Arrhythmic mitral valve prolapse in 2023: Evidence-based update

Patients with mitral valve prolapse (MVP) may develop ventricular arrhythmias, ranging from premature ventricular contractions through more complex non-sustained ventricular tachycardia to sustained life-threatening ventricular arrhythmias. The prevalence of MVP in autopsy series of young adults who died suddenly has been estimated to be between 4% and 7%. Thus, “arrhythmic MVP” has been reported as an underappreciated cause of sudden cardiac death, leading to a renewed interest in the study of this association. The term “arrhythmic MVP” refers to a small subset of patients who have, in the absence of any other arrhythmic substrate, MVP, with or without mitral annular disjunction, and frequent or complex ventricular arrhythmias. Our understanding of their coexistence in terms of contemporary management and prognosis is still incomplete. While literature regarding the arrhythmic MVP may be contrasting despite recent consensus document, the present review summarizes the relevant evidence concerning the diagnostic approach, prognostic implications, and targeted therapies for MVP-related ventricular arrhythmias. We also summarize recent data supporting left ventricular remodeling, which complicates the coexistence of MVP with ventricular arrhythmias. As the evidence for a putative link between MVP-associated ventricular arrhythmias and sudden cardiac death is scarce and based on scant and retrospective data, risk prediction remains a challenge. Thus, we aimed at listing potential risk factors from available seminal reports for further use in a more reliable prediction model that requires additional prospective data. Finally, we summarize evidence and guidelines on targeted therapies of ventricular arrhythmias in the setting of MVP, including implantable cardioverter defibrillators and catheter ablation. Our review highlights current knowledge gaps and provides an action plan for structured research on the pathophysiological genesis, diagnosis, prognostic impact, and optimal management of patients with arrhythmic MVP

Watchful surgery in asymptomatic mitral valve prolapse

The most common organic etiology of mitral regurgitation is degenerative and consists of mitral valve prolapse (MVP). Volume overload because of mitral regurgitation is the most common complication of MVP. Advocating surgery before the consequences of volume overload become irreparable restores life expectancy, but carries a risk of mortality in patients who are often asymptomatic. On the other hand, the post-surgical outcome of symptomatic patients is dismal and life expectancy is impaired. In the present article, we aim to bridge the gap between these two therapeutic approaches, unifying the concepts of watchful waiting and early surgery in a “watchful surgery approach”

Medical and surgical outcome of tricuspid regurgitation caused by flail leaflets

AbstractObjectiveWe sought to evaluate the medical and surgical outcome of tricuspid regurgitation caused by flail leaflets.MethodsWe analyzed the cause, clinical presentation, outcome, and natural history of 60 patients with tricuspid regurgitation caused by flail leaflets, a cause of mostly severe and organic tricuspid regurgitation, diagnosed by means of echocardiography between 1980 and 2000.ResultsThe main cause was traumatic (62%). Clinical presentation was often severe: 57% were symptomatic, 33% had a history of congestive heart failure, and 40% had a history of atrial fibrillation. Compared with expected survival of the US matched population, excess mortality (39% ± 10% at 10 years or 4.5% yearly, P < .01) was observed. Even patients asymptomatic at presentation experienced high tricuspid-related event rates (at 10 years, 75% ± 15% had symptoms or heart failure, atrial fibrillation, surgical intervention, or death). In those patients severe enlargement of right-sided chambers was predictive of poor outcome (at 5 years: 86% ± 9% vs 39% ± 11%, P < .01) independent of cause (P = .31). The poor medical outcome was further confirmed by high event rates (69% ± 9% at 15 years) in the natural history beginning from the flail's occurrence. Tricuspid operations were performed in 33 patients (55% ± 7% at 5 years), with valve repair in 82%, low mortality (3%), and, despite frequently refractory atrial fibrillation, symptomatic improvement in 88%.ConclusionTricuspid regurgitation caused by flail leaflets is a serious disease associated with excess mortality and high morbidity. Tricuspid valve repair can often be performed with low risk, allowing symptomatic improvement. These results suggest that surgical intervention should be considered early in the course of the disease before the occurrence of irreversible consequences

Use of echocardiography in the management of congestive heart failure in the community

