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[Primary cardiac rhabdomyosarcoma involving the mitral valve].

Primary cardiac rhabdomyosarcoma is rare and its extension to the mitral valve even rarer. We report a case of left atrial rhabdomyosarcoma involving the mitral valve. The patient was a 62-year-old man who complained of recurrent pre-syncopal episodes, dyspnoea often sudden in onset, asthenia and major weight loss (10 kg in one month). 2-D echocardiography revealed a 4.9 cm2 wide mass attached to the atrial side of the anterior mitral leaflet and to the adjacent inferior interatrial septum, where it seemed to have origin. CT scan and scintigraphy revealed bone, kidney and spleen metastases. The patient underwent emergency cardiac surgery because of increasing pre-syncopal and dyspnoeic episodes due to obstruction by the intracardiac mass. At surgery a tumor was found infiltrating the left atrial wall, the interatrial septum, the mitral anulus and the anterior mitral leaflet up to its tip. Invasion of mitral anulus did not allow mitral valve replacement, so that an excision of the intracardiac mass was performed as extensively as possible. Histology revealed a rhabdomyosarcoma. A post-operative chemotherapy cycle had to be stopped due to onset of atrial fibrillation and dyspnoea. 2-D echo monitoring revealed rapid new growth of the tumor across the basal portion of mitral valve leaflet to the atrioventricular orifice. After several episodes of increasing dyspnoea, the patient had a pulmonary oedema and died.

Dyspnea↗

Severe calcification does not affect long-term outcome of mitral valve repair.

Some surgeons have suggested that the presence of severe calcification in the mitral valve annulus or leaflets precludes successful repair. Our institution has attempted to repair these calcified valves when good annular and leaflet mobility could be achieved by annular debridement and leaflet resection. From June 1979 through June 1993 558 mitral valve repairs were performed using Carpentier's techniques. When calcified valves were encountered, these techniques were modified to include annular debridement and mechanical leaflet decalcification. Calcification was identified preoperatively in 49 patients (8.8%) by either left ventricular fluoroscopy or echocardiography and was debrided in 64 patients (11.5%). This included 24 annular debridements, 28 leaflet debridements, and 12 annular and leaflet debridements. Patient ages ranged from 13 to 83 years (mean age, 62.3 years), and 25 patients (39.1%, 25/64) had concomitant cardiac procedures. Operative mortality was 6.2% (4/64) overall and 2.6% (1/39) for isolated mitral valve repairs. Calcium debridement was performed in 19.3% (23/119) of patients with a rheumatic cause compared with 9.3% (41/439) of the nonrheumatic patients (p < 0.01). Long-term follow-up revealed the necessity for reoperation in 7.8% (5/64) in patients with calcium debridement as compared with 7.7% (38/494) with no debridement (p = not significant). Cumulative freedom from reoperation at 10 years was 83.3% for all patients, 88.1% for debrided patients, and 82.6% for nondebrided patients (p = not significant). Cox proportional hazards analysis revealed that the presence of rheumatic disease significantly increased the risk of reoperation (odds ratio = 3.28; p < 0.001), whereas calcium debridement had no significant effect. These results demonstrate that when good annulus and leaflet motion can be achieved in calcified mitral valves, calcium debridement allows durable repairs.

Adolescent↗

Mitral valve repair in severe ischemic cardiomyopathy.

BACKGROUND: Patients with ischemic mitral valve insufficiency (MR) and poor left ventricular (LV) function present a high operative risk. Whether to repair or replace these valves is controversial, while some suggest that heart transplant offers a better solution. We investigated our early and late results in this difficult subset of patients. METHODS: Between 1993-1999,115 patients underwent mitral valve repair (MVR) in our department. Twenty-one patients had severe LV dysfunction with ejection fraction < 25%. Mean age was 60 years (range 45-81). Nineteen (90%) were in New York Heart Association (NYHA) Class IV, 7 (33%) underwent emergency surgery, 3 (14%) were in cardiogenic shock, and 2 (10%) were taken to the operating room under cardiopulmonary resuscitation. All underwent coronary artery bypass grafting (CABG) in addition to MVR, with a mean number of grafts 2.9 per patient. RESULTS: There were no early operative deaths. The average stay in intensive care was 5.9 days (range 1-52). There were three late deaths (14%). Follow-up evaluation up to 3 years showed marked improvement in clinical status. Twelve (67%) patients are in NYHA Class I-II, and three (17%) in Class III. Echocardiography revealed good function of the mitral valve in all, although overall LV function did not change significantly. CONCLUSION: (1) MVR in patients with severe ischemic cardiomyopathy can be accomplished with excellent results. (2) There is marked symptomatic improvement in these patients, even though LV function did not seem to be improved. (3) Long-term survival still needs to be defined.

