Diffuse myocardial fibrosis in patients with mitral valve prolapse and ventricular arrhythmia

Background: Mitral valve prolapse (MVP) is a frequent disease that can be complicated by mitral regurgitation (MR), heart failure, arterial embolism, rhythm disorders and death. Left ventricular (LV) replacement myocardial fibrosis, a marker of maladaptive remodeling, has been described in patients with MVP, but the implications of this finding remain scarcely explored. We aimed at assessing the prevalence, pathophysiological and prognostic significance of LV replacement myocardial fibrosis through late gadolinium enhancement (LGE) by cardiac magnetic resonance (CMR) in patients with MVP. Methods: Four hundred patients (53±15 years, 55% male) with MVP (trace to severe MR by echocardiography) from 2 centers, who underwent a comprehensive echocardiography and LGE CMR, were included. Correlates of replacement myocardial fibrosis (LGE+), influence of MR degree, and ventricular arrhythmia were assessed. The primary outcome was a composite of cardiovascular events (cardiac death, heart failure, new-onset atrial fibrillation, arterial embolism, and life-threatening ventricular arrhythmia). Results: Replacement myocardial fibrosis (LGE+) was observed in 110 patients (28%; 91 myocardial wall including 71 basal inferolateral wall, 29 papillary muscle). LGE+ prevalence was 13% in trace-mild MR, 28% in moderate and 37% in severe MR, and was associated with specific features of mitral valve apparatus, more dilated LV and more frequent ventricular arrhythmias (45 vs 26%, P<0.0001). In trace-mild MR, despite the absence of significant volume overload, abnormal LV dilatation was observed in 16% of patients and ventricular arrhythmia in 25%. Correlates of LGE+ in multivariable analysis were LV mass (OR 1.01, 95% CI [1.002-1.017], P=0.009) and moderate-severe MR (OR: 2.28, 95% CI [1.21-4.31], P=0.011). LGE+ was associated with worse 4-year cardiovascular event-free survival (49.6±11.7 in LGE+ vs 73.3±6.5% in LGE-, P<0.0001). In a stepwise multivariable Cox model, MR volume and LGE+ (HR: 2.6 [1.4-4.9], P=0.002) were associated with poor outcome. Conclusions: LV replacement myocardial fibrosis is frequent in patients with MVP, is associated with mitral valve apparatus alteration, more dilated LV, MR grade, ventricular arrhythmia, and is independently associated with cardiovascular events. These findings suggest a MVP-related myocardial disease. Finally, CMR provides additional information to echocardiography in MVP.

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Abstract 16798: Evidence of Abnormal T1 Mapping in Arrhythmic Mitral Valve Prolapse Without Significant Mitral Regurgitation

Circulation ◽

10.1161/circ.142.suppl_3.16798 ◽

2020 ◽

Vol 142 (Suppl_3) ◽

Author(s):

Brian B Agbor-Etang ◽

Lisa J Lim ◽

Karen G Ordovas ◽

Francesca N Delling

Keyword(s):

