scholarly journals Mechanistic insights of the left ventricle structure and fibrosis in the arrhythmogenic mitral valve prolapse

2018 ◽  
Vol 2018 (1) ◽  
Author(s):  
Leticia Fernández-Friera ◽  
Rafael Salguero ◽  
Luca Vannini ◽  
Ana Fidalgo Argüelles ◽  
Fernando Arribas ◽  
...  
Keyword(s):  
Sudden Cardiac Death ◽  
Mitral Valve ◽  
Mitral Valve Prolapse ◽  
Cardiac Death ◽  
Ventricular Arrhythmias ◽  
Imaging Techniques ◽  
Current Evidence ◽  
Special Focus ◽  
Benign Condition ◽  
Life Threatening

Mitral valve prolapse (MVP) is a common and benign condition. However, some anatomic forms have been recently associated with life-threatening ventricular arrhythmias and sudden cardiac death. Imaging MVP holds the promise of individualized MVP risk assessment. Noninvasive imaging techniques available today are playing an increasingly important role in the diagnosis, prognosis and monitoring of MVP. In this article, we will review the current evidence on arrhythmogenic MVP, with special focus on the utility of echocardiography and CMR for identifying benign and ‘‘malignant’’ forms of MVP. The clinical relevance of this manuscript lies in the value of imaging technology to improve MVP risk prediction, including those arrhythmic-MVP cases with a higher risk of sudden cardiac death. 

scholarly journals Identifying Mitral Valve Prolapse at Risk for Ventricular Arrhythmias and Myocardial Fibrosis from 12-lead Electrocardiograms using Deep Learning

2021 ◽  
Author(s):  
Geoffrey H Tison ◽  
Sean Abreau ◽  
Lisa Lim ◽  
Valentina Crudo ◽  
Joshua Barrios ◽  
...  
Keyword(s):  
Machine Learning ◽  
At Risk ◽  
Cardiac Arrest ◽  
Sudden Cardiac Death ◽  
Mitral Valve ◽  
Mitral Regurgitation ◽  
Myocardial Fibrosis ◽  
Mitral Valve Prolapse ◽  
Cardiac Death ◽  
Ventricular Arrhythmias

Background: Mitral valve prolapse (MVP) is a common valvulopathy, with a subset of MVP patients developing sudden cardiac death or cardiac arrest. Complex ventricular ectopy (ComVE) represents a marker of arrhythmic risk that is associated with myocardial fibrosis and increased mortality in MVP. We hypothesize that an ECG-based machine-learning model can identify MVP with ComVE and/or myocardial fibrosis on cardiac magnetic resonance (CMR) imaging. Methods: A deep convolutional neural network (CNN) was trained to detect ComVE using 6,916 12-lead ECGs from 569 MVP patients evaluated at the University of California San Francisco (UCSF) between 2012 and 2020. A separate CNN was also trained to detect late gadolinium enhancement (LGE) using 87 ECGs from MVP patients with contrast CMR. Results: The prevalence of ComVE was 160/569 or 28% (20 patients or 3% had cardiac arrest or sudden cardiac death). The area under the curve (AUC) of the CNN to detect ComVE was 0.81 (95% CI, 0.78-0.84). AUC remained high even after excluding patients with moderate-severe mitral regurgitation (MR) [0.80 (95% CI, 0.77-0.83)], or with bileaflet MVP [0.81 (95% CI, 0.76-0.85)]. The top ECG segments able to discriminate ComVE vs no ComVE were related to ventricular depolarization and repolarization (early-mid ST and QRS fromV1, V3, and III). LGE in the papillary muscles or basal inferolateral wall was present in 21 (24%) of 87 patients with available CMR. The AUC for detection of LGE was 0.75 (95% CI, 0.68-0.82). Conclusions: Standard 12-lead ECGs analyzed with machine learning can detect MVP at risk for ventricular arrhythmias and fibrosis and can identify novel ECG correlates of arrhythmic risk regardless of leaflet involvement or mitral regurgitation severity. ECG-based CNNs may help select those MVP patients requiring closer follow-up and/or a CMR. 