Aged↗

Myxoma attached to both atrial and ventricular sides of the mitral valve: report of a case and review of 31 cases of mitral myxoma.

We report for the first time upon a case of myxoma that involved both atrial and ventricular sides of the anterior mitral valve leaflet. Electron beam tomography (EBT) showed that the tumor was attached to both the atrial and ventricular sides of the mitral valve, while echocardiography did not. EBT also diagnosed dynamic obstruction of left ventricular outflow tract caused by the tumor.

Adult↗

Percutaneous transarterial balloon dilatation of the mitral valve: five year experience.

OBJECTIVE: To examine the value of transarterial balloon dilatation of the mitral valve for treatment of patients with mitral stenosis over a period of five years. DESIGN: Analysis of patients' functional state, and haemodynamic and echocardiographic variables, before and immediately after the procedure and during a follow up of up to five years. SETTING: A cardiovascular centre in Belgrade, Yugoslavia. PATIENTS: Two hundred and ninety four patients who underwent percutaneous transarterial dilatation of the mitral valve between February 1985 and February 1990. RESULTS: Mean mitral valve area was enlarged by 109%. Complications included death (0.7%), severe mitral insufficiency (2.3%), mild mitral insufficiency (9.9%), cerebral embolism (2%), and injury to the femoral artery (2%). Two more patients died at two and 11 months after the procedure. Late cardiac surgery was needed in eight patients (mitral insufficiency in three, restenosis in three, thrombus in one, and endocarditis in one. Restenosis occurred in seven patients. Four of these underwent repeat dilatation and three had surgery. Improvement of symptoms was seen in 94% of patients during the follow up. CONCLUSION: Transarterial balloon dilatation of the mitral valve gave good results with acceptable morbidity and mortality and had some advantages over the anterograde approach.

Adolescent↗

Preservation of anterior leaflet chordae tendineae during mitral valve replacement.

Preservation of anterior mitral leaflet papillary muscle attachments during mitral valve replacement was achieved in 20 patients by excising the central portion of the anterior leaflet and suturing the rim of leaflet tissue containing marginal chordae to the remaining edge of leaflet attached to the left atrium. This closure tacks the chordae to the fibrous trigones, opens the subvalvular area, and avoids a potentially thrombogenic or obstructive redundant leaflet ridge along the rim of an inserted valve. The technique is simple, safe, reproducible, and teachable.

Cardiac Surgical Procedures↗

Technique of mitral valve replacement.

A technique for mitral valve replacement is described that provides adequate exposure for excision of the valve and secure suturing of the annulus, even in patients with a small left atrium. The technique has been used in more than 100 patients and has resulted in only a minimal amount of perivalvular leak.

Aortic Valve Stenosis↗

A computational study of the hemodynamics after "edge-to-edge" mitral valve repair.

Edge-to-edge mitral valve repair consists in suturing the free edge of the leaflets to re-establish coaptation in prolapsing valves. The leaflets are frequently sutured at the middle and a double orifice valve is created. In order to study the hemodynamic implications, a parametric model of the left heart has been developed. Different valve areas and shapes have been investigated. Results show that the simplified Bernoulli formula provides a good estimation of the pressure drop and that the pressure drop may be predicted on the basis of the pre-operative geometric and hemodynamics data by means of customized models.

Blood Flow Velocity↗

OPEN-HEART SURGERY FOR MITRAL VALVE DISEASE.

The findings in and experiences with 19 consecutive patients subjected to open mitral valve surgery are described. All patients underwent a right-heart catheterization. In order to exclude multivalvular heart disease, a left-heart catheterization was performed in 10 patients and angiographic studies of the aortic valve area in 12. Pulmonary function studies were performed on 11 patients. The FEV (0.75 sec.) and the MMFR were found to correlate well with existing pulmonary reserves.Fourteen of the 19 patients subjected to open mitral valve surgery survived the operation and have been followed up for three months to 2(1/2) years. Four patients had a mitral valvuloplasty. Three of these four have deteriorated and will require a valvular replacement. Ten of 15 patients subjected to a mitral valve replacement are alive; five of these 10 have had signs and symptoms indicating peripheral embolization. Mitral valvuloplasty is preferable to a valvular replacement as far as embolic complications are concerned, while valvular replacements result in a more perfect and lasting hemodynamic repair. The high incidence of peripheral embolization following valvular replacements focuses attention on the need for improved mitral valve prostheses.

