scholarly journals Mitral Valve Prolapse, Ventricular Arrhythmias, and Sudden Death

Circulation ◽  
2019 ◽  
Vol 140 (11) ◽  
pp. 952-964 ◽  
Author(s):  
Cristina Basso ◽  
Sabino Iliceto ◽  
Gaetano Thiene ◽  
Martina Perazzolo Marra
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Mitral Valve ◽  
Sudden Death ◽  
Mitral Valve Prolapse ◽  
Ventricular Arrhythmias ◽  
Left Ventricular ◽  
Premature Ventricular Beats ◽  
Electrophysiologic Studies

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 arrhythmic MVP are unknown. The clinical profile is characterized by a patient, usually female, with mostly bileaflet myxomatous disease, mid-systolic click, repolarization abnormalities in the inferior leads, and complex ventricular arrhythmias with polymorphic/right bundle branch block morphology, without significant regurgitation. Among the various pathophysiologic mechanisms of electrical instability, left ventricular fibrosis in the papillary muscles and inferobasal wall, mitral annulus disjunction, and systolic curling have been recently described by pathological and cardiac magnetic resonance studies in sudden death victims and patients with arrhythmic MVP. In addition, premature ventricular beats arising from the Purkinje tissue as ventricular fibrillation triggers have been documented by electrophysiologic studies in MVP patients with aborted sudden death. The genesis of malignant ventricular arrhythmias in MVP probably recognizes the combination of the substrate (regional myocardial hypertrophy and fibrosis, Purkinje fibers) and the trigger (mechanical stretch) eliciting premature ventricular beats because of a primary morphofunctional abnormality of the mitral valve annulus. The main clinical challenge is how to identify patients with arrhythmic MVP (which imaging technique and in which patient) and how to treat them to prevent sudden death. Thus, there is a necessity for prospective multicenter studies focusing on the prognostic role of cardiac magnetic resonance and electrophysiologic studies and on the therapeutic efficacy of targeted catheter ablation and mitral valve surgery in reducing the risk of life-threatening arrhythmias, as well as the role of implantable cardioverter defibrillators for primary prevention.

scholarly journals Role of Cardiac Magnetic Resonance Imaging in the Evaluation of Athletes with Premature Ventricular Beats

2022 ◽  
Vol 11 (2) ◽  
pp. 426
Author(s):  
Giulia Brunetti ◽  
Alberto Cipriani ◽  
Martina Perazzolo Marra ◽  
Manuel De Lazzari ◽  
Barbara Bauce ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Training Session ◽  
Left Ventricular ◽  
Special Focus ◽  
Premature Ventricular Beats ◽  
Myocardial Substrate ◽  
Diagnostic Work ◽  
Cmr Imaging

Premature ventricular beats (PVBs) in athletes are not rare. The risk of PVBs depends on the presence of an underlying pathological myocardial substrate predisposing the subject to sudden cardiac death. The standard diagnostic work-up of athletes with PVBs includes an examination of family and personal history, resting electrocardiogram (ECG), 24 h ambulatory ECG (possibly with a 12-lead configuration and including a training session), maximal exercise testing and echocardiography. Despite its fundamental role in the diagnostic assessment of athletes with PVBs, echocardiography has very limited sensitivity in detecting the presence of non-ischemic left ventricular scars, which can be revealed only through more in-depth studies, particularly with the use of contrast-enhanced cardiac magnetic resonance (CMR) imaging. The morphology, complexity and exercise inducibility of PVBs can help estimate the probability of an underlying heart disease. Based on these features, CMR imaging may be indicated even when echocardiography is normal. This review focuses on interpreting PVBs, and on the indication and role of CMR imaging in the diagnostic evaluation of athletes, with a special focus on non-ischemic left ventricular scars that are an emerging substrate of cardiac arrest during sport.

Multimodality imaging assessment of mitral annular disjunction in mitral valve prolapse

Heart ◽  
2020 ◽  
Vol 107 (1) ◽  
pp. 25-32 ◽  
Author(s):  
Valentina Mantegazza ◽  
Valentina Volpato ◽  
Paola Gripari ◽  
Sarah Ghulam Ali ◽  
Laura Fusini ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Mitral Valve ◽  
Mitral Valve Prolapse ◽  
Ventricular Arrhythmias ◽  
Multimodality Imaging ◽  
Imaging Techniques ◽  
Transoesophageal Echocardiography ◽  
Small Length ◽  
Imaging Approach

