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Localization and quantification of mitral valve prolapse using three-dimensional echocardiography.

AIMS: Mitral valve prolapse is a common source of severe mitral regurgitation in Western countries. Three-dimensional echocardiography can provide views of the entire valve, allowing a complete assessment of the valve leaflets and commissures. It has the potential to precisely locate and quantify mitral valve prolapse. METHODS AND RESULTS: Between January 1997 and December 2000, 91 patients with severe mitral regurgitation due to mitral valve prolapse underwent a transesophageal echocardiography with three-dimensional reconstruction of the mitral valve as part of their pre-operative work-up. The location and extent of the prolapse by echo was compared to the surgical status. The volume of prolapsing leaflet was calculated and compared to the volume of resected tissue whenever a repair was attempted. There was an excellent correspondence between the echographic localization of the prolapse and surgical inspection, and between the volume of prolapsing and surgically resected tissue (r=0.94, p<0.0001). CONCLUSIONS: In patients with severe mitral regurgitation due to mitral valve prolapse, 3D echo allowed a precise localization and an accurate quantification of the prolapsing portion of the leaflets. This technique can provide refinements in the surgical planning of mitral valve repair and in the selection of candidates for this intervention.

Adult↗

Mapping of a first locus for autosomal dominant myxomatous mitral-valve prolapse to chromosome 16p11.2-p12.1.

Myxomatous mitral-valve prolapse (MMVP), also called Barlow disease, is a common cardiac abnormality and affects up to 5% of the population. It is characterized by an excess of tissue that leads to billowing of the mitral leaflets, sometimes complicated by prolapse. Typical histological findings include myxomatous degeneration and degradation of collagen and elastin. Previous reports have proposed an autosomal dominant inheritance of the trait, with age- and sex-dependent expression. By systematic echocardiographic screening of the first-degree relatives of 17 patients who underwent mitral-valve repair, we have identified four pedigrees showing such an inheritance. Genomewide linkage analysis of the most informative pedigree (24 individuals, three generations) showed a significant linkage for markers mapping to chromosome 16p, with a two-point maximum LOD score for D16S3068 (Zmax=3.30 at straight theta=0). Linkage to D16S3068 was confirmed in a second family (Zmax=2.02 at straight theta=0) but was excluded for the two remaining families, thus demonstrating the genetic heterogeneity of the disease. Multipoint linkage analysis performed, with nine additional markers, on the two families with linkage gave maximum multipoint LOD scores of 5.45 and 5.68 for D16S3133, according to a conservative and a stringent model, respectively. Haplotype analysis defined a 5-cM minimal MMVP-1 locus between D16S3068 (16p11.2) and D16S420 (16p12. 1) and a 34-cM maximal interval between D16S404 and D16S3068 when recombination events were taken into account only in affected individuals. The identification of this locus represents a first step toward a new molecular classification of mitral-valve prolapse.

Chromosome Mapping↗

Mitral valve prolapse: a study of 45 children.

The knowledge pertaining to mitral valve prolapse is mainly based on studies in adults. In this study, the clinical profile as described in adults was compared with that found in children up to the age of 13 years. Forty-five children with echocardiographic-proven mitral valve prolapse and who met the inclusion criteria were included in the study. The male:female ratio in this study was 1:1.37 and was not statistically significantly different from reported ratios. Most of the children were asymptomatic. Twenty-one of the 31 patients referred from outside the hospital had an incidentally found murmur. The symptoms found in this study were not similar to those described in adults. The most commonly found symptoms were shortness of breath and fatigue, in contrast to those of chest pain and palpitations described in adults. Comparing males to females in this study, significantly lower weight (p = 0.005) and body mass index (p = 0.003) were found in girls, and a significantly lower pulse rate (p = 0.002) in boys. Left-sided cardiac enlargement was diagnosed in 11 patients on chest X-ray and in six patients on electrocardiogram. One patient had Marfan syndrome and four others had a Marfanoid appearance. In conclusion, most children with mitral valve prolapse are asymptomatic. Mitral valve prolapse is not an uncommon finding in children younger than 13 years of age. Patients with mitral valve regurgitation were advised to take infective endocarditis prophylaxis prior to invasive procedures.

Adolescent↗

Five-year experience with a suture annuloplasty for mitral valve repair.