AbstractObjectives. We evaluated the use and the impact of echocardiography in patients receiving an initial diagnosis of congestive heart failure in Olmsted County, Minnesota, in 1991.Background. The American College of Cardiology/American Heart Association clinical practice guidelines recommend echocardiography in all patients with suspected congestive heart failure. No data are available on use and impact of echocardiography in management of congestive heart failure in a community.Methods. The medical records linkage system of the Rochester Epidemiology Project was used to identify all 216 patients who satisfied the Framingham criteria for congestive heart failure. Of these, 137 (63%) underwent echocardiography within 3 weeks before or after the episode of congestive heart failure (Echo group), and the other 79 patients constitute the No-Echo group.Results. The No-Echo group patients were older (p = 0.022), were more likely to be female (p = 0.072), had milder symptoms (p = 0.001) and were less often hospitalized at diagnosis (p = 0.001). Fewer patients in the No-Echo group were treated with angiotensin-converting enzyme inhibitors (p = 0.001). Advanced age (≥80 years), lower New York Heart Association functional class, absence of a fourth heart sound on examination, absence of cardiomegaly or signs of congestive heart failure on chest radiography and absence of known valve disease were independently related to the decision not to obtain an echocardiogram. Survival after adjustment for age, functional class and gender was lower in the No-Echo group than the Echo group (risk ratio = 0.607, p = 0.017).Conclusions. The underuse of echocardiography appears to be associated with poorer survival and underuse of angiotensin-converting enzyme inhibitor therapy

Prospective comparison of speckle tracking longitudinal bidimensional strain between two vendors

SummaryBackgroundSpeckle tracking is a relatively new, largely angle-independent technique used for the evaluation of myocardial longitudinal strain (LS). However, significant differences have been reported between LS values obtained by speckle tracking with the first generation of software products.AimsTo compare LS values obtained with the most recently released equipment from two manufacturers.MethodsSystematic scanning with head-to-head acquisition with no modification of the patient's position was performed in 64 patients with equipment from two different manufacturers, with subsequent off-line post-processing for speckle tracking LS assessment (Philips QLAB 9.0 and General Electric [GE] EchoPAC BT12). The interobserver variability of each software product was tested on a randomly selected set of 20 echocardiograms from the study population.ResultsGE and Philips interobserver coefficients of variation (CVs) for global LS (GLS) were 6.63% and 5.87%, respectively, indicating good reproducibility. Reproducibility was very variable for regional and segmental LS values, with CVs ranging from 7.58% to 49.21% with both software products. The concordance correlation coefficient (CCC) between GLS values was high at 0.95, indicating substantial agreement between the two methods. While good agreement was observed between midwall and apical regional strains with the two software products, basal regional strains were poorly correlated. The agreement between the two software products at a segmental level was very variable; the highest correlation was obtained for the apical cap (CCC 0.90) and the poorest for basal segments (CCC range 0.31–0.56).ConclusionsA high level of agreement and reproducibility for global but not for basal regional or segmental LS was found with two vendor-dependent software products. This finding may help to reinforce clinical acceptance of GLS in everyday clinical practice

Arrhythmias in Patients With Valvular Heart Disease: Gaps in Knowledge and the Way Forward.

peer reviewedThe prevalence of both organic valvular heart disease (VHD) and cardiac arrhythmias is high in the general population, and their coexistence is common. Both VHD and arrhythmias in the elderly lead to an elevated risk of hospitalization and use of health services. However, the relationships of the two conditions is not fully understood and our understanding of their coexistence in terms of contemporary management and prognosis is still limited. VHD-induced left ventricular dysfunction/hypertrophy and left atrial dilation lead to both atrial and ventricular arrhythmias. On the other hand, arrhythmias can be considered as an independent condition resulting from a coexisting ischemic or non-ischemic substrate or idiopathic ectopy. Both atrial and ventricular VHD-induced arrhythmias may contribute to clinical worsening and be a turning point in the natural history of VHD. Symptoms developed in patients with VHD are not specific and may be attributable to hemodynamical consequences of valve disease but also to other cardiac conditions including arrhythmias which are notably prevalent in this population. The issue how to distinguish symptoms related to VHD from those related to atrial fibrillation (AF) during decision making process remains challenging. Moreover, AF is a traditional limit of echocardiography and an important source of errors in assessment of the severity of VHD. Despite recent progress in understanding the pathophysiology and prognosis of postoperative AF, many questions remain regarding its prevention and management. Furthermore, life-threatening ventricular arrhythmias can predispose patients with VHD to sudden cardiac death. Evidence for a putative link between arrhythmias and outcome in VHD is growing but available data on targeted therapies for VHD-related arrhythmias, including monitoring and catheter ablation, is scarce. Despite growing evidences, more research focused on the prognosis and optimal management of VHD-related arrhythmias is still required. We aimed to review the current evidence and identify gaps in knowledge about the prevalence, prognostic considerations, and treatment of atrial and ventricular arrhythmias in common subtypes of organic VHD