Electrocardiographic and echocardiographic abnormalities associated with mitral valve prolapse in patients with Marfan syndrome

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
A Lasne ◽  
O Milleron ◽  
G Delorme ◽  
F Arnoult ◽  
N Hanna ◽  
...  
Keyword(s):  
Sudden Cardiac Death ◽  
Mitral Valve ◽  
Marfan Syndrome ◽  
Mitral Valve Prolapse ◽  
Cardiac Death ◽  
Ventricular Arrhythmias ◽  
Qtc Interval ◽  
Interval Prolongation ◽  
High Prevalence ◽  
Qtc Interval Prolongation

Abstract Introduction Marfan syndrome (MFS) is responsible for cardiovascular disorders such as aortic aneurism and mitral valve prolapse (MVP). A malignant MVP phenotype combining clinical, electrical and morphological features has been described in symptomatic patients who have experienced sudden cardiac death or complex ventricular arrhythmias. We have taken advantage of the high prevalence of MVP in MFS patient to study the clinical, electrical and echocardiographic abnormalities associated with MVP. Purpose The aim of this study is to describe the clinical, electrical and morphological cardiac abnormalities associated with MVP in a cohort of MFS patients with FBN1 mutations with a high prevalence of MVP and who did not suffer from severe ventricular arrhythmias. Methods All consecutive patients coming to the National Reference Center for Marfan syndrome were evaluated prospectively i.e. clinical examination, 12-lead electrocardiogram, standard transthoracic echocardiography study and molecular genetic screening. Results 352 consecutive patients were included from April 2015 to October 2016 [250 FBN1 mutation carriers (MFS) and 102 healthy relatives (HR)]. None of the patients had a history of sudden cardiac death or complex ventricular arrhythmia. MFS vs HR: MFS patients were younger (33 vs 41yo p<0.001) and 2/3 were women in both groups. In the MFS group, abnormal T waves repolarization in lateral leads were more common [172 MFS (70.2%) vs. 87 HR (86.14%) p<0,0012], as was MVP [38.37% vs 1.96%; p<0,0001], and diastolic hypertrophy of the basal segment of the inferolateral wall (thickness >11mm) [22.31% vs. 9.18%; p<0.0001]. In MFS, MVP affected either one valve (21.22%), or both (17.14%), and was not associated with electric abnormalities. However, diastolic basal inferolateral wall hypertrophy was associated with mitral valve prolapse (p<0,0001), QTc interval prolongation (p<0.0229), abnormal T waves repolarization in the inferior leads (p=0.004), and higher aortic Z-Score (p=0.274). Conclusion In MFS patients, the prevalence of MVP is high and no significant association between MVP and electrical abnormalities was found. In contrast, basal inferolateral wall hypertrophy is associated with MVP and repolarization disorders in inferior leads and QTc interval prolongation, i.e, electrocardiographic abnormalities described in malignant MVP. QTc and basal inferolateral hypertrophy Funding Acknowledgement Type of funding source: None

scholarly journals 1642 Mitral annulus disjunction, a rare form of focal arrhythmogenic cardiomyopathy

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
A Nagy ◽  
A Apor ◽  
C S Czimbalmos ◽  
N Szegedi ◽  
A Toth ◽  
...  
Keyword(s):  
Sudden Cardiac Death ◽  
Mitral Valve ◽  
Mitral Valve Prolapse ◽  
Cardiac Death ◽  
Longitudinal Strain ◽  
Imaging Techniques ◽  
Mitral Annulus ◽  
Severe Mitral Regurgitation ◽  
Papillary Muscles ◽  
Non Invasive

