Left Ventricular Remodeling in Non-syndromic Mitral Valve Prolapse: Volume Overload or Concomitant Cardiomyopathy?

Pype, L; Bertrand, Philippe; Paelinck, Bernard P.; Heidbüchel, Hein; Craenenbroeck, Emeline M. Van; Heyning, Caroline M. Van De

doi:10.3389/fcvm.2022.862044

Cited by 11 publications

(13 citation statements)

References 124 publications

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“…Myxomatous degeneration, also known as Barlow’s disease, associates bileaflet MVP, marked annular dilatation, frequent mitral annulus disjunction, and disproportionate left ventricular (LV) dilatation ( 44 , 45 ) even in the absence of significant MR ( 46 ). Several hypotheses have been suggested to explain the intriguing disproportionate LV dilatation associated to myxomatous MVP, namely (1) an underestimation of volume overload linked to the pseudoaneurysmal prolapse sequestering a significant volume of blood ( 9 , 47 ), (2) a compensatory mechanism to frequent ventricular ectopic beats ( 48 ), and (3) a unique type of associated cardiomyopathy ( 44 ) which could be linked to a genetic background.…”

Section: A Genetic Association Between Mvp and Cardiomyopathy?mentioning

confidence: 99%

Genetics and pathophysiology of mitral valve prolapse

Delwarde

Capoulade²,

Mérot³

et al. 2023

Front. Cardiovasc. Med.

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Mitral valve prolapse (MVP) is a common condition affecting 2–3% of the general population, and the most complex form of valve pathology, with a complication rate up to 10–15% per year in advanced stages. Complications include mitral regurgitation which can lead to heart failure and atrial fibrillation, but also life-threatening ventricular arrhythmia and cardiovascular death. Sudden death has been recently brought to the forefront of MVP disease, increasing the complexity of management and suggesting that MVP condition is not properly understood. MVP can occur as part of syndromic conditions such as Marfan syndrome, but the most common form is non-syndromic, isolated or familial. Although a specific X-linked form of MVP was initially identified, autosomal dominant inheritance appears to be the primary mode of transmission. MVP can be stratified into myxomatous degeneration (Barlow), fibroelastic deficiency, and Filamin A-related MVP. While FED is still considered a degenerative disease associated with aging, myxomatous MVP and FlnA-MVP are recognized as familial pathologies. Deciphering genetic defects associated to MVP is still a work in progress; although FLNA, DCHS1, and DZIP1 have been identified as causative genes in myxomatous forms of MVP thanks to familial approaches, they explain only a small proportion of MVP. In addition, genome-wide association studies have revealed the important role of common variants in the development of MVP, in agreement with the high prevalence of this condition in the population. Furthermore, a potential genetic link between MVP and ventricular arrhythmia or a specific type of cardiomyopathy is considered. Animal models that allow to advance in the genetic and pathophysiological knowledge of MVP, and in particular those that can be easily manipulated to express a genetic defect identified in humans are detailed. Corroborated by genetic data and animal models, the main pathophysiological pathways of MVP are briefly addressed. Finally, genetic counseling is considered in the context of MVP.

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Section: A Genetic Association Between Mvp and Cardiomyopathy?mentioning

confidence: 99%

Genetics and pathophysiology of mitral valve prolapse

Delwarde

Capoulade²,

Mérot³

et al. 2023

Front. Cardiovasc. Med.

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“…Late gadolinium enhancement (LGE)-CMR has a high sensitivity, specificity and reproducibility to assess focal myocardial replacement fibrosis by a relative accumulation of gadolinium in the extracellular matrix, but is not sensitive for identifying reactive interstitial fibrosis ( 13 , 32 – 34 ). Furthermore, CMR T1 mapping is suitable to quantify diffuse interstitial fibrosis by increased extracellular volume (ECV) expansion and seems useful to detect an early stage of fibrosis ( 13 , 31 , 35 ).…”

Section: Cmr-based Quantification Of Fibrosis In Mvp Patientsmentioning

confidence: 99%

“…Such fibrosis is clinically relevant because of a demonstrated association with ventricular arrhythmias and sudden cardiac death (SCD) ( 7 , 9 , 12 ). Cardiovascular imaging is the clinical noninvasive method used to detect myocardial fibrosis ( 13 ), while histological assessment is the validation standard for its detection and quantification ( 14 , 15 ). To provide a clinical benefit for MVP patients in the future, three steps are recommended for preclinical and clinical research: (i) improvement and refinement in describing fibrosis, (ii) improvement in detecting fibrosis, and (iii) targeting fibrosis by surgical or pharmacological treatment ( 16 ).…”

Section: Introductionmentioning

confidence: 99%

Histopathological insights into mitral valve prolapse-induced fibrosis

Dieterlen

Klaeske²,

Spampinato³

et al. 2023

Front. Cardiovasc. Med.