Outcomes in Mitral Regurgitation Due to Flail Leaflets. A Multicenter European Study

Objectives: The purpose of this study was to assess incidence and predictors of events associated with nonsurgical and surgical management of severe mitral regurgitation (MR) in European institutions. Background: The management of patients with MR remains disputed, warranting multicenter studies to define clinical outcome in routine clinical practice. Methods: The MIDA (Mitral Regurgitation International DAtabase) is a registry created for multicenter study of MR with echocardiographically diagnosed flail leaflet as a model of pure, organic MR. Our cases were collected from 4 European centers. We enrolled 394 patients (age 64 ± 11 years; 67% men; 64% in New York Heart Association functional class I to II; left ventricular ejection fraction 67 ± 10%). Results: During a median follow-up of 3.9 years, linearized event rates/year under nonsurgical management were 5.4% for atrial fibrillation (AF), 8.0% for heart failure (HF), and 2.6% for death. Mitral valve (MV) surgery was performed in 315 (80%) patients (repair in 250 of 315, 80%). Perioperative mortality, defined as death within 30 days from the operation, was 0.7% (n = 2). Surgery during follow-up was independently associated with reduced risk of death (adjusted hazard ratio [HR] 0.42, 95% confidence interval [CI] 0.21 to 0.84; p = 0.014). Benefit was largely driven by MV repair (adjusted HR vs. replacement 0.37, 95% CI 0.18 to 0.76; p = 0.007). In 102 patients strictly asymptomatic and with normal ventricular function, 5-year combined incidence of AF, HF, or cardiovascular death (CVD) was 42 ± 8%. In these patients, surgery also reduced rates of CVD/HF (HR 0.26, 95% CI 0.08 to 0.89; p = 0.032). Conclusions: In this multicenter study, nonsurgical management of severe MR was associated with notable rates of adverse events. Surgery especially MV repair performed during follow-up was beneficial in reducing rates of cardiac events. These findings support surgical consideration in patients with MR due to flail leaflets for whom MV repair is feasible. © 2008 American College of Cardiology Foundation

Increased risk of left heart valve regurgitation associated with benfluorex use in patients with diabetes mellitus: a multicenter study.

International audienceBACKGROUND: Benfluorex was withdrawn from European markets in June 2010 after reports of an association with heart valve lesions. The link between benfluorex and valve regurgitations was based on small observational studies and retrospective estimations. We therefore designed an echocardiography-based multicenter study to compare the frequency of left heart valve regurgitations in diabetic patients exposed to benfluorex for at least 3 months and in diabetic control subjects never exposed to the drug. METHODS AND RESULTS: This reader-blinded, controlled study conducted in 10 centers in France between February 2010 and September 2011 prospectively included 376 diabetic subjects previously exposed to benfluorex who were referred by primary care physicians for echocardiography and 376 diabetic control subjects. Through the use of propensity scores, 293 patients and 293 control subjects were matched for age, sex, body mass index, smoking, dyslipidemia, hypertension, and coronary artery disease. The main outcome measure was the frequency of mild or greater left heart valve regurgitations. In the matched sample, the frequency and relative risk (odds ratio) of mild or greater left heart valve regurgitations were significantly increased in benfluorex patients compared with control subjects: 31.0% versus 12.9% (odds ratio, 3.55; 95% confidence interval, 2.03-6.21) for aortic and/or mitral regurgitation, 19.8% versus 4.7% (odds ratio, 5.29; 95% confidence interval, 2.46-11.4) for aortic regurgitation, and 19.4% versus 9.6% (odds ratio, 2.38; 95% confidence interval, 1.27-4.45) for mitral regurgitation. CONCLUSIONS: Our results indicate that the use of benfluorex is associated with a significant increase in the frequency of left heart valve regurgitations in diabetic patients. The natural history of benfluorex-induced valve abnormalities needs further research