Abstract Funding Acknowledgements Project no. NVKP_16-1-2016-0017 has been implemented with the support provided from the National Research, Development and Innovation Fund of Hungary The rate of sudden cardiac death in mitral valve prolapse (MVP) patients is roughly twice that observed in the general population. Arrhythmogenic bileaflet MVP syndrome has been recently described and mitral annulus disjunction (MAD) has been found associated with papillary muscle fibrosis and ventricular arrhythmias even without severe mitral regurgitation. An underlying primary structural myocardial disease might be expected and the non-invasive imaging techniques are playing an increasingly important role in the detection of myocardial fibrosis as a focal arrhythmogenic substrate. Standard 2D-, 3D- and speckle tracking echocardiography and cardiac MRI can help to differentiate between the benign and malignant forms of MVP. A 52-year-old man with a history of hypertension was referred for evaluation of frequent palpitations and presyncope. Ambulatory Holter monitoring showed paroxysmal atrial fibrillation and non-sustained ventricular tachycardias. Dilated left ventricle (LVEDD: 65mm, LVESD: 45 mm) and left atrium (LAVi: 52mL/m2), moderately reduced left ventricular (LV) systolic function (LVEF: 42%), and reduced LV global longitudinal strain (GLS: -13.5%) with severely decreased segmental longitudinal strain in the basal inferolateral segment were measured by transthoracic echocardiography. The diastolic function was preserved, however Pickelhaube sign was noticed in the lateral mitral annulus TDI curves. MAD (17 mm) and curling motion of the inferolateral basal segment of the left ventricle was observed. Transesophageal echocardiography showed bileaflet mitral valve prolapse with the involvement of all the six scallops (mitral valve prolapse volume: 10.2 mL). Severe mitral regurgitation (3D vena contracta area: 1cm2) and extremely dilated and dyskinetic mitral annulus (diameters: 55x72x12 mm, area: 34 cm2) was measured by 3D echocardiography. MRI showed transmural late gadolinium enhancement in the underlying myocardium of both papillary muscles and midmyocardial enhancement in the basal inferior, inferolateral and anteroseptal LV segments. During electrophysiology study ventricular fibrillation was easily induced and ICD was placed for primary prevention of sudden cardiac death. The patient was referred to the heart surgeon for mitral valve replacement. This case highlights the importance of early recognition of MAD and arrhythmogenic MVP syndrome using novel non-invasive imaging techniques. The presence of MAD, the curling motion of the inferolateral basal LV segment, the Pickelhaube sign, the decreased LVEF and GLS, the characteristic segmental longitudinal strain pattern and the presence of late enhancement in the papillary muscles and the surrounding myocardium could be warning signs of the malignant form of MVP. Abstract 1642 Figure. CMR images of arrhythmogenic MAD

scholarly journals Mitral valve and left ventricular features in malignant mitral valve prolapse

Open Heart ◽  
2018 ◽  
Vol 5 (2) ◽  
pp. e000925 ◽  
Author(s):  
Madalina Garbi ◽  
Patrizio Lancellotti ◽  
Mary N Sheppard
Keyword(s):  
Sudden Cardiac Death ◽  
Mitral Valve ◽  
Mitral Valve Prolapse ◽  
Cardiac Death ◽  
Left Ventricular ◽  
Abnormal Finding ◽  
Benign Condition ◽  
Artery Disease ◽  
Ventricular Fibrosis ◽  
Death Cases

ObjectiveMitral valve prolapse is a benign condition, however with occasional reports of sudden cardiac death or out-of-hospital cardiac arrest in the absence of severe mitral regurgitation or coronary artery disease, suggesting the existence of a malignant form. The objective of our study was to contribute to the characterisation of malignant mitral valve prolapse.MethodsWe performed a retrospective analysis of pathology findings in 68 consecutive cases of sudden cardiac death with mitral valve prolapse as lone abnormal finding, reported as cause of death.ResultsAll mitral valve prolapse sudden death cases had mitral valve characteristics of Barlow disease, with extensive bileaflet multisegmental prolapse and dilatation of the annulus. The majority of cases (80.9%) had microscopic left ventricular fibrosis with associated hypertrophy and degenerative features of the myocytes, and some cases (10.9%) had right ventricular fibrosis as well.ConclusionsMalignant mitral valve prolapse is Barlow disease. Sudden cardiac death in mitral valve prolapse is due to Barlow disease, which besides the typical mitral valve degeneration may comprise a distinct Barlow disease cardiomyopathy, as suggested by myocyte degeneration and bi-ventricular involvement.

scholarly journals Mitral Valve Prolapse, Arrhythmias, and Sudden Cardiac Death: The Role of Multimodality Imaging to Detect High-Risk Features

Diagnostics ◽  
2021 ◽  
Vol 11 (4) ◽  
pp. 683
Author(s):  
Anna Giulia Pavon ◽  
Pierre Monney ◽  
Juerg Schwitter
Keyword(s):  
Sudden Cardiac Death ◽  
Mitral Valve ◽  
Mitral Valve Prolapse ◽  
Cardiac Death ◽  
Multimodality Imaging ◽  
Imaging Evaluation ◽  
Benign Condition ◽  
The 1960S

Mitral valve prolapse (MVP) was first described in the 1960s, and it is usually a benign condition. However, a subtype of patients are known to have a higher incidence of ventricular arrhythmias and sudden cardiac death, the so called “arrhythmic MVP.” In recent years, several studies have been published to identify the most important clinical features to distinguish the benign form from the potentially lethal one in order to personalize patient’s treatment and follow-up. In this review, we specifically focused on red flags for increased arrhythmic risk to whom the cardiologist must be aware of while performing a cardiovascular imaging evaluation in patients with MVP.

