Varied Extent of Mitral Annular Disjunction Among Cases With Different Phenotypes of Mitral Valve Prolapse

Toh, Hiroyuki; Mori, Shigeo; Izawa, Yu; Toba, Takayoshi; Watanabe, Yoshiaki; Kono, Atsushi K.; Hirata, Ken–ichi

doi:10.1016/j.jaccas.2021.06.038

Cited by 4 publications

(4 citation statements)

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“…Height and extent of MAD as well as mitral annulus dilatation in different MVP phenotypes were described by Toh et al. using cardiac CT 52 . Although TTE is less accurate than cardiac CT, the combination of 3D TTE and 2D TTE can provide a reliable extent of annular diameter during the cardiac cycle in addition to MAD evaluation.…”

Section: Complex Ventricular Arrhythmias Sudden Cardiac Arrest and Mvpmentioning

confidence: 99%

“…19 This induces an accelerated degenerative process of the mitral valve and apparatus and localized fibrosis of the myocardium. Height and extent of MAD as well as mitral annulus dilatation in different MVP phenotypes were described by Toh et al using cardiac CT. 52 Although TTE is less accurate than cardiac CT, the combination of 3D TTE and 2D TTE can provide a reliable extent of annular diameter during the cardiac cycle in addition to MAD evaluation. 3D TTE allows visualization of the circumferential extension of MAD using the multi-slice option with an output quite similar to CMR (Figures 5 and 6).…”

Section: Mitral Annular Contraction In Mvpmentioning

confidence: 99%

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Transthoracic echocardiography for arrhythmic mitral valve prolapse: Phenotypic characterization as first step

et al. 2022

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Mitral valve prolapse (MVP) is the most frequent valvulopathy with a prevalence of 1.2%–2.4% in general population and it is characterized by a benign course. Although it can be associated with some complications, ventricular arrhythmias (VA) and sudden cardiac death (SCD) as ultimate expressions, are the most worrying. The estimated risk of SCD in MVP is between 0.2% and 1.9% per year including both MVP patients with left ventricular (LV) dysfunction due to severe MR and MVP patients without significant MR. The latter ones constitute a particular phenotype called “malignant MVP” characterized by bileaflet myxomatous prolapse, ECG repolarization abnormalities and complex VAs (c‐VAs) with polymorphic/right bundle branch block morphology (RBBB) and LV fibrosis of the papillary muscles (PMs) and inferobasal wall secondary to mechanical stretching visualized as late gadolinium enhancement (LGE) areas by cardiac magnetic resonance (CMR). In MVP, the first diagnostic approach is transthoracic echocardiography (TTE) that defines the presence of mitral annular disjunction (MAD) which seems to be associated with “arrhythmic MVP” (AMVP). From an ECG point of view, AMVP is characterized by frequent premature ventricular contractions (PVCs) arising from one or both PMs, fascicular tissue, and outflow tract, as well as by T‐wave inversion in the inferolateral leads. The aim of the present paper is to describe TTE red flags that could identify MVP patients at high risk to develop complex arrhythmias as supported by the corresponding findings of LGE‐CMR and anatomy studies. TTE could be a co‐partner in phenotyping high‐risk arrhythmic MVP patients.

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Section: Complex Ventricular Arrhythmias Sudden Cardiac Arrest and Mvpmentioning

confidence: 99%

Section: Mitral Annular Contraction In Mvpmentioning

confidence: 99%

Transthoracic echocardiography for arrhythmic mitral valve prolapse: Phenotypic characterization as first step

et al. 2022

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“…Recognition of MAD syndrome with myocardial fibrosis is of major importance, as it has been correlated with malignant arrhythmias and sudden cardiac death ( 4 , 17 ). Patients with myxomatous mitral valve disease should be investigated for the presence of MAD ( 18 ). This information is also important when planning surgical repair for such patients, as the surgeon may need to use longer stitches and stabilize the annulus ring close to the posterior wall of the left ventricle ( 19 , 20 ).…”

Section: Discussionmentioning

confidence: 99%

The added value of three-dimensional transthoracic echocardiography in mitral annular disjunction: a case report

Papadopoulos,

Ikonomidis,

Vannan

2024

Front. Cardiovasc. Med.