Functional anatomy of mitral regurgitation Accuracy and outcome implications of transesophageal echocardiography

AbstractOBJECTIVESThis study was performed to determine the accuracy and outcome implications of mitral regurgitant lesions assessed by echocardiography.BACKGROUNDIn patients with mitral regurgitation (MR), valve repair is a major incentive to early surgery and is decided on the basis of the anatomic mitral lesions. These lesions can be observed easily with transesophageal echocardiography (TEE), but the accuracy and implications for outcome and clinical decision-making of these observations are unknown.METHODSIn 248 consecutive patients operated on for MR, the anatomic lesions diagnosed with TEE were compared with those observed by the surgeon and those seen on 216 transthoracic echocardiographic (TTE) studies, and their relationship to postoperative outcome was determined.RESULTSCompared with surgical diagnosis, the accuracy of TEE was high: 99% for cause and mechanism, presence of vegetations and prolapsed or flail segment, and 88% for ruptured chordae. Diagnostic accuracy was higher for TEE than TTE for all end points (p < 0.001), but the difference was of low magnitude (<10%) except for mediocre TTE imaging or flail leaflets (both p < 0.001). The type of mitral lesions identified by TEE (floppy valve, restricted motion, functional lesion) were determinants of valve repairability and postoperative outcome (operative mortality and long-term survival; all p < 0.001) independent of age, gender, ejection fraction and presence of coronary artery disease.CONCLUSIONSTransesophageal echocardiography provides a highly accurate anatomic assessment of all types of MR lesions and has incremental diagnostic value if TTE is inconclusive. The functional anatomy of MR defined by TEE is strongly and independently predictive of valve repairability and postoperative outcome. Therefore, the mitral lesions assessed by echocardiography represent essential information for clinical decision making, particularly for the indication of early surgery for MR

Clinical and prognostic implications of phenomapping in patients with heart failure receiving cardiac resynchronization therapy

Despite having an indication for cardiac resynchronization therapy according to current guidelines, patients with heart failure with reduced ejection fraction who receive cardiac resynchronization therapy do not consistently derive benefit from it.; To determine whether unsupervised clustering analysis (phenomapping) can identify distinct phenogroups of patients with differential outcomes among cardiac resynchronization therapy recipients from routine clinical practice.; We used unsupervised hierarchical cluster analysis of phenotypic data after data reduction (55 clinical, biological and echocardiographic variables) to define new phenogroups among 328 patients with heart failure with reduced ejection fraction from routine clinical practice enrolled before cardiac resynchronization therapy. Clinical outcomes and cardiac resynchronization therapy response rate were studied according to phenogroups.; Although all patients met the recommended criteria for cardiac resynchronization therapy implantation, phenomapping analysis classified study participants into four phenogroups that differed distinctively in clinical, biological, electrocardiographic and echocardiographic characteristics and outcomes. Patients from phenogroups 1 and 2 had the most improved outcome in terms of mortality, associated with cardiac resynchronization therapy response rates of 81% and 78%, respectively. In contrast, patients from phenogroups 3 and 4 had cardiac resynchronization therapy response rates of 39% and 59%, respectively, and the worst outcome, with a considerably increased risk of mortality compared with patients from phenogroup 1 (hazard ratio 3.23, 95% confidence interval 1.9-5.5 and hazard ratio 2.49, 95% confidence interval 1.38-4.50, respectively).; Among patients with heart failure with reduced ejection fraction with an indication for cardiac resynchronization therapy from routine clinical practice, phenomapping identifies subgroups of patients with differential clinical, biological and echocardiographic features strongly linked to divergent outcomes and responses to cardiac resynchronization therapy. This approach may help to identify patients who will derive most benefit from cardiac resynchronization therapy in "individualized" clinical practice