Angiocardiography↗

[Ventricular arrhythmias in patients with mitral valve prolapse and spurious cords in heart ventricles].

The aim of the work to assess the frequency of ventricular arrhythmias in patients with isolated mitral valve prolapse isolated spurious cords in heart ventricles as well as in those patients who had both, mitral valve prolapse and spurious cord in heart ventricle. Out of 4156 successive electrocardiographic investigations carried out at the Department and Clinic of Cardiology, Medical Academy in Wroclaw, coexistence of spurious cords in heart ventricle and mitral valve prolapse was diagnosed in 32 patients (0.8% of all subjects), isolated mitral valve prolapse--in 184 patients (4.4%) and presence of isolated spurious cords not accompanied by mitral valve prolapse--in 91 patients (2.2%). In 20 patients with coexistence of spurious cords in heart ventricles and mitral valve prolapse, ventricular arrhythmias occurred in 12 subjects (60.0%) and in 5 of them they were significant. In the group of 29 patients with isolated spurious cords, disturbances of the ventricular rhythm occurred in 13 subjects (44.8%), while significant ventricular arrhythmias were found in 6 patients (20.7%). Out of 60 subjects with isolated mitral valve prolapse disturbances of the ventricular rhythm were found in 20 patients (33.3%) and significant arrhythmias--in 12 subjects (20.0%). The observations did not reveal increased prevalence of significant ventricular disturbances of the heart rhythm in patients with coexisting mitral valve prolapse and spurious cords in the heart ventricles.

Adult↗

Left atrial performance indices in chronic mitral valve disease.

Multidimensional left atrial (LA) performance indices have not been extensively studied in chronic mitral valve disease. LA maximal volume, stroke volume (LA volume at atrial systole minus LA minimal volume), LA ejection fraction (stroke volume/volume at atrial systole) and A-wave velocity, were measured in 14 patients with mitral stenosis (mean mitral valve area 1.5 cm2); 14 patients with chronic mitral regurgitation all in sinus rhythm; and were compared to 20 age and sex matched normal control subjects using biplane transthoracic echo and pulsed Doppler. Although LA volumes--maximal and at onset of atrial systole--were greater in mitral regurgitation and mitral stenosis (p < 0.01) compared to normal subjects, LA ejection fraction was not statistically different among the three groups. LA stroke volume was greater in mitral regurgitation and mitral stenosis compared to normal subjects, p < 0.01. LA kinetic energy (LAKE) = 1/2 mv2 (m = LA stroke volume x 1.06, blood's specific gravity, v = A wave velocity) was increased in mitral stenosis and mitral regurgitation compared to normal subjects (p < 0.001). An inverse correlation (r = 0.66, p < 0.01) was present between LAKE and mitral valve area in mitral stenosis. It is concluded that LA function, a complex interplay of multiple factors, requires multidimensional methods of analysis beyond standard measurements of size and volume, which provide additional insight into normal LA function, and better definition of LA function changes involved in the natural history of chronic mitral valve disease.

Adult↗

Emergency correction of coagulation for mitral valve replacement in an orally anticoagulated 17-year-old patient with pronounced hepatic dysfunction.

A 17-year-old patient with Shone's disease had to be readmitted to the hospital 3 months after implantation of an artificial aortic valve because of extreme mitral insufficiency with consecutive pulmonary edema and hepatic dysfunction. He had been orally anticoagulated and presented with a high international normalized ratio of 6.7. Emergency replacement of the mitral valve was possible only after administration of prothrombin-complex concentrate, as vitamin K(1) and fresh frozen plasma did not correct the hemostatic defect sufficiently.

Adolescent↗

Prosthetic mitral valve motion during cardiac dysrhythmias as determined by echocardiography.