scholarly journals Electrophysiological Substrate in Patients with Barlow’s Disease

2021 ◽  
Vol 10 (1) ◽  
pp. 33-37
Author(s):  
Pasquale Vergara ◽  
Savino Altizio ◽  
Giulio Falasconi ◽  
Luigi Pannone ◽  
Simone Gulletta ◽  
...  
Keyword(s):  
Risk Factors ◽  
Catheter Ablation ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Ventricular Arrhythmias ◽  
Imaging Techniques ◽  
Treatment Options ◽  
Clinical Syndrome ◽  
Benign Condition ◽  
Barlow’S Disease

Mitral valve prolapse (MVP) is the most common valvular heart disease, affecting 2–3% of the general population. Barlow’s disease is a clinical syndrome characterised by MVP. Initially thought a benign condition, MVP is now recognised as a cause of sudden cardiac death and ventricular arrhythmias. The development of new imaging techniques has contributed recently to the identification of novel risk factors. Catheter ablation of ventricular arrhythmias in patients affected by MVP is traditionally considered challenging. In this review, the authors summarise the evidence on arrhythmogenesis in the context of MVP, along with risk stratification of sudden cardiac death and the available treatment options, including new catheter ablation techniques.

scholarly journals Cardiac Imaging for Risk Assessment of Malignant Ventricular Arrhythmias in Patients With Mitral Valve Prolapse

2021 ◽  
Vol 8 ◽  
Author(s):  
Bhupendar Tayal ◽  
Francesca N. Delling ◽  
Maan Malahfji ◽  
Dipan J. Shah
Keyword(s):  
Sudden Cardiac Death ◽  
Mitral Valve ◽  
Cardiac Imaging ◽  
Mitral Valve Prolapse ◽  
Cardiac Death ◽  
Ventricular Arrhythmias ◽  
Mechanical Dispersion ◽  
Increased Risk ◽  
Replacement Fibrosis ◽  
Imaging Markers

Recent studies have described the occurrence of complex ventricular arrhythmias and sudden cardiac death among patients with mitral valve prolapse (MVP). The reported incidence rate of sudden cardiac death or ventricular tachycardia is about 1–1.5% among patients with MVP. Various imaging markers have been associated with this increased risk, including mitral annular disjunction, replacement fibrosis by late gadolinium enhancement, and mechanical dispersion. In this review, we briefly discuss how multimodality cardiac imaging can be applied to identify MVP patients with high risk of sudden cardiac death and complex ventricular arrhythmias.

scholarly journals Malignant Arrhythmic Mitral Valve Prolapse: A Continuum of Clinical Challenges from Diagnosis to Risk Stratification and Patient Management

2020 ◽  
Vol 8 (1) ◽  
pp. 2
Author(s):  
Idit Yedidya ◽  
Aniek L. van Wijngaarden ◽  
Nina Ajmone Marsan
Keyword(s):  
Mitral Valve ◽  
Mitral Valve Prolapse ◽  
Ventricular Arrhythmias ◽  
Clinical Decision Making ◽  
Clinical Presentation ◽  
Clinical Decision ◽  
Benign Condition ◽  
Long Period ◽  
Patients Experience ◽  
Hospital Cardiac Arrest

Mitral valve prolapse (MVP) is a common valvular disease, which may remain a benign condition for a long period of time. However, some patients experience malignant ventricular arrhythmias and sudden cardiac death (SCD). It is still largely unknown how to risk-stratify these patients, and no specific recommendations have been proposed to help the clinical decision-making. We present the case of a young man whose first clinical presentation was an out-of-hospital cardiac arrest and was subsequently diagnosed with MVP. We highlighted the possible risk factors for SCD and the challenges in the clinical management of these patients.

scholarly journals SUDDEN CARDIAC DEATH IN MALIGNANT BILEAFLET MITRAL VALVE PROLAPSE

2015 ◽  
Vol 65 (10) ◽  
pp. A751
Author(s):  
Swapna Kanuri ◽  
Pallavi Bellamkonda ◽  
Aryan Mooss
Keyword(s):  
Sudden Cardiac Death ◽  
Mitral Valve ◽  
Mitral Valve Prolapse ◽  
Cardiac Death
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