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BackgroundMitral annular disjunction (MAD) refers to the arrhythmic mitral valve prolapse (MVP) syndrome associated with ventricular arrhythmias and sudden cardiac death. Although the pathophysiology of this disease is still under investigation, specific imaging criteria that establish the diagnosis have been recognized. In this article, we demonstrate most of these criteria using three-dimensional transthoracic echocardiography (3D-TTE) and provide added value in the management of MAD syndrome.Case presentationA 50-year-old male patient with recent syncope and a history of mitral regurgitation (MR) and MAD was admitted to our clinic for further investigation. According to our protocol, the patient underwent a complete 3D-TTE, laboratory blood exams, and 24 h ambulatory electrocardiogram (ECG). Our investigation confirmed the presence of MAD syndrome with bileaflet prolapse, severe MR, and non-sustained ventricular tachycardia, necessitating an implantable cardioverter defibrillator (ICD) and surgical mitral valve repair. The 3D-TTE analysis of the mitral valve demonstrated mitral annular systolic expansion and systolic flattening of the saddle-shaped annulus and quantified the extent of the disjunction arc. Additionally, four-dimensional (4D) strain analysis of the left ventricle revealed the presence of fibrosis of the posteromedial papillary muscle and basal inferolateral wall, which are variables that are required for the diagnosis and therapeutic management of MAD syndrome.Conclusions3D-TTE and 4D strain offer valuable insights for diagnosing and managing patients with MAD syndrome. This method seems to correlate well with the other imaging modalities and could be included in the management protocol of MAD syndrome.

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“…CCT is another useful modality for the evaluation of mitral valve anatomy and MAD but does not allow tissue characterization or MR severity quantification, so it is rarely utilized. Toh et al [ 66 ] defined different phenotypes of MVP, such as the cases of fibroelastic deficiency and forme fruste of MVP demonstrated less extensive disjunction than Barlow disease. It is worth mentioning that the same authors described the presence of MAD in 96% of patients without heart disease [ 67 ].…”

Section: Definition Of Mvp and Mitral Apparatus Associated Anomaliesmentioning

confidence: 99%

Mitral Valve Prolapse and Sudden Cardiac Death in Athletes at High Risk

Vriz

Landi

Eltayeb

et al. 2023

CCR

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Mitral valve prolapse (MVP) is the most frequent valvulopathy in the general population, with usually a favourable prognosis. Although it can be associated with some complications, ventricular arrhythmias (VA) and sudden cardiac death (SCD) are the most worrying. The estimated risk of SCD in MVP is between 0.2% to 1.9% per year, including MVP patients with and without severe mitral regurgitation (MR). The association between SCD and MVP is expressed by a phenotype called “malignant MVP” characterized by transthoracic echocardiography (TTE) findings such as bileaflet myxomatous prolapse and mitral annulus disjunction (MAD), ECG findings such as repolarization abnormalities, complex ventricular arrhythmias (c-VAs) and LV fibrosis of papillary muscles (PMs) and inferobasal wall visualized by late gadolinium enhancement cardiac magnetic resonance (LGE-CMR). Therefore, attention is raised for patients with “arrhythmic MVP” characterized from an ECG point of view by frequent premature ventricular contractions (PVCs) arising from one or both PMs as well as by T-wave inversion in the inferolateral leads. In athletes, SCD is the most frequent medical cause of death and in young subjects (< 35 years) usually is due to electrical mechanism affecting who has a silent cardiovascular disease and are not considered per se a cause of increased mortality. In MVP, SCD was reported to happen during sports activity or immediately after and valve prolapse was the only pathological aspect detected. The aim of the present paper is to explore the association between SCD and MVP in athletes, focusing attention on ECG, TTE in particular, and CMR findings that could help to identify subjects at high risk for complex arrhythmias and eventually SCD. In addition, it is also examined if sports activity might predispose patients with MVP to develop major arrhythmias.

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Varied Extent of Mitral Annular Disjunction Among Cases With Different Phenotypes of Mitral Valve Prolapse

Cited by 4 publications

References 11 publications

Transthoracic echocardiography for arrhythmic mitral valve prolapse: Phenotypic characterization as first step

Transthoracic echocardiography for arrhythmic mitral valve prolapse: Phenotypic characterization as first step

The added value of three-dimensional transthoracic echocardiography in mitral annular disjunction: a case report

Mitral Valve Prolapse and Sudden Cardiac Death in Athletes at High Risk

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