We present five years' experience with mitral plication annuloplasty, performed with a semicircular buttressed suture around the posterior leaflet in 130 patients (mean age 58 +/- 11 years) with primary mitral valve disease (n = 71) or functional mitral regurgitation (n = 59). In 65 cases the mitral valve itself was also repaired. Concomitant myocardial revascularization was performed in 40 cases and aortic valve replacement in 43. All but three patients were followed up (97.6%). Postoperative echocardiography showed acceptable mitral area (2.28 +/- 0.39 cm2) and good valve competence in all cases. Inhospital mortality was 3% and late mortality 4.8%. During the follow-up period (22.8 +/- 10.9 months) 8 patients (6.6%) required mitral valve replacement because of progression of native valve disease (n = 4), technical failure (2) or expansion of the annuloplasty suture (2). Mitral annuloplasty thus can be performed simply and with good results, using a strong, non-stretchable buttressed suture. This procedure can be an inexpensive alternative to ring implantation.

Aged↗

Minimally invasive, robotic, and off-pump mitral valve surgery.

A significant transformation is occurring in the management of mitral valve disease. Earlier surgery is now recommended. Mitral valve repair is the standard of care, and newer methods of reconstructing the mitral valve are developing. Surgery with videoscopic assistance can be effectively performed without sternotomy. Robotics systems are gaining wider adoption. Implantable devices to repair or replace the mitral valve off-pump and percutaneously are emerging.

Cardiopulmonary Bypass↗

Left ventricular function after mitral valve replacement with or without chordal preservation.

The clinical significance of the chordae tendinae regarding postoperative left ventricular performance was evaluated in 148 patients with mitral regurgitation or mitral stenosis who underwent either mitral valve replacement using St. Jude Medical valve with complete chordal preservation, or with conventional mitral valve replacement, or valve repair. Mitral valve replacement preserving the autologous chordae tendinae (n = 28) or replacing them with Gore-Tex sutures (n = 16) was performed in 44 patients, 24 with mitral regurgitation and 20 with mitral stenosis. Their hemodynamic parameters were compared to those of patients who underwent conventional mitral valve replacement involving 25 with mitral regurgitation and 28 with mitral stenosis, or who underwent valve repair in 24 with mitral regurgitation, or commissurotomy in 27 patients with mitral stenosis. The LV performance was analyzed by cineangiography in the early (mean 1.2 months), and by multiple gated blood scintigraphy (MUGA), or echocardiography (UCG) in the late postoperative periods (mean 5.4 years) in the three groups of patients. In the mitral regurgitation group, the LV ejection fraction (EF) was unchanged in the chordal preserved group, but it was decreased in the conventional replacement and repair groups. The LV contractility index was better in the chordal preserved than in the conventional group. Both the LVEF by MUGA, and LV fractional shortening (FS) by UCG were significantly higher after chordal preservation or repair than after conventional valve replacement. The chordal preserved group exhibited superior LV performance than the conventional group, especially in those with mitral regurgitation and depressed preoperative left ventricular function (EF < 0.50). There were no significant difference between the three groups in patients with mitral stenosis. The results support the concept that maintenance of continuity between the mitral annulus and the papillary muscle has a beneficial effect on postoperative left ventricular performance, especially in patients with mitral regurgitation and depressed preoperative left ventricular function, but had no major effect in patients with mitral stenosis.

Adult↗

Mitral balloon valvotomy using the Inoue balloon technique for selected patients with severe pliable rheumatic mitral valve stenosis: immediate and short-term results.

We selected 40 patients with severe symptomatic rheumatic mitral stenosis for balloon valvotomy using the Inoue balloon technique. The patients' mean age was 31 +/- 14 years and there were 24 females and 16 males. The patients were selected according to the following echo/Doppler criteria; 1. Severe mitral stenosis, i. e. mitral valve area (MVA) less than 1.1 cm2; 2. pliable anterior mitral valve leaflet; 3. absence of calcification of the mitral commissures and 4. absence of significant subvalvular mitral valve disease (Block echo score less than 8). We failed to cross the mitral valve in three cases and repeat attempts in two patients with higher transeptal puncture was successful. Thirty-nine procedures were technically successful (98%). There were no complications. We used an Inoue balloon size 24-30 mm using echo/Doppler guided stepwise mitral dilatation. After mitral balloon valvotomy, the MVA increased from 0.8 +/- 0.2 to 1.7 +/- 0.5 cm2 (p less than 0.001). Five patients developed mild mitral regurgitation and in one patient the degree of mitral regurgitation increased from mild to moderate. The mean mitral valve area 48 hours after the procedure measured 1.9 +/- 0.4 cm2 (echo/Doppler); one patient (2.5%) had residual mitral stenosis (MVA less than 1.5 cm2). At six weeks follow-up study the mean mitral valve area was 1.9 +/- 0.5 cm2 (Echo/Doppler), with no restenosis. We conclude that in selected cases of severe pliable mitral stenosis, the Inoue balloon technique achieves a greater than 100% increase of the MVA, without inducing significant iatrogenic mitral regurgitation or residual stenosis.