Late Gadolinium Enhancement ◽

Mitral Valve ◽

Mitral Regurgitation ◽

Myocardial Fibrosis ◽

Mitral Valve Prolapse ◽

Ventricular Arrhythmias ◽

T1 Mapping ◽

Diffuse Myocardial Fibrosis ◽

Diffuse Fibrosis ◽

Gadolinium Enhancement

Background: Prior cardiac magnetic resonance (CMR) studies have reported abnormal T1 mapping, reflective of diffuse myocardial fibrosis, in patients with mitral valve prolapse (MVP) and ventricular arrhythmias. However, T1 mapping was derived from conventional Look-Locker sequences and/or obtained in selected MVP patients with severe mitral regurgitation (MR) and a clinical indication for CMR. Hypothesis: We hypothesize that extracellular volume (ECV) fraction, a marker of diffuse fibrosis derived from research-based, MOLLI T1 mapping sequences, is increased in MVP subjects with ventricular arrhythmias, even in the absence of significant MR. Methods: We performed CMRs in 10 consecutive, randomly selected MVP patients identified through our echocardiographic database, age/gender matched to 10 controls free of significant cardiac disease. All 10 MVPs underwent ambulatory EKG monitoring. CMR images were acquired using a GE 3.0T Discovery MR750w scanner. Global ECV fraction was calculated using pre- and 10 minutes post-contrast T1 times after administration of 0.1 mmol/kg of gadobutrol (Gadavist). Late gadolinium enhancement (LGE) was also obtained. MR fraction was quantified by velocity encoded CMR. Mild MR was defined as MR fraction < 16%. Results: MVP patients had significantly higher ECV fraction compared to controls (mean ECV (%) 32 ± 4 vs 20 ± 6, p = 0.0002), with 5/10 demonstrating non-sustained VT on ambulatory EKG monitoring. The majority (9/10 or 90%) of MVPs had mild or no MR (MR fraction < 16%), and 1/10 or 10% had moderate MR (MR fraction 18%). Only one individual in the MVP group had late gadolinium enhancement (LGE) in the papillary muscles. Conclusion: MVP with ventricular arrhythmias is associated with increased global ECV reflective of diffuse myocardial fibrosis, even in the absence of significant MR or LGE. Our preliminary findings highlight for the first time a primary interstitial derangement in MVP. Larger studies are needed to understand the mechanisms and prognostic significance of primary diffuse fibrosis in MVP.

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Cardiac magnetic resonance evidence of diffuse myocardial fibrosis in patients with mitral valve prolapse

Journal of Cardiovascular Magnetic Resonance ◽

10.1186/1532-429x-17-s1-p337 ◽

2015 ◽

Vol 17 (S1) ◽

Cited By ~ 1

Author(s):

An H Bui ◽

Sébastien Roujol ◽

Murilo Foppa ◽

Kraig V Kissinger ◽

Beth Goddu ◽

...

Keyword(s):

Cardiac Magnetic Resonance ◽

Magnetic Resonance ◽

Mitral Valve ◽

Myocardial Fibrosis ◽

Mitral Valve Prolapse ◽

Diffuse Myocardial Fibrosis

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Replacement myocardial fibrosis in patients with mitral valve prolapse. Relation to mitral regurgitation, ventricular remodeling and arrhythmia

Archives of Cardiovascular Diseases Supplements ◽

10.1016/j.acvdsp.2021.04.028 ◽

2021 ◽

Vol 13 (3) ◽

pp. 250-251

Author(s):

A.L. Constant Dit Beaufils ◽

O. Huttin ◽

A. Jobbe-Duval ◽

C. Cueff ◽

N. Piriou ◽

...

Keyword(s):

Mitral Valve ◽

Mitral Regurgitation ◽

Myocardial Fibrosis ◽

Mitral Valve Prolapse ◽

Ventricular Remodeling

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CARDIAL MANIFESTATIONS OF NON-DIFFERENTIATED CONNECTIVE TISSUE DYSPLASIA AND THEIR INFLUENCE ON PREGNANCY

Scientific digest of association of obstetricians and gynecologists of Ukraine ◽

10.35278/2664-0767.1(47).2021.242663 ◽

2021 ◽

pp. 14-22

Author(s):

V. Yu. DOBRIANSKA ◽

S. M. HERYAK ◽

L. M. MALANCHUK ◽

M. I. SHVED ◽

I. V. KORDA

Keyword(s):

