that such cases would present clinically as "tricuspid atresia" and therefore are correctly designated as right atrial orificial atresia. The results show that the majority of hearts with right atrial orificial atresia have the ventricular morphology of primitive ventricle, most with, but a few without an outlet chamber. However, in a minority of hearts an imperforate membrane interposes between the right atrium and a formed but hypoplastic right ventricle. In two of the hearts, the imperforate membrane showed features of Ebstein's malformation. The hearts could be further subdivided according to the ventriculoarterial connection. Most had normally connected arteries (66 of 83), and all but 2 also had normal relations between the arteries; in these 2 hearts there was "anatomically corrected malposition". Twelve hearts showed transposition, one had double-outlet outlet chamber, and another persistent truncus arteriosus. The remaining 3 hearts, all without outlet chamber, had by definition a double outlet connection. A segmental approach provides the best way of classifying this anomaly, and an embryological explanation is offered for the variations in anatomy observed.

Aortic Valve Stenosis↗
PubMed1987

Intraoperative evaluation of reconstruction of the atrioventricular valves by transesophageal echocardiography.

In a total of 30 operations the mitral valve was reconstructed in 18, the tricuspid valve in 11, and both AV-valves in one patient. The result of reconstruction was tested first in the open arrested heart by injection of crystalloid solution into the appropriate ventricle either through the reconstructed valve or one of the great arteries. Upon termination of cardiopulmonary bypass the valve function was examined in the beating heart by means of transesophageal contrast echocardiography (TEE). For visualization 0.5-1.0 cc of agitated Gelifundol was injected into the ventricle. In 22 patients open testing as well as echocardiographic visualization showed identical and good operative results. In 6 patients TEE revealed mild insufficiency, but no further surgery was done. In three patients with mitral valve repair, contrast-echocardiography showed severe insufficiency, which had not been detected during cardiac arrest. In these patients changes in the level of the subvalvular apparatus caused systolic dislocation of the leaflets and massive reflux. Because of these findings definive valve replacement was performed in the same operation. Intraoperative TEE is a simple, safe and reliable method für assessment of AV-valve repair, thereby motivating the surgeon towards valve preservation and improving the outcome of valve surgery.

Adult↗
PubMed1982

Rest and exercise hemodynamics after the Fontan procedure.

Sixteen consecutive patients (12 with tricuspid atresia) underwent a Fontan procedure, with no operative deaths. There were three late deaths. Eleven of the survivors were electively catheterized 4-25 months postoperatively. Rest and exercise hemodynamics were measured in five patients, and resting hemodynamics alone were measured in three. All exercised patients were New York Heart Association class I Cardiac index was low at rest (2.3 +/- 0.61/min/m2) and during exercise (4.9 +/- 1.11/min/m2) due to a low stroke index both at rest (28 ml) and exercise (35 ml). Accordingly, mixed venous oxygen saturations were decreased (66% at rest and 31% during exercise). These values are significantly lower than those at rest and during exercise from 23 control patients of similar age and size. Heart rates, pulmonary vascular resistances, and left ventricular filling pressures appeared normal both at rest and during exercise. High right atrial pressure at rest (15 mm Hg) was associated with minimal conduit gradient (2 mm Hg). However, exercise increased the mean conduit gradient to 8 mm Hg, demonstrating significant functional conduit obstruction. As expected, the cardiovascular response to exercise is abnormal after the Fontan procedure, even in asymptomatic patients. This abnormal response may be exacerbated by conduit obstruction, and conduit obstruction may not be apparent during resting studies.

Adolescent↗
PubMed1999

Circumscribed intestinal protein loss with deficiency in CD4+ lymphocytes after the Fontan procedure.

UNLABELLED: Protein-losing enteropathy is an important complication after right heart bypass operations (Fontan procedure). Laboratory examinations usually reveal hypoalbuminaemia, hypoproteinaemia, elevated alpha(1)-antitrypsin clearance, and lymphocytopenia. A case of protein-losing enteropathy after Fontan procedure is reported with a circumscribed protein loss in the region of the terminal ileum despite good haemodynamics. The patient developed only mild hypoalbuminaemia and no diarrhoea but severe cellular and humoral immune abnormalities, namely a markedly decreased proportion of CD4+ lymphocytes but normal proportion of CD8+ lymphocytes (CD4+ 14%, CD8+ 23%) and decreased serum levels of immunoglobulin G. Intestinal biopsies revealed normal mucosa. This report is unique as it is the first to describe a ratio of CD4+ to CD8+ lymphocytes <1 due to an almost selective loss of CD4+ lymphocytes and a circumscribed intestinal protein loss in a patient who developed protein-losing enteropathy after Fontan operation. CONCLUSION: There is a severe decrease of CD4+ lymphocytes of unknown origin in a patient with circumscribed intestinal protein loss after Fontan operation. Passive leakage of lymph fluid due to abnormal systemic venous pressure is not a sufficient explanation of the almost selective loss of CD4+ lymphocytes. Primary or secondary activation of the immune system may influence structural integrity and permeability of the intestinal wall and may play a triggering role in protein-losing enteropathy after the Fontan procedure.

CD4 Lymphocyte Count↗