To assess the changes and physiologic mechanisms of prosthetic valve motion during cardiac dysrhythmias as well as the role of atrial systole in the closure of the mitral valve, M-mode echocardiography was performed in 36 patients with normally functioning prosthetic mitral valves (Björk-Shiley, Starr-Edwards, and Beall valve). Premature closure of the prosthetic mitral valve in diastole with a "sharp" closing motion was seen during first-degree atrioventricular block, atrial fibrillation with ventricular rates less than 60 beats/min, and atrial flutter. A "rounded" premature valve closure due to atrial systole was seen during atrial tachycardia and complete heart block. Atrial systole initiates a closing motion of the prosthetic mitral valve at end-diastole, and ventricular systole completes this closure during normal sinus rhythm. When first-degree atrioventricular block is present, atrial systole alone completes this closure before ventricular contraction. Atrial contraction alone also can effectively close the prosthetic mitral valve during atrial flutter and atrial tachycardia. Other factors (such as left ventricular diastolic volume) may play a role in the effective closure of the prosthetic mitral valve during atrial fibrillation with slow ventricular rates and complete heart block. These findings must be considered in the echocardiographic evaluation of suspected malfunctioning prosthetic mitral valves. A baseline postoperative echocardiogram after prosthesis insertion is important for future evaluation when clinically indicated.

Aged↗

Mortality and morbidity after mitral valve repair: the importance of left ventricular dysfunction.

A retrospective study of 219 consecutive patients who underwent mitral valve repair for mitral regurgitation or mixed mitral valve disease with at least moderate regurgitation was undertaken. The etiology was degenerative in 151 (68.9%) patients, endocarditis in 24 (10.9%), rheumatic in 22 (10.0%), ischemic in 13 (5.9%), congenital in five (2.3%) and cardiomyopathy in two (0.9%). The average age was 64.8 +/- 10.9 years, the average follow up 30.2 +/- 24.1 months. Pre-operatively, 74% were in NYHA functional class III or IV. Left ventricular function was assessed by angiography and echocardiography, and moderate or severe impairment (ejection fraction < or = 40%) was assumed to represent significant left ventricular dysfunction. There were six (2.7%) hospital deaths, four of which were due to left ventricular dysfunction. The seven-year mortality and seven-year combined mortality and morbidity were 14.4% and 37.1% respectively for complications related to left ventricular dysfunction, and 2.5% and 24.2% respectively for unrelated complications. Subgroup analysis showed that five-year mortality and five-year combined mortality and morbidity due to left ventricular dysfunction were significantly worse in patients who had pre-operative left ventricular dysfunction than those who did not: 28.5% vs. 6.1% (p < 0.001) and 52.4% vs. 17.8% (p < 0.001). There was nevertheless a significant incidence of postoperative left ventricular dysfunction in patients with satisfactory preoperative left ventricular function. In this group, five-year mortality and five-year combined mortality and morbidity due to left ventricular dysfunction were higher in patients who were in NYHA class III or IV preoperatively than in those who were not: 11.2% vs. 0% (NS) and 25.9% vs. 0% (p < 0.01) respectively, particularly if they also had early (3-10 days) post-operative left ventricular dysfunction: 20.4% (p < 0.001) and 41.7% (p < 0.001) respectively. Despite preservation of the mitral apparatus, left ventricular dysfunction remains a major cause of mortality and morbidity following mitral valve repair for mitral regurgitation.

Adolescent↗

Observer variation in the echocardiographic diagnosis of mitral valve prolapse.

To assess inter- and intraobserver variation in the echocardiographic diagnosis of mitral valve prolapse, three independent observers analyzed M-mode echocardiograms (n = 80) and two-dimensional echocardiograms (n = 65) of patients with a mobile midsystolic click with or without a late or holosystolic murmur. In addition, a control group of 100 normal echocardiograms were interspersed among the echocardiograms of patients with mitral valve prolapse and were then interpreted. Each of the three observers analyzed all M-mode and two-dimensional echocardiograms initially and then 2 weeks later for the presence or absence of mitral valve prolapse. M-mode echocardiographic criteria for mitral valve prolapse consisted of late systolic posterior motion (greater than or equal to 3 mm) of one or both mitral leaflets or holosystolic hammocking (greater than or equal to 3 mm) of one or both mitral leaflets. Two-dimensional echocardiographic criteria for mitral valve prolapse consisted of: posterior systolic arching of one or both mitral leaflets in the parasternal long-axis view, and/or posterior systolic bowing of one or both mitral leaflets in the apical four-chamber view posterior to the plane of the mitral anulus, and/or excessive posterior coaptation of the mitral leaflets in either view flush with or posterior to the plane of the mitral anulus. There was insignificant observer variation both in the M-mode and two-dimensional echo groups, as determined using Cochran's Q test.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

The risk of thromboembolism and hemorrhage following mitral valve replacement. A comparative analysis between the porcine xenograft valve and Ionescu-Shiley bovine pericardial valve.

