Association between malignant mitral valve prolapse and sudden cardiac death: A review

Published on Sep 1, 2020in JAMA Cardiology14.676
· DOI :10.1001/JAMACARDIO.2020.1412
Lakshmi Muthukumar3
Estimated H-index: 3
(UW: University of Wisconsin-Madison),
Arshad Jahangir50
Estimated H-index: 50
(UW: University of Wisconsin-Madison)
+ 3 AuthorsA. Jamil Tajik16
Estimated H-index: 16
(UW: University of Wisconsin-Madison)
Sources
Abstract
Importance Malignant arrhythmic mitral valve prolapse (MVP) phenotype poses a substantial risk of sudden cardiac death (SCD), and an estimated 26 000 individuals in the United States are at risk of SCD per year. Thus, identifying risk-stratification strategies for SCD is imperative. Observations Patients with MVP have a heterogenous clinical spectrum, ranging from a benign course to a devastating complication such as SCD. Some of the high-risk markers of MVP, which are identified electrocardiographically, include inverted or biphasic T waves, QT dispersion, QT prolongation, and premature ventricular contractions originating from the left ventricular outflow tract and papillary muscles. Morphofunctional characteristics of SCD are leaflet thickness of 5 mm or greater, mitral annulus disjunction, paradoxical systolic increase of the mitral annulus diameter, increased tissue Doppler velocity of the mitral annulus, and higher mechanical dispersion on echocardiography and fibrosis identified by late gadolinium enhancement on cardiac magnetic resonance imaging. Conclusions and Relevance Findings from this review suggest that SCD can occur earlier in the course of MVP from complex arrhythmias that are triggered by the repeated tugging and traction of the chordopapillary muscle unit and basal mid-myocardium, even before macrofibrosis can be identified in these regions by late gadolinium enhancement on cardiac magnetic resonance imaging. Some of the newer markers identified by speckle-tracking Doppler, such as mechanical dispersion, myocardial work index, and postsystolic shortening, need further validation in a larger population.
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#1Yasmine L. Hiemstra (LUMC: Leiden University Medical Center)H-Index: 7
#2Aniek L van Wijngaarden (LUMC: Leiden University Medical Center)H-Index: 4
Last. Nina Ajmone Marsan (LUMC: Leiden University Medical Center)H-Index: 54
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BackgroundInitial studies have suggested the familial clustering of mitral valve prolapse, but most of them were either community based among unselected individuals or applied non-specific diagnost...
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#1Cristina Basso (UNIPD: University of Padua)H-Index: 109
#2Sabino Iliceto (UNIPD: University of Padua)H-Index: 68
Last. Martina Perazzolo Marra (UNIPD: University of Padua)H-Index: 33
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Despite a 2% to 3% prevalence of echocardiographically defined mitral valve prolapse (MVP) in the general population, the actual burden, risk stratification, and treatment of the so-called arrhythm...
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#1Barry J. Maron (Tufts Medical Center)H-Index: 178
#2Ethan J. Rowin (Tufts Medical Center)H-Index: 27
Last. Martin S. Maron (Tufts Medical Center)H-Index: 73
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Hypertrophic cardiomyopathy (HCM) is a worldwide genetic heart disease and a common cause of sudden death in the young. Penetration of the implantable cardioverter-defibrillator (ICD) into this pat...
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#1Roberta Manganaro (University of Liège)H-Index: 4
#2Stella Marchetta (University of Liège)H-Index: 9
Last. Patrizio Lancellotti (University of Liège)H-Index: 111
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Aims - To obtain the normal ranges for 2D echocardiographic (2DE) indices of myocardial work (MW) from a large group of healthy volunteers over a wide range of ages and gender. Methods and results - A total of 226 (85 men, mean age: 45 ± 13 years) healthy subjects were enrolled at 22 collaborating institutions of the Normal Reference Ranges for Echocardiography (NORRE) study. Global work index (GWI), global constructive work (GCW), global work waste (GWW), and global work efficiency (GWE) were e...
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#1Sahej Bains (Mayo Clinic)H-Index: 1
#2David J. Tester (Mayo Clinic)H-Index: 77
Last. John R. Giudicessi (Mayo Clinic)H-Index: 22
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Abstract A 51-year-old man with a long-standing history of bileaflet mitral valve prolapse accompanied by mitral annular disjunction and mild mitral regurgitation presented for evaluation of increasingly frequent palpitations. Ambulatory Holter monitoring, cardiac magnetic resonance imaging, serial transthoracic echocardiography, and diagnostic electrophysiology studies were consistent with a diagnosis of arrhythmogenic bileaflet mitral valve prolapse syndrome. Because of the presence of a simil...
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#1Martina Perazzolo Marra (UNIPD: University of Padua)H-Index: 33
#2Cristina Basso (UNIPD: University of Padua)H-Index: 109
Mitral valve prolapse (MVP) represents the most common degenerative valve disease, mainly characterised by the onset of significant mitral regurgitation. However, a small cohort of patients with MVP presents a potentially ‘malignant’ MVP–ventricular arrhythmia phenotype’.1 Traditionally, the ‘arrhythmic profile’ is that of young women with bileaflet MVP, biphasic or inverted T waves in the inferior leads, and frequent complex ventricular ectopic beats with documented ventricular bigeminy or vent...
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#1Esther ScheirlynckH-Index: 4
#2Lars A. DejgaardH-Index: 9
Last. Kristina H. HaugaaH-Index: 50
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Objective Displacement of the mitral valve, mitral annulus disjunction (MAD), is described as a possible aetiology of sudden cardiac death. Stress-induced fibrosis in the mitral valve apparatus has been suggested as the underlying mechanism. We aimed to explore the association between stretch-related and fibrosis-related biomarkers and ventricular arrhythmias in MAD. We hypothesised that soluble suppression of tumourigenicity-2 (sST2) and transforming growth factor-β1 (TGFβ1) are markers of vent...