Catheterization↗

Reoperation for hemolytic, anaemia complicating mitral valve repair.

OBJECTIVE: To identify the possible cause(s) of hemolysis after mitral valve repair for mitral regurgitation (MR) and to evaluate the late outcome of surgical treatment. METHODS: We reviewed all patients who had reoperation after valve repair for mitral regurgitation. Ten patients had reoperation because of hemolytic anaemia. The diagnosis of hemolysis was made by decreased serum haptoglobin, elevation of serum lactate dehydrogenase (LDH), and schistocytosis. No other causes of anaemia or hemolysis were identified in these six men and four women (ages 35-84 years; median 59 years). Interval between initial mitral valve repair and reoperation ranged from 40 to 165 days (median 87 days), and prior to reoperation, red cell transfusions (range 2-12 units; median 5 units) were required in all patients. Seven patients were symptomatic: two complained of easy fatigability and five were severely limited. Transesophageal echocardiogram during hemolytic evaluation showed only mild MR in two patients, moderate in five, moderately severe in two and severe in one. RESULTS: Etiology of hemolysis was suggested from echocardiography and confirmed at reoperation. In one patient, an eccentric MR jet struck a pledget of a commissural annuloplasty. In the remaining nine patients, the regurgitant jet struck a non-endothelialized portion of the annuloplasty ring (Carpentier-Edwards n = 5; Duran n = 2; Cosgrove-Edwards n = 2). Seven patients had prosthetic replacement and three patients had re-repair. There were no operative deaths and all patients had resolution of hemolytic anaemia. CONCLUSIONS: Relatively minor degrees of regurgitation after mitral valve repair can produce hemolytic anaemia which is manifested within the first few postoperative months. Most patients are highly symptomatic because of anaemia. The mechanism of red cell destruction is a high velocity eccentric stream of blood impacting on a small area of a prosthetic ring or pledget. This process retards endothelialization of the ring. Reoperation with re-repair or mitral valve replacement is safe and effectively relieves the hemolysis.

Adult↗

Beating mitral valve replacement for a patient with porcelain aorta.

Beating mitral valve replacement was performed for mitral valve stenosis in a patient with a porcelain aorta after aortic valve replacement. The 77-year-old patient had developed heart failure several times. A chest computed tomographic scan revealed severe calcification on the ascending aorta and aortic arch. A cardiopulmonary bypass was established by bicaval drainage and aortic return after epiaortic echographic evaluation of the ascending aorta. The mitral valve was successfully replaced under a beating heart.

Aged↗

Does preservation of the posterior chordae tendineae enhance survival during mitral valve replacement?

During a 30-month period, 51 patients underwent mitral valve replacement. There were 3 hospital deaths (5.9%), 2 of which were due to ventricular rupture. The 3 patients who died were among 13 patients in whom mitral valve replacement was combined with tricuspid or aortic valve operation or both. Postmortem findings in the 2 patients who died of ventricular rupture showed that the ventricular tears were located between the atrioventricular groove and the unresected papillary muscle stumps, in an area of ventricle formerly tethered by the posterior chordae tendineae. In the last 14 patients in the series, the posterior leaflet of the mitral valve and its chordae tendineae were left intact, and there was no mortality or prosthetic valve dysfunction. In patients with myxomatous or ischemic disease, the posterior leaflet was left completely intact. For patients with fibrocalcific rheumatic disease, we have developed a technique of partial excision and debridement of the posterior leaflet, preserving the intermediate and basal chordae tendineae attachments. With the techniques described, preservation of all or part of the posterior leaflet and its chordae tendineae does not appear to interfere with prosthetic valve function and, by reducing the risk of ventricular rupture, should enhance survival after mitral valve replacement.

Adult↗

[Diagnosis wi transesophageal echocardiography and clinical course of 35 patients with flail mitral valve].

