The Mitral Annular Disjunction of Mitral Valve Prolapse

Essayagh, Benjamin; Sabbag, Avi; Antoine, Clémence; Benfari, Giovanni; Batista, Roberta; Yang, Li-Tan; Maalouf, Joseph F.; Thapa, Prabin; Asirvatham, Samuel J.; Michelena, Héctor I.; Enríquez-Sarano, Maurice

doi:10.1016/j.jcmg.2021.04.029

Cited by 112 publications

(96 citation statements)

References 44 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…MAD is also associated with annular curling. MAD has been associated with VA both in patients without MVP [13] and those with MVP [13,17,21,23]. A meta-analysis reported MVP patients with MAD had a higher risk of VA, with a risk ratio of 1.90 (p < 0.0001) [49].…”

Section: Mitral Annular Disjunction and Annular Curlingmentioning

confidence: 99%

“…More recently, results regarding the effect of mitral valve surgery on ventricular ectopy have been mixed [90,91]. For MAD, medically managed patients had significantly worse arrhythmia-free survival compared with MADfree controls, but MAD patients undergoing mitral surgery had similar arrhythmia-free survival to controls [23]. Surgical cryoablation, often utilized for atrial fibrillation treatment during cardiac surgery, also holds some promise for PVC treatment.…”

Section: Surgical Interventionmentioning

confidence: 99%

“…The displacement distance can be measured non-invasively by echocardiography, cardiac computed tomography (CT), and cardiac magnetic resonance (CMR). In subsequent studies of various cohorts of MVP, MAD has been identified in approximately 15-55% of patients with significant variation based on the different cohorts studied and imaging modalities utilized [19][20][21][22][23][24][25][26][27]. A systematic review found MAD in about 33% of MVP patients [19].…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Arrhythmic Mitral Valve Prolapse and Mitral Annular Disjunction: Clinical Features, Pathophysiology, Risk Stratification, and Management

Chakrabarti

Bogun

Liang

2022

JCDD

View full text Add to dashboard Cite

Mitral valve prolapse (MVP) is a common cause of valvular heart disease. Although many patients with MVP have a benign course, there is increasing recognition of an arrhythmic phenotype associated with ventricular arrhythmias and sudden cardiac death (SCD). Pathophysiologic mechanisms associated with arrhythmias include cardiac fibrosis, mechanical stress induced changes in ventricular refractory periods, as well as electrophysiologic changes in Purkinje fibers. Clinically, a variety of risk factors including demographic, electrocardiographic, and imaging characteristics help to identify patients with MVP at the highest at risk of SCD and arrhythmias. Once identified, recent advances in treatment including device therapy, catheter ablation, and surgical interventions show promising outcomes. In this review, we will summarize the incidence of ventricular arrhythmias and SCD in patients with MVP, the association with mitral annular disjunction, mechanisms of arrhythmogenesis, methods for arrhythmic and SCD risk stratification including findings with multimodality imaging, and treatments for the primary and secondary prevention of SCD.

show abstract

Section: Mitral Annular Disjunction and Annular Curlingmentioning

confidence: 99%

Section: Surgical Interventionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Arrhythmic Mitral Valve Prolapse and Mitral Annular Disjunction: Clinical Features, Pathophysiology, Risk Stratification, and Management

Chakrabarti

Bogun

Liang

2022

JCDD

View full text Add to dashboard Cite

show abstract

“…It represents the hypermobility of the mitral valve apparatus in which there is a paradoxical systolic increase of the mitral valve annulus. MAD has been reported to be present in about 30% of MVP patients and associated with AMVP in several studies, and it has been described as a possible mechanism leading to fibrosis [ 69 ]. It has been shown to be significantly longer in those with LGE on MRI compared to those who did not have LGE on MRI.…”

Section: Echocardiographymentioning

confidence: 99%

Developing a Mechanistic Approach to Sudden Death Prevention in Mitral Valve Prolapse

Kelley

Chaudry

Syed

2022

JCM

View full text Add to dashboard Cite

Sudden cardiac death (SCD) from ventricular fibrillation (VF) can occur in mitral valve prolapse (MVP) in the absence of other comorbidities including mitral regurgitation, heart failure or coronary disease. Although only a small proportion with MVP are at risk, it can affect young, otherwise healthy adults, most commonly premenopausal women, often as the first presentation of MVP. In this review, we discuss arrhythmic mechanisms in MVP and mechanistic approaches for sudden death risk assessment and prevention. We define arrhythmogenic or arrhythmic MVP (AMVP) as MVP associated with complex and frequent ventricular ectopy, and malignant MVP (MMVP) as MVP with high risk of SCD. Factors predisposing to AMVP are myxomatous, bileaflet MVP and mitral annular disjunction (MAD). Data from autopsy, cardiac imaging and electrophysiological studies suggest that ectopy in AMVP is due to inflammation, fibrosis and scarring within the left ventricular (LV) base, LV papillary muscles and Purkinje tissue. Postulated mechanisms include repetitive injury to these regions from systolic papillary muscle stretch and abrupt mitral annular dysmotility (excursion and curling) and diastolic endocardial interaction of redundant mitral leaflets and chordae. Whereas AMVP is seen relatively commonly (up to 30%) in those with MVP, MVP-related SCD is rare (2–4%). However, the proportion at risk (i.e., with MMVP) is unknown. The clustering of cardiac morphological and electrophysiological characteristics similar to AMVP in otherwise idiopathic SCD suggests that MMVP arises when specific arrhythmia modulators allow for VF initiation and perpetuation through action potential prolongation, repolarization heterogeneity and Purkinje triggering. Adequately powered prospective studies are needed to assess strategies for identifying MMVP and the primary prevention of SCD, including ICD implantation, sympathetic modulation and early surgical mitral valve repair. Given the low event rate, a collaborative multicenter approach is essential.

show abstract

“…Severe VAs defined as non-sustained VT ≥ 180 beats/min or a proven history of sustained VT/VF are infrequent (9%) but associated with excess mortality subsequent to their diagnosis ( 80 ). Mitral annular disjunction (MAD) in the context of MVP can be diagnosed by cardiac MRI or echocardiography after cardiac arrest ( Figure 1 ) ( 79 , 81 ), and has been suggested a harbinger of such events but recent outcome data demonstrate that MAD is indeed associated with secondary development of arrhythmias but during the first 10 years is not associated with excess mortality and should not in and by itself lead to risky electrophysiological interventions ( 82 ). The link MAD-VA is hypothesized to be secondary to local fibrosis induced by the tension generated by such ample prolapse ( 15 ).…”

Section: Mitral Regurgitationmentioning

confidence: 99%

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

Kubala

Chillou

Bohbot

et al. 2022

Front. Cardiovasc. Med.

Self Cite

View full text Add to dashboard Cite

The 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.

show abstract

The Mitral Annular Disjunction of Mitral Valve Prolapse

Cited by 112 publications

References 44 publications

Arrhythmic Mitral Valve Prolapse and Mitral Annular Disjunction: Clinical Features, Pathophysiology, Risk Stratification, and Management

Arrhythmic Mitral Valve Prolapse and Mitral Annular Disjunction: Clinical Features, Pathophysiology, Risk Stratification, and Management

Developing a Mechanistic Approach to Sudden Death Prevention in Mitral Valve Prolapse

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

Contact Info

Product

Resources

About