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#1Simon Ermakov (UCSF: University of California, San Francisco)H-Index: 7
#2Radhika Gulhar (UCSF: University of California, San Francisco)H-Index: 1
Last. Francesca N. Delling (UCSF: University of California, San Francisco)H-Index: 20
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Objective Bileaflet mitral valve prolapse (MVP) with either focal or diffuse myocardial fibrosis has been linked to ventricular arrhythmia and/or sudden cardiac arrest. Left ventricular (LV) mechanical dispersion by speckle-tracking echocardiography (STE) is a measure of heterogeneity of ventricular contraction previously associated with myocardial fibrosis. The aim of this study is to determine whether mechanical dispersion can identify MVP at higher arrhythmic risk. Methods We identified 32 co...
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#1Jonathan Chan (Griffith University)H-Index: 25
#2N. Edwards (Griffith University)H-Index: 4
Last. Gregory M. Scalia (UQ: University of Queensland)H-Index: 20
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Aims: Non-invasive left ventricular (LV) pressure–strain loop (PSL) provides a novel method of quantifying myocardial work (MW) with potential advantages over conventional global longitudinal strain (GLS) by incorporating measurements of myocardial deformation and LV pressure. We investigated different patterns of LV PSL and global MW index (GWI) in patients with hypertension (HTN) and dilated cardiomyopathy (CMP). Methods and results: Seventy-four patients underwent transthoracic echocardiograp...
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#1Chrishan J. NalliahH-Index: 21
#2Rajiv Mahajan (Royal Adelaide Hospital)H-Index: 32
Last. Prashanthan Sanders (Royal Adelaide Hospital)H-Index: 104
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Objectives Mitral valve prolapse (MVP) is commonly observed as a benign finding. However, the literature suggests that it may be associated with sudden cardiac death (SCD). We performed a meta-analysis and systematic review to determine the: (1) prevalence of MVP in the general population; (2) prevalence of MVP in all SCD and unexplained SCD; (3) incidence of SCD in MVP and (4) risk factors for SCD. Methods The English medical literature was searched for: (1) MVP community prevalence; (2) MVP pr...
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Cited By10
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Abstract null null Objectives null The aim of this study was to assess in patients with mitral valve prolapse (MVP) mitral annular disjunction (MAD) prevalence, phenotypic characteristics, and long-term outcomes (clinical arrhythmic events and excess mortality). null null null Background null Clinical knowledge regarding MAD of MVP remains limited and controversial, and its potential link with untoward outcomes is unsubstantiated. null null null Methods null A cohort of 595 (278 women, mean age ...
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#1Gianna L. Wilkie (UMass: University of Massachusetts Amherst)
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Mitral valve prolapse (MVP) is the most common valvular heart disease in women of reproductive age. Whether MVP increases the likelihood of adverse outcomes in pregnancy is unknown. The study objective was to examine the cardiac and obstetric outcomes associated with MVP in pregnant women. This retrospective cohort study, using the Healthcare Cost and Utilization Project National Readmission Sample database between 2010 and 2017, identified all pregnant women with MVP using the International Cla...
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#1Federico Oliveri (UNIPV: University of Pavia)H-Index: 1
#2Fotios KakargiasH-Index: 1
Last. Pousette Hamid (Ain Shams University Faculty of Medicine)H-Index: 3
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BACKGROUND Although frequently considered a benign condition, new evidence has shown that mitral valve prolapse (MVP) is associated with complex ventricular arrhythmias (VAs) and sudden cardiac death (SCD). Therefore, we conducted a systematic review and meta-analysis of the relevant studies to investigate the parameters that could identify MVP patients at higher risk of experiencing complex VAs. METHOD We performed a systematic literature search of PubMed for potential studies between January 2...
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#1John R. Giudicessi (Mayo Clinic)H-Index: 22
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Abstract null null Background null Mitral valve prolapse (MVP) is largely considered a benign condition. However, MVP is overrepresented consistently in sudden unexplained death in the young (SUDY) cohorts. null null null Objective null To determine the prevalence and potential genetic underpinnings of suspected arrhythmogenic MVP in a referral cohort of SUDY cases. null null null Methods null In this retrospective study, medical records/autopsy reports and whole exome molecular autopsy (WEMA) r...
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Since 1989, SIC Sport and a FMSI, in partnership with leading Italian Cardiological Scientific Associations (ANCE, ANMCO and SIC) have produced Cardiological Guidelines for Completive Sports Eligibility for athletes with heart disease (COCIS -- 1989, 1995, 2003, 2009 and 2017). The English version of the Italian Cardiological Guidelines for Competitive Sports Eligibility for athletes with heart disease was published in 2013 in this Journal. This publication is an update with respect to the docum...
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Mitral valve prolapse is a common cardiac condition, with an estimated prevalence between 1% and 3%. Most patients have a benign course, but ever since its initial description mitral valve prolapse has been associated to sudden cardiac death. Although the causal relationship between mitral valve prolapse and sudden cardiac death has never been clearly demonstrated, different factors have been implicated in arrhythmogenesis in patients with mitral valve prolapse. In this work, we offer a comprehe...
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We determined the frequency of mitral valve prolapse (MVP) in healthy Turkish school children using the current echocardiographic diagnostic criteria. This epidemiological survey was performed on 2550 school children. All children were screened with echocardiography and the family of children with MVP were also screened. The prolapse of mitral leaflets into left atrium ≥ 2 mm in parasternal long-axis view was used as diagnostic criteria. MVP was classified as classical or non-classical according...
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