ObjectiveMitral annular disjunction (MAD) is an abnormality linked to mitral valve prolapse (MVP), possibly associated with malignant ventricular arrhythmias. We assessed the agreement among different imaging techniques for MAD identification and measurement.Methods131 patients with MVP and significant mitral regurgitation undergoing transthoracic echocardiography (TTE) and cardiac magnetic resonance (CMR) were retrospectively enrolled. Transoesophageal echocardiography (TOE) was available in 106 patients. MAD was evaluated in standard long-axis views (four-chamber, two-chamber, three-chamber) by each technique.ResultsConsidering any-length MAD, MAD prevalence was 17.3%, 25.5%, 42.0% by TTE, TOE and CMR, respectively (p<0.05). The agreement on MAD identification was moderate between TTE and CMR (κ=0.54, 95% CI 0.49 to 0.59) and good between TOE and CMR (κ=0.79, 95% CI 0.74 to 0.84). Assuming CMR as reference and according to different cut-off values for MAD (≥2 mm, ≥4 mm, ≥6 mm), specificity (95% CI) of TTE and TOE was 99.6 (99.0 to 100.0)% and 98.7 (97.4 to 100.0)%; 99.3 (98.4 to 100.0)% and 97.6 (95.8 to 99.4)%; 97.8 (96.2 to 99.3)% and 93.2 (90.3 to 96.1)%, respectively; sensitivity (95% CI) was 43.1 (37.8 to 48.4)% and 74.5 (69.4 to 79.5)%; 54.0 (48.7 to 59.3)% and 88.9 (85.2 to 92.5)%; 88.0 (84.5 to 91.5)% and 100.0 (100.0 to 100.0)%, respectively. MAD length was 8.0 (7.0-10.0), 7.0 (5.0-8.0], 5.0 (4.0-7.0) mm, respectively by TTE, TOE and CMR. Agreement on MAD measurement was moderate between TTE and CMR (ρ=0.73) and strong between TOE and CMR (ρ=0.86).ConclusionsAn integrated imaging approach could be necessary for a comprehensive assessment of patients with MVP and symptoms suggestive for arrhythmias. If echocardiography is fundamental for the anatomic and haemodynamic characterisation of the MV disease, CMR may better identify small length MAD as well as myocardial fibrosis.

scholarly journals Prognostic significance of cardiac magnetic resonance-based markers in patients with hypertrophic cardiomyopathy

2021 ◽  
Author(s):  
Zsofia Dohy ◽  
Liliana Szabo ◽  
Attila Toth ◽  
Csilla Czimbalmos ◽  
Rebeka Horvath ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Hypertrophic Cardiomyopathy ◽  
Magnetic Resonance ◽  
Myocardial Fibrosis ◽  
Ventricular Arrhythmias ◽  
Prognostic Significance ◽  
Strain Analysis ◽  
Left Ventricular ◽  
Icd Therapy ◽  
Lv Mass

AbstractThe prognosis of patients with hypertrophic cardiomyopathy (HCM) varies greatly. Cardiac magnetic resonance (CMR) is the gold standard method for assessing left ventricular (LV) mass and volumes. Myocardial fibrosis can be noninvasively detected using CMR. Moreover, feature-tracking (FT) strain analysis provides information about LV deformation. We aimed to investigate the prognostic significance of standard CMR parameters, myocardial fibrosis, and LV strain parameters in HCM patients. We investigated 187 HCM patients who underwent CMR with late gadolinium enhancement and were followed up. LV mass (LVM) was evaluated with the exclusion and inclusion of the trabeculae and papillary muscles (TPM). Global LV strain parameters and mechanical dispersion (MD) were calculated. Myocardial fibrosis was quantified. The combined endpoint of our study was all-cause mortality, heart transplantation, malignant ventricular arrhythmias and appropriate implantable cardioverter defibrillator (ICD) therapy. The arrhythmia endpoint was malignant ventricular arrhythmias and appropriate ICD therapy. The LVM index (LVMi) was an independent CMR predictor of the combined endpoint independent of the quantification method (p < 0.01). The univariate predictors of the combined endpoint were LVMi, global longitudinal (GLS) and radial strain and longitudinal MD (MDL). The univariate predictors of arrhythmia events included LVMi and myocardial fibrosis. More pronounced LV hypertrophy was associated with impaired GLS and increased MDL. More extensive myocardial fibrosis correlated with impaired GLS (p < 0.001). LVMi was an independent CMR predictor of major events, and myocardial fibrosis predicted arrhythmia events in HCM patients. FT strain analysis provided additional information for risk stratification in HCM patients.

scholarly journals Incremental value of feature-tracking cardiac magnetic resonance imaging in detecting myocardial dysfunction in patients with idiopathic ventricular arrhythmias

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
C Nikolaidou ◽  
C Kotanidis ◽  
J Leal-Pelado ◽  
K Kouskouras ◽  
VP Vassilikos ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Ventricular Arrhythmias ◽  
Feature Tracking ◽  
Myocardial Dysfunction ◽  
Global Longitudinal Strain ◽  
Left Ventricular ◽  
Volume Index ◽  
Control Subjects ◽  
Cmr Imaging