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Mitral valve prolapse (MVP) is a cardiac valve disease that not only affects the mitral valve (MV), provoking mitral regurgitation, but also leads to maladaptive structural changes in the heart. Such structural changes include the formation of left ventricular (LV) regionalized fibrosis, especially affecting the papillary muscles and inferobasal LV wall. The occurrence of regional fibrosis in MVP patients is hypothesized to be a consequence of increased mechanical stress on the papillary muscles and surrounding myocardium during systole and altered mitral annular motion. These mechanisms appear to induce fibrosis in valve-linked regions, independent of volume-overload remodeling effects of mitral regurgitation. In clinical practice, quantification of myocardial fibrosis is performed with cardiovascular magnetic resonance (CMR) imaging, even though CMR has sensitivity limitations in detecting myocardial fibrosis, especially in detecting interstitial fibrosis. Regional LV fibrosis is clinically relevant because even in the absence of mitral regurgitation, it has been associated with ventricular arrhythmias and sudden cardiac death in MVP patients. Myocardial fibrosis may also be associated with LV dysfunction following MV surgery. The current article provides an overview of current histopathological studies investigating LV fibrosis and remodeling in MVP patients. In addition, we elucidate the ability of histopathological studies to quantify fibrotic remodeling in MVP and gain deeper understanding of the pathophysiological processes. Furthermore, molecular changes such as alterations in collagen expression in MVP patients are reviewed.

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“…three components of left atrial function are (95% confidence intervals): for reservoir strain 38%-41%, for conduit strain 21%-25% and for the contractile strain of 16%-19%. [35][36][37] The evaluation of LA function by MDI (especially, LA reservoir function) measured as peak atrial longitudinal strain (PALS) is recommended even before gross anatomical changes occur (Figure 6). The reservoir function of LA depends on atrial compliance, which diminishes with interstitial fibrosis.…”

Section: Left Atrial Function In Primary Mr: Why and How To Assess It...mentioning

confidence: 99%

“…Even before gross anatomical changes, there are pieces of the evidence of the ultrastructural alterations which could indirectly be discovered by MDI and LA strain. The normal reference ranges for the three components of left atrial function are (95% confidence intervals): for reservoir strain 38%–41%, for conduit strain 21%–25% and for the contractile strain of 16%–19% 35–37 …”

Section: Introductionmentioning

confidence: 99%

Multimodality imaging for the management of patients with primary mitral regurgitation

Vratonjic¹,

Jovanovic²,

Petrović

et al. 2022

J of Clinical Ultrasound

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Advanced cardiac imaging (ACI), including myocardial deformation imaging, 3D echocardiography and cardiac magnetic resonance, overcomes the limitations of conventional echocardiography in the assessment of patients with primary mitral regurgitation (MR). They enable a more precise MR quantification and reveal early changes before advanced and irreversible remodeling with depressed heart function occurs. ACI permits a thorough analysis of mitral valvular anatomy and MR mechanisms (important for planning and guiding percutaneous and surgical procedures) and helps to identify structural and functional changes coupled with a high arrhythmogenic potential, especially the occurrence of atrial fibrillation and heart failure development. The key question is how the data provided by ACI can improve the current management of primary MR.

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Left Ventricular Remodeling in Non-syndromic Mitral Valve Prolapse: Volume Overload or Concomitant Cardiomyopathy?

Cited by 11 publications

References 124 publications

Genetics and pathophysiology of mitral valve prolapse

Genetics and pathophysiology of mitral valve prolapse

Histopathological insights into mitral valve prolapse-induced fibrosis

Multimodality imaging for the management of patients with primary mitral regurgitation

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