Abstract 20364: Successful Ablation of Ventricular Fibrillation in Malignant Bileaflet Mitral Valve Prolapse Syndrome

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Faisal F Syed ◽  
Peter Noseworthy ◽  
Christopher McLeod ◽  
Suraj Kapa ◽  
Siva Mulpuru ◽  
...  
Keyword(s):  
Ventricular Fibrillation ◽  
Mitral Valve ◽  
Papillary Muscle ◽  
Mitral Valve Prolapse ◽  
Left Ventricular ◽  
Beta Blockade ◽  
Successful Ablation ◽  
Procedural Success ◽  
Variable Coupling

Introduction: Although the vast majority of mitral valve prolapse (MVP) is benign, women with bileaflet MVP (biMVP), complex ventricular ectopy (VE), and abnormal T waves may comprise the recently described malignant biMVP syndrome. The mechanism of ventricular arrhythmia is unknown. To further characterize the arrhythmic substrate, we reviewed our center’s ablation experience in 6 biMVP patients with prior cardiac arrest and recurrent ICD shocks for drug refractory ventricular fibrillation (VF). Methods and Results: Six women with biMVP (median age 31.5 [range 24.2 - 58.7] years, EF 65 [45 - 67]%, all ≤moderate mitral regurgitation) experienced 6 (3 - 25) appropriate ICD shocks over 4.8 (2.8 - 10.7) years and underwent index ablation between 2/2007 - 10/2013. All had multiple VE morphologies (median 7 [3 - 24]) with variable coupling intervals but with a predominant VE trigger for the VF. A median 2 (1 - 4) VE foci were ablated. Sites of successful ablation of VF-triggering and other dominant VE were left ventricular papillary muscles [PM] (1 anterior, 1 posterior, 1 both), fascicles (1 anterior, 1 posterior), or both (1 both PM and posterior fascicle). Outflow tract VE was also present and targeted (1 left, 1 right)i. Two underwent repeat ablation (288 and 312 days) for recurrent complex VE without shocks, with different foci to the index ablation (1 posterior fascicle, 1 both fascicles). The VF-triggering VE in all patients was confirmed as originating from within the left fascicular system, which in 3/6 was at a papillary muscle. Acute procedural success was seen in all with no complications to date. A VF storm occurred within 24 hours of ablation in a single patient. At follow-up of a mean 662 (47 - 2099) days, 1 patient received a single shock (p=0.03 vs. preablation). Symptomatic VE was reduced in all; while 3/6 continue Class 1c antiarrhythmics and 5/6 have beta blockade. Conclusion: Malignant biMVP syndrome is characterized by fascicular and papillary muscle PVCs that trigger ventricular fibrillation, yet in all patients, the VE is multifocal. Ablation of at least one focus appears to improve symptoms and reduce shocks.

scholarly journals P302 Forgotten and neglected: LV hypertrophy and LV remodeling as important predictors in patients with mitral valve prolapse

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
D Trifunovic ◽  
O Petrovic ◽  
M Tomic-Dragovic ◽  
I Paunovic ◽  
V Tutus ◽  
...  
Keyword(s):  
Heart Failure ◽  
Mitral Valve ◽  
Mitral Valve Prolapse ◽  
Left Ventricular ◽  
Secondary Outcome ◽  
Multivariable Model ◽  
Chi Square ◽  
Increased Risk ◽  
Lv Mass