Mitral Valve ◽

Connective Tissue ◽

Ventricular Arrhythmia ◽

Pregnant Women ◽

Mitral Valve Prolapse ◽

Clinical Signs ◽

Clinical Manifestations ◽

Adequate Treatment ◽

Pregnancy And Childbirth ◽

Connective Tissue Dysplasia

Among the visceral manifestations of NDST in pregnant women most often diagnosed mitral valve prolapse (20-25%) that accompanied by more cardiovascular and obstetric complications during pregnancy. It demonstrates the high clinical significance of the problem of connective tissue dysplasia with mitral valve prolaps for pregnancy and requires adequate treatment programs for prevention of complications and management of pregnant women with connective tissue dysplasia. Aim. Determination of the frequency of pregnancy pathology in women with clinical signs of NDST and MVP complicated by extrasystolic arrhythmia. Materials and methods. 138 pregnant women with MVP and concomitant signs of NDST and 54 healthy pregnant women were selected for analysis. Clinical manifestations of NDST, different variants of arrhythmias and the total number of complications of pregnancy and childbirth were evaluated. Results. In pregnant women with clinical signs of NDST and MVP complicated by extrasystolic arrhythmia, cases of frequent sinus extrasystole were significantly more often compared to frequent ventricular arrhythmia (47.8% vs. 18.1%, p<0.001) and cases of combination of frequent sinus extrasystole and ventricular arrhythmia (13.3 % vs. 1.5%, p <0.05). They significantly more often identified both symptoms of arrhythmological nature and symptoms that indicated a violation of autonomic status. The presence of NDST syndrome is more often accompanied by the development of complications of pregnancy and childbirth. These pregnant women have genetic and phenotypic risk factors for the development of pathological pregnancy and childbirth, birth trauma, disability of mother and newborn, which justifies such patients in a separate risk group for individualized programs of the prevention and treatment of visceral (cardiac) manifestations of NDST and possible complications of pregnancy and childbirth. Conclusions. 3.1% of pregnant women are diagnosed with phenotypic signs (stigma) of undifferentiated connective tissue dysplasia, and the most common visceral cardiac manifestation is mitral valve prolapse. The presence of mitral valve prolapse and extrasystolic arrhythmia in pregnant women with NDST is accompanied by significantly more frequent development of pregnancy and childbirth complications in these patients.

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Left ventricular mechanical dispersion predicts arrhythmic risk in mitral valve prolapse

Heart ◽

10.1136/heartjnl-2018-314269 ◽

2019 ◽

Vol 105 (14) ◽

pp. 1063-1069 ◽

Cited By ~ 13

Author(s):

Simon Ermakov ◽

Radhika Gulhar ◽

Lisa Lim ◽

Dwight Bibby ◽

Qizhi Fang ◽

...

Keyword(s):

Mitral Valve ◽

Ejection Fraction ◽

Myocardial Fibrosis ◽

Mitral Valve Prolapse ◽

Longitudinal Strain ◽

Global Longitudinal Strain ◽

Left Ventricular ◽

Peak Strain ◽

Mechanical Dispersion ◽

Lv Ejection Fraction

ObjectiveBileaflet 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.MethodsWe identified 32 consecutive arrhythmic MVPs (A-MVP) with a history of complex ventricular ectopy on Holter/event monitor (n=23) or defibrillator placement (n=9) along with 27 MVPs without arrhythmic complications (NA-MVP) and 39 controls. STE was performed to calculate global longitudinal strain (GLS) as the average peak longitudinal strain from an 18-segment LV model and mechanical dispersion as the SD of the time to peak strain of each segment.ResultsMVPs had significantly higher mechanical dispersion compared with controls (52 vs 42 ms, p=0.005) despite similar LV ejection fraction (62% vs 63%, p=0.42) and GLS (−19.7 vs −21, p=0.045). A-MVP and NA-MVP had similar demographics, LV ejection fraction and GLS (all p>0.05). A-MVP had more bileaflet prolapse (69% vs 44%, p=0.031) with a similar degree of mitral regurgitation (mostly trace or mild in both groups) (p>0.05). A-MVP exhibited greater mechanical dispersion when compared with NA-MVP (59 vs 43 ms, p=0.0002). Mechanical dispersion was the only significant predictor of arrhythmic risk on multivariate analysis (OR 1.1, 95% CI 1.02 to 1.11, p=0.006).ConclusionsSTE-derived mechanical dispersion may help identify MVP patients at higher arrhythmic risk.

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