To ascertain the risk of thromboembolism and anticoagulant-related hemorrhage following mitral valve replacement with bioprostheses, an 8 year retrospective study between two groups of patients was analyzed. Group I included 206 patients undergoing mitral valve replacement with porcine xenograft valves. They were placed on a regimen of long-term oral anticoagulation (greater than 8 weeks, mean 6 months). Follow-up was 524.3 patient-years, mean 30.5 months. There were 24 thromboembolic events (4.6% per patient-year), four of which were fatal. Actuarially, 80.7% +/- 4.3% are free of thromboembolism at 8 years. There were 12 instances of major bleeding episodes, for a linearized incidence of 2.5% per patient-year; two were fatal. Group II included 322 patients undergoing mitral valve replacement with a bovine pericardial valve. They were placed on a program of short-term anticoagulation (6 weeks only). Follow-up was 1,106 patient-years, mean 46.4 months. There were four thromboembolic episodes (none fatal), an incidence of 0.36% per patient-year. Seven bleeding episodes occurred, 0.63% per patient-year; none was fatal. The difference between the groups reached statistical significance (p less than 0.001). The low risk of thromboembolism with the bovine pericardial valve appears to be due to its superior hydraulic characteristics. Use of this valve allows mitral valve replacement without long-term oral anticoagulation and the associated risk of anticoagulant-related hemorrhage.

Adolescent↗

Mitral valve repair: the Manipal alternative.

BACKGROUND AND AIM OF THE STUDY: An easily reproducible, rational and durable method of repairing the incompetent mitral valve, which does not require complex chordal procedures or the use of an expensive prosthesis and long-term anticoagulation, remains a desirable goal. Here, we describe such a method that has been developed at our institute. METHODS: The step-wise repair comprises: (i) preparation of a ring from a 3 x 110 mm strip of Dacron felt covered with untreated autologous pericardium; (ii) mitral commissurotomy and mobilization of the subvalvular apparatus, when required; (iii) infolding of the small portion of flail unsupported mitral leaflet, when present, by interrupted stitches; (iv) anchoring of the pre-prepared ring to the mitral annulus with interrupted horizontal mattress sutures, the sutures on the posterior annulus stopping short of the commissures by 12-15 mm and on the anterior annulus by 8-10 mm; (v) excision of the unanchored portions of the ring opposite the commissures, leaving behind 76-84 mm of the anchored parts; (vi) placement of two 'U-on-side' pericommissural annuloplasty sutures passed through the cut ends of the incomplete ring, then through the respective annulus, and finally emerging near the anterolateral and posteromedial commissures; and (vii) tying off the two pericommissural sutures over Teflon pledgets. RESULTS: Between January 1988 and December 1997, the technique was used to repair 107 mitral valves. Among 90 patients who had mitral valve repair alone or combined with tricuspid or aortic valve repair, only one hospital death occurred. One patient required reoperation due to an unacceptable degree of hemolysis. Among survivors followed up from one to >10 years, 80% were in NYHA functional class I, and 70% did not have clinical mitral regurgitation. CONCLUSION: This alternative technique of mitral valve repair is simple to perform, and relatively inexpensive. It provides gratifying results in acquired mitral valve disease, as well as in mitral valve prolapse subjects, and the repaired valve appears to function well, even after 10 years.

Adult↗

Juvenile autoimmune thyroiditis and mitral valve prolapse.

An increased incidence of mitral valve prolapse (MVP) has been reported in adult patients with autoimmune thyroid disease. The aim of this study was to assess the incidence of MVP in children and adolescents with juvenile autoimmune thyroiditis (JAT). Cardiac echo studies using M-mode, 2D, and Doppler examinations were performed on 23 patients (21 females, 2 males). The patients were studied at a median age of 12 years (range 5-20 years). Only one patient was found to have evidence suggestive of MVP, an incidence (4.3%) similar to that seen in the normal pediatric population. We, therefore, conclude that the incidence of MVP in children and adolescence with JAT is not increased.

Adolescent↗