Abstract Current ESC guidelines recommends left ventricular (LV) end-systolic diameter (ESD), LV ejection fraction (LV EF), systolic pulmonary arterial pressure (SPAP) as key parameters in a multifactorial treatment algorithm for chronic severe primary MR. However, LV hypertrophy (LVH) and LV remodelling during the process of adaptation to chronic MR can influence further clinical course. Aim of this study was to test whether LVH and distinctive LV geometry are coupled with increased risk for heart failure (HF) development and occurrence of major adverse cardiac event (MACE) among patients with MVP and can they improve power of statistical models for HF and MACE prediction based on parameters supported by the current guidelines. Methods 376 pts diagnosed with mitral valve prolapse (MVP) between 1. January 2014. and 31. December 2017 and with complete medical chart and follow-up data from central echo laboratory in the tertiary health center were enrolled in the study. Four types of LV geometry were identified: Type 1 (normal LV mass with normal geometry), Type 2 (normal LV mass with concentric remodeling), Type 3 (eccentric hypertrophy) and Type 4 (concentric hypertrophy). The primary outcome was HF and secondary outcome was MACE (HF development, myocardial infarction, myocardial revascularisation (both PCI and/or ACBG) and cardiac death). Results The distribution of patients was as follow: 51.2% (Group 1) vs 3.3% (Group 2) vs 41.4 % (Group 3) vs 4.1% (Group 4). In multivariable model the highest OR for HF development after adjustment for age, ESD and LVH, had concentric LVH (OR= 5.361, p= 0.004, 95% CI 1.696-16.648), then EF < 60% (OR= 3.025, p = 0.004, 95% CI 1.427-6.411) and the lowest OR had SPAP > 40 mmHg (OR = 2.274, p = 0.039, 95% 1.43-4.958). Adding LVH significantly increased model’s power to predict HF above traditional parameters (Chi-square from 19.386 to 23.640, p < 0.001; Nagelkerke R square from 0.090 to 0.110), whereas addition of LV geometry increased it even more (Chi-square from 23.640 to 28.729, p < 0.001; Negelkerke R square from 0.110 to 0.132). Independent MACE predictors in multivariable model were: EF < 60% (OR 3.645, p < 0.001, 95% CI 1.808- 7.50), new onset atrial fibrillation during the follow-up (OR =3.327, p = 0.012, 95% CI 0.305-8.484), concentric LVH (OR= 4.241, p = 0.015, 95% CI 1.327-13.550) and normal LV geometry without LVH (OR= 0.514, p = 0.002, 95% CI 0.288-0.918), even after adjustment for MV surgery. Adding LVH significantly improved model’s power (Chi-square from 29.026 to 35.112, p < 0.001; Nagelkerke R square 0.121 to 0.146) to predict MACE and addition of type of LV geometry provided additional strength (Chi-square from 35.112 to 39.707, p < 0.001; Nagelkerke R square from 0.146 to 0.164). Conclusion LVH and especially concentric LVH are independent predictors of heart failure development and MACE in mitral valve prolapse and significantly improves predictive powers of the models based on traditional parameters.

Abstract 314: Sensory Innervation of the Papillary Muscle: A Neural Link Between Mitral Valve Prolapse and Malignant Ventricular Arrhythmias?

2016 ◽  
Vol 119 (suppl_1) ◽  
Author(s):  
Krishan J Patel ◽  
Olujimi A Ajijola ◽  
Michael Fishbein ◽  
Kalyanam Shivkumar
Keyword(s):  
Mitral Valve ◽  
Papillary Muscle ◽  
Mitral Valve Prolapse ◽  
Ventricular Arrhythmias ◽  
Left Ventricular ◽  
Sensory Innervation ◽  
Papillary Muscles ◽  
Neuronal Marker ◽  
Significant Difference ◽  
Afferent Innervation

Background: Malignant mitral valve prolapse (MVP) identifies a subset of patients with MVP associated with ventricular arrhythmias (VAs), including sudden cardiac death (SCD). We hypothesized that papillary muscles, which see significant mechanical stress in MVP, are richly innervated with sensory nerves, which induce severe autonomic imbalance on the heart and may trigger malignant VAs. Methods and Results: Longitudinal sections of the anterior and posterior left ventricular papillary muscles, right ventricular outflow tract (RVOT), ventricular septum, and basolateral left ventricular wall (LV) of Yorkshire pigs (n=8) were excised, formalin-fixed, paraffin-embedded, and sectioned. Immuno-staining for protein gene product 9.5 (PGP9.5), a pan-neuronal marker, and calcitonin-gene related peptide (CGRP), a sensory afferent neuron marker, was performed. Areas of immunoreactivity (IR) for CGRP were verified by direct comparison to PGP9.5 IR. The density of CGRP was then compared across anatomical regions. The mean CGRP IR area was 1229.44 ± 116.65 μm 2 /nucleus in papillary muscles, while in the septum, basolateral LV, and RVOT, the CGRP stained areas were 699.36 ± 88.28 μm 2 /nucleus, 681.51 ± 81.90 μm 2 /nucleus, and 381.98 ± 31.14 μm 2 /nucleus, respectively (p<0.001). There was no significant difference between CGRP IR area in the anterior papillary muscle (1091.36 ± 189.82 μm 2 /nucleus) and the posterior papillary muscle (1347.79 ± 140.50 μm 2 /nucleus) (p>0.3). Conclusions: There is a significantly greater amount of afferent innervation in the papillary muscle compared to the septum, basolateral LV, and RVOT. This enrichment of afferent innervation warrants further study to understand how afferent neurotransmission during abnormal mitral valve function may impact ventricular electrophysiology.

scholarly journals Left ventricular remodeling in mitral valve prolapse patients: implications of apical papillary muscle implantation