PURPOSE: To describe transesophageal echocardiographic findings in patients presenting with flail mitral valve and to obtain their clinical follow-up. METHODS: From January/93 to March/97, 1675 patients were submitted to transesophageal echocardiogram at our institution; 35 of them were diagnosed as having flail mitral valve and their clinical follow-up was obtained. RESULTS: Thirty-five patients with the diagnosis of flail mitral valve were studied. Mean age was 65 +/- 15 years (12-87) and 27 (77%) were male. The posterior leaflet was involved in 25 (71%) patients. Ruptured chrodae tendineae was present in all but one patient in whom flail mitral valve was due to a very elongated and redundant chordae. The etiology was prolapse and/or mixomatous degeneration in 16 patients, degenerative in 10, ischemic in 5, rheumatic in 4 and endocarditis in 3. Mitral regurgitation was severe in 25 (71%) patients and moderate in 10 (29%). The mean follow-up was 375 +/- 395 days (1 to 1380). Nineteen patients were followed clinically and 16 were submitted to surgery (transesophageal echocardiogram findings were confirmed in all 16). Overall mortality was high (34%). Among the survivors, 17 are in NYHA class I and 6 in class II. CONCLUSION: The diagnosis of flail mitral valve by transesophageal echocardiography is accurate, allowing for the definition of its mechanism and etiology, as well as the evaluation of hemodynamic compromise. The observed high mortality at the time of diagnosis may be related to the severity of the disease causing the flail mitral valve. Although non-operated survivors are doing well, the observed low functional class in these patients may be related to the short period of follow-up.

Adolescent↗

Mitral valve reconstruction: eight years' experience in 531 patients.

Between January 1988 and June 1996, a total of 531 patients underwent reconstruction of the mitral valve for mitral regurgitation (MR) with or without mitral stenosis (MS). Of these patients, 423 (79.7%) were aged under 30 years. The cause of mitral regurgitation was rheumatic in 471 patients (88.7%). Combined lesion (MR + MS) was present in 296 cases (55.7%). Operative mortality rate was 5.1% (27 patients) and there were 11 late deaths. Ten patients have been reoperated on for residual MR. All surviving patients were followed up by physical examination and echocardiography; follow up was 93% complete. The actuarial survival rate at 84 months was 93.1% and event-free survival rate was 87.3%. We conclude that mitral valve repair in the young rheumatic population is feasible, the probability of reoperation is low, and the symptomatic relief is gratifying.

Adolescent↗

Diagnosis and management of the double orifice mitral valve: three case reports.

Double orifice mitral valve (DOMV) is an uncommon congenital heart defect. The isolated occurrence of this anomaly is very rare and, more often, is associated with another congenital malformation, dominated by atrioventricular canal defects (AVCD). Mitral insufficiency and/or stenosis may complicate this malformation. Treatment may be summarized as abstention, surgical valve repair, or valve replacement. In here, we report three cases with DOMV. The first patient was an 18-month-old boy who was operated for severe mitral valve stenosis and mild insufficiency (in 1980s), the second was a 47-year-old female, who was operated recently for mitral valve replacement (MVR) under cardiopulmonary bypass due to severe mitral valve insufficiency. This patient had been operated due to the secundum-type atrial septal defect in our institution previously. The last one, a 3-year-old boy, with DOMV and three papillary muscles, was on clinical follow-up because he had no symptoms.

Female↗

Edge-to-edge mitral valve repair: the Columbia Presbyterian experience.

BACKGROUND: The edge-to-edge mitral valve repair, first described by Alfieri in 1995 treats mitral regurgitation when standard reparative techniques are difficult, unlikely to succeed, or have failed. This study examines one institution's medium-term experience with this procedure. METHODS: This study involved patients undergoing edge-to-edge mitral valve repair at a single institution from 1997 to 2003. Preoperative and postoperative echocardiograms were compared. Postoperative morbidity was examined including need for reoperation and long-term medical management. Thirty-day survival and long-term actuarial survival were also determined. RESULTS: Seventy-one patients comprised this study. Mitral regurgitation on echocardiogram went from 3.43 +/- 0.86 to 0.39 +/- 0.61 (p < 0.001) following repair. Thirty-day mortality was 3 of 71 (4.2%) patients. Actuarial survivals at 24 and 60 months were 84.5% and 58.3%, respectively; adjusted excluding noncardiac death they were 89.5% and 82.3%, respectively. Forty (56.3%) patients had concomitant ring placement and experienced similar survival to those repaired with the bow-tie stitch alone. Home telephone follow-up was conducted, and current medical therapy was determined on 51 patients; 59% were on a beta-blocker, 31% were on an angiotensin-converting enzyme (ACE) inhibitor, 27% were on a diuretic, and 22% were on digoxin. All were New York Heart Association (NYHA) class I or II. Three patients (4.2%) underwent mitral valve reoperation after a mean of 299 +/- 429 days. In no case did the bow-tie suture rupture. CONCLUSIONS: Edge-to-edge mitral valve repair is a valuable tool in the armamentarium available to treat complex cases of mitral insufficiency or as an adjunct to standard repair techniques that fail to achieve an acceptable result.

Adolescent↗