2021 ◽  
Vol 22 (Supplement_2) ◽  
Author(s):  
S Moura-Ferreira ◽  
B Vandenberk ◽  
PG Masci ◽  
T Dresselaers ◽  
C Garweg ◽  
...  
Keyword(s):  
Mitral Valve ◽  
Papillary Muscle ◽  
Mitral Valve Prolapse ◽  
Ventricular Remodeling ◽  
Left Ventricular Remodeling ◽  
Strong Association ◽  
Lateral Wall ◽  
Left Ventricular ◽  
Angle Variation ◽  
Lv Remodeling

Abstract Funding Acknowledgements Type of funding sources: None. BACKGROUND Mitral valve prolapse (MVP) causes left ventricle (LV) remodeling even in the absence of significant mitral regurgitation. PURPOSE We sought to evaluate whether apical implantation of the papillary muscle (PM) has an influence on the pattern and severity of MVP-related LV remodeling. METHODS All MVP patients who underwent Cardiovascular Magnetic Resonance at our institution between December 2008 and December 2019 were included, thoroughly reviewed and grouped according to apical/non-apical PM implantation. RESULTS Apical PM implantation was found in 53/92 patients (58%) and associated with mitral leaflet thickening (p &lt; 0.01) and a trend toward higher prevalence of mitral annular disjunction (p = 0.05). Whereas there were no differences between groups concerning ventricular volumes and ejection fraction, mitral valve prolapse location or severity of mitral valve insufficiency, patients with apical PM implantation showed more lateral wall remodeling with mid lateral wall thinning (2.1 [1.8-2.5]mm vs. 4.0 [3.5-5.0]mm, p &lt; 0.01), increased LV eccentricity and a lower Global Circumferential Strain at this level (15 ± 3% vs. 20 ± 3%, p &lt; 0.01). In long-axis direction, increased end-diastolic mid lateral wall angulation was found (i.e., angle &lt;155° measured in the thinnest point of the mid lateral wall in 4-chamber view) with a higher angle variation during systole (25 ± 11° vs. 17 ± 8° p &lt; 0.01). Remarkably, PM fibrosis was significantly more frequent in patients with apical PM implantation (i.e., 66% vs. 28%, p &lt; 0.01). Importantly, PM fibrosis was observed in the apically implanted PM in the vast majority of cases (86%), showing a strong association between PM fibrosis and its apical implantation. Finally, a higher burden of premature ventricular complexes (&gt;5%) and non-sustained ventricular tachyarrhythmias was found in patients with apical PM implantation: 53% vs. 25% (p = 0.04) and 38% vs. 18% (p = 0.04), respectively. CONCLUSIONS Apical PM implantation is part of the phenotypic spectrum of MVP, significantly impacts LV remodeling and potentially may be related to increased ventricular arrhythmogenicity.

scholarly journals 1047 Increased interstitial fibrosis in patients with mitral valve prolapse and mitro-annular dysjunction

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
A Pavon ◽  
S Hugelshofer ◽  
T Rutz ◽  
P Pascale ◽  
E Pruvot ◽  
...  
Keyword(s):  
Mitral Valve ◽  
Papillary Muscle ◽  
Mitral Valve Prolapse ◽  
Interstitial Fibrosis ◽  
Extracellular Volume ◽  
Ventricular Myocardium ◽  
Left Ventricular ◽  
Control Group ◽  
Left Ventricular Myocardium ◽  
Inferior Wall

Abstract Background in patients with myxomatous mitral valve prolapse (MVP), mitral annular disjunction (MAD) has been associated with the presence of late gadolinium enhancement (LGE) at papillary muscle level and the risk of sudden cardiac death. However, patients with MAD but no detectable LGE still may have arrhythmia. We investigated the relation between MAD and the presence of interstitial fibrosis in the basal inferior left ventricular myocardium. Methods 28 patient with MVP and associated MAD underwent Cardiovascular Magnetic Resonance imaging (CMR) at 1.5 T scanner (Aera, Siemens Medical Solutions, Erlangen, Germany). Exclusion criteria were ischemic heart disease, infiltrative cardiomyopathy and contraindication to CMR. 12 patients with mitral valve regurgitation but no MAD and 10 patients without mitral disease served as the control group. MAD severity was measured from LA wall-posterior MV leaflet junction to the top of the LV infero-basal wall during end systole. Insterstitial fibrosis was assessed by calculating the extracellular volume (ECV) from T1 mapping of the left ventricular basal slice acquired before and after Gadolinium injection. Results Mean age was 47,5+\-23,3 years and 60% were male. ECV was higher in patients with MVP compared with controls (basal septum: 0.27 ± 0.04 vs 0.23 ± 0.03 p = 0.006; basal inferoposterior wall 0.28 ± 0.03 vs 0.23 ± 0.02 p = 0.003) and there was a significant correlation between MAD severity and ECV of the basal inferior wall (spearman rho 0.68, p &lt; 0.0001) (Figure 1). Among MVP patients, ECV of the basal inferoposterior wall was higher in patients positive for LGE in the papillary muscles (ECV 0.31 ± 0.03 vs 0.27 ± 0.03 p 0,004). Conclusion In MVP patients, MAD severity was associated with a higher amount of interstitial fibrosis even in the absence of detectable macroscopic fibrosis in the papillary muscle region. Abstract 1047 Figure 1

scholarly journals 423 Effects of mitral valve repair on left ventricular remodeling and ventricular arrhythmia in mitral valve prolapse patients: five-year follow-up

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
E Malev ◽  
M Omelchenko ◽  
L Mitrofanova ◽  
M Gordeev ◽  
B Bondarenko ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Mitral Valve ◽  
Ventricular Arrhythmia ◽  
Mitral Valve Repair ◽  
Mitral Valve Prolapse ◽  
Left Ventricular ◽  
Severe Mitral Regurgitation ◽  
Valve Repair ◽  
Myxomatous Degeneration

Abstract Introduction There is limited data on the efficacy of surgical repair in reducing ventricular arrhythmia (VA) in mitral valve prolapse (MVP) patients. Improvement in malignant ventricular arrhythmias has been reported only in isolated cases after mitral valve surgery. Our aim was to evaluate the possible effects of mitral valve repair on left ventricular (LV) reverse remodeling and incidence of VA in MVP patients in mid-term follow-up. Methods 30 consecutive patients (mean age 53.1 ± 9.4, 47% male) undergoing mitral valve repair for severe mitral regurgitation (MR) due to mitral valve prolapse were enrolled in our observational, prospective, single-center study. Resected abnormal segments of the mitral leaflets were examined by experienced pathologists for signs of myxomatous degeneration. Transthoracic echocardiography extended with speckle-tracking echocardiography and 24-hour Holter monitoring were performed pre- and postoperatively annually. Atrial fibrillation, PVCs and nonsustained ventricular tachycardia (VT) runs were reviewed. Results During 144 person-years of follow-up no deaths, and 3 cases (10%) of recurrent moderate or severe (≥2) MR occurred. The total number of PVCs and non-sustained ventricular tachycardia runs dropped significantly in 1st (p=.04, Wilcoxon matched pairs test) and 2nd (p=.03), years of postoperative follow-up. Postoperative incidence of PVC and VT correlates strongly with postoperative end-diastolic LV diameter (EDD rs=.70; p=.005), moderate negatively with LV ejection fraction (EF rs=-.55; p=.01), but not postoperative MR (p&gt;.05). EDD (58.8 ± 7.6 mm vs. 49.9 ± 5.6 mm; p=.00001) and EDV (156.6 ± 32.1 ml vs. 104.1 ± 22.8 ml; p=.00001) decreased in 1st year after repair with non-significant changes in EF (63.8 ± 12.8% vs. 59.6 ± 14.5%; p=.20), global systolic longitudinal strain –13.8 ± 2.5% vs. –14.6 ± 2.7%; p=.20) and SR (–0.93 ± 0.12 s-1 vs. –0.98 ± 0.13 s-1; p=.09) values. In univariate analysis, postoperative end-diastolic LV diameter (p=.001), low EF (p=.003), myxomatous degeneration (p=.008) were identified as risk factors of persistent PVCs/VT after surgery. Conclusions Mitral valve repair in MVP with severe mitral regurgitation is associated with reduction in ventricular arrhythmia, which strongly correlates with postoperative LV dimensions and function. Further investigation in larger cohorts to evaluate the association between degenerative mitral valve disease and ventricular arrhythmia is needed.

Abstract 17048: Prognostic Implication of Enlarged Left Ventricle in Patients Who Underwent Valve Surgery due to Mitral Valve Prolapse or Flail Mitral Valve: Volume versus Dimension

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Jeong Yoon Jang ◽  
Se Hun Kang ◽  
Jae Seok Bae ◽  
Cheol Hyun Lee ◽  
Yu Na Kim ◽  
...  
Keyword(s):  
Mitral Valve ◽  
Mitral Valve Prolapse ◽  
Coronary Bypass ◽  
Left Ventricular ◽  
Maze Procedure ◽  
Optimal Timing ◽  
Prognostic Information ◽  
Clinical Events ◽  
Lv Dysfunction

Introduction: Left ventricular end-systolic dimension (LVESD) has been being used to guide the optimal timing of surgery in patients with mitral valve prolapse (MVP) or flail mitral valve (FMV). We sought to evaluate whether LVES volume (LVESV) measured by echocardiography can provide additive prognostic information. Methods: Of patients who underwent MV surgery due to MVP or FMV from 2000 to 2014, after exclusion of patients whose rhythm was atrial fibrillation or who needed concomitant maze procedure, aortic valve or coronary bypass surgery, a total of 648 patients (age 51±14 years; ejection fraction 64±7%; LVESD 38±6 mm; LVESV 60±26 mL; repair/replacement = 612/36) was selected. Clinical outcomes included cardiovascular (CV) death, admission due to heart failure (HF) and development of LV dysfunction (EF <45% at the last follow-up). Results: During median follow up of 4.2 years (interquartile range, 1.8-6.6 years), 5 patients died of CV death, 36 admitted for HF, and 38 developed LV dysfunction. Increased LVESD (≥45mm) could not predict CV death. But patients with enlarged LVESV index (LVESV/ body surface area ≥50 ml/m 2 , n=64, 10%) showed higher rate of CV death (p=0.04), HF admission (p=0.04) and composite clinical events (p<0.001). In multivariate regression analysis, enlarged LVESV index was the only independent variable associated with composite clinical events (hazard ratio 2.67, 95% confidence interval 1.39-5.13, p=0.003). Conclusions: In the modern era of MV repair surgery for MVP and FMV, LV volume provides more robust prognostic information than LV dimension.

scholarly journals Possible mechanism of late systolic mitral valve prolapse: systolic superior shift of leaflets secondary to annular dilatation that causes papillary muscle traction

2019 ◽  
Vol 316 (3) ◽  
pp. H629-H638 ◽  
Author(s):  
Soshi Hei ◽  
Mai Iwataki ◽  
Jeong-Yoon Jang ◽  
Hiroshi Kuwaki ◽  
Keitaro Mahara ◽  
...  
Keyword(s):  
Mitral Valve ◽  
Papillary Muscle ◽  
Mitral Valve Prolapse ◽  
Causal Relation ◽  
Left Ventricular Pressure ◽  
Left Ventricular ◽  
Annular Dilatation ◽  
Force Reduction ◽  
Dimensional Echocardiography ◽  
Annulus Dilatation

Progressive superior shift of the mitral valve (MV) during systole is associated with abnormal papillary muscle (PM) superior shift in late systolic MV prolapse (MVP). The causal relation of these superior shifts remains unclarified. We hypothesized that the MV superior shift is related to augmented MV superiorly pushing force by systolic left ventricular pressure due to MV annular dilatation, which can be corrected by surgical MV plasty, leading to postoperative disappearance of these superior shifts. In 35 controls, 28 patients with holosystolic MVP, and 28 patients with late systolic MVP, the MV coaptation depth from the MV annulus was measured at early and late systole by two-dimensional echocardiography. The PM tip superior shift was monitored by echocardiographic speckle tracking. MV superiorly pushing force was obtained as MV annular area × (systolic blood pressure − 10). Measurements were repeated after MV plasty in 14 patients with late systolic MVP. Compared with controls and patients with holosystolic MVP, MV and PM superior shifts and MV superiorly pushing force were greater in patients with late systolic MVP [1.3 (0.5) vs. 0.9 (0.6) vs. 3.9 (1.0) mm/m2, 1.3 (0.5) vs. 1.2 (1.0) vs. 3.3 (1.3) mm/m2, and 487 (90) vs. 606 (167) vs. 742 (177) mmHg·cm2·m−2, respectively, means (SD), P < 0.001]. MV superior shift was correlated with PM superior shift ( P < 0.001), which was further related to augmented MV superiorly pushing force ( P < 0.001). MV and PM superior shift disappeared after surgical MV plasty for late systolic MVP. These data suggest that MV annulus dilatation augmenting MV superiorly pushing force may promote secondary superior shift of the MV (equal to late systolic MVP) that causes subvalvular PM traction in patients with late systolic MVP. NEW & NOTEWORTHY Late systolic mitral valve prolapse (MVP) is associated with mitral valve (MV) and papillary muscle (PM) abnormal superior shifts during systole, but the causal relation remains unclarified. MV and PM superior shifts were correlated with augmented MV superiorly pushing force by annular dilatation and disappeared after surgical MV plasty with annulus size and MV superiorly pushing force reduction. This suggests that MV annulus dilatation may promote secondary superior shifts of the MV (late systolic MVP) that cause subvalvular PM traction.

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