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Cardiac magnetic resonance (CMR) imaging can identify the underlying substrate in patients with ventricular arrhythmias (VAs) and normal echocardiography. Myocardial strain has emerged as a superior index of systolic performance compared to ejection fraction (EF), with an incremental prognostic value in many cardiac diseases. Purpose To assess myocardial deformation using 2-D feature-tracking CMR strain imaging (CMR-FT) in patients with frequent VAs (≥500 ventricular premature contractions (VPC)/24 hours; and/or non-sustained ventricular tachycardia), and structurally normal hearts on echocardiography without evidence of coronary artery disease. Methods Sixty-eight consecutive patients (mean age 46 ± 16 years; 54% female) and 72 healthy controls matched for age and body surface area were included in the study. CMR imaging was performed on a 1.5T Magnetom Avanto (Siemens, Erlangen, Germany) scanner using a standard cardiac protocol. Results CMR showed normal findings in 30 patients (44%), while 16 (24%) had previous myocarditis, 6 (9%) had a diagnosis of non-ischaemic cardiomyopathy (NICM), 15 (22%) were diagnosed with VPC-related cardiomyopathy, and 1 patient had subendocardial infarction [excluded from strain analysis]. Mean left ventricular EF (LVEF) in patients was 62% ± 6% and right ventricular EF 64% ± 6% (vs. 65% ± 3% and 66% ± 4% in controls, respectively). Compared to control subjects, patients with VAs had impaired peak LV global radial strain (GRS) (28.88% [IQR: 25.87% to 33.97%] vs. 36.65% [IQR:33.19% to 40.2%], p &lt; 0.001) and global circumferential strain (GCS) (-17.73% [IQR: -19.8% to -16.33%] vs. -20.66% [IQR: -21.72% to -19.6%], p &lt; 0.001, Panel A). Peak LV GRS could differentiate patients with previous myocarditis from patients with NICM and those with VPC-related cardiomyopathy (Panel B). Peak LV GCS could differentiate patients with previous myocarditis from patients with NICM (Panel C). Peak LV GRS showed excellent diagnostic accuracy in detecting patients from control subjects (Panel D). In a multivariable regression model, subjects with a low GRS (&lt;29.91%-determined by the Youden’s index) had 5-fold higher odds of having VAs (OR:4.99 [95%CI: 1.2-21.95]), after adjusting for LVEF, LV end-diastolic volume index, age, sex, BMI, smoking, hypertension, and dyslipidaemia. Peak LV global longitudinal strain (GLS) and RV strain indices were not statistically different between patients and controls. Conclusion Peak LV GRS and GCS are impaired in patients with frequent idiopathic VAs and can detect myocardial contractile dysfunction in patients with different underlying substrates. Our findings suggest that LV strain indices on CMR-FT constitute independent markers of myocardial dysfunction on top and independently of EF. Abstract Figure.

scholarly journals Diagnostic and prognostic impact of cardiac magnetic resonance, including scar quantification and strain imaging in patients with malignant ventricular arrhythmias and nonobstructed coronary arteries

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
L Szabo ◽  
V Bodi ◽  
CS Czimbalmos ◽  
ZS Dohy ◽  
V Horvath ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Coronary Arteries ◽  
Ventricular Arrhythmias ◽  
Left Ventricular ◽  
Myocardial Scar ◽  
Scar Formation ◽  
Structural Abnormality ◽  
Icd Therapy ◽  
All Cause Mortality

Abstract Funding Acknowledgements Type of funding sources: Public grant(s) – National budget only. Main funding source(s): Development and Innovation Fund of Hungary, National Research, Development and Innovation Office Background In case of malignant ventricular arrhythmias (VA) and nonobstructed coronary arteries, the differential diagnosis of the  underlying diseases is still challenging, due to the board spectrum of possible causes. Cardiac magnetic resonance (CMR) provides functional, morphological and tissue specific information, including necrotic and scar-tissue.  Aims   We aimed to assess the diagnostic and prognostic implications of CMR parameters including global strain values and myocardial scar in patients after ventricular fibrillation (VF) or sustained ventricular tachycardia (SVT) and nonobstructed coronary arteries. Methods Between 2011 and 2019, 99 patients (42 ±17 years, 54 male) presenting with VF or SVT and nonobstructed coronary arteries, who underwent CMR examination before secondary prevention implantable cardioverter defibrillator (ICD) implantation were included in our study. Post-processing included feature-tracking strain analysis and left ventricular (LV) scar quantification. Patients were followed for the combined endpoint of all-cause-mortality and appropriate ICD therapy. Results CMR examination proved structural myocardial disease in 72%: dilated (n = 21), arrhythmogenic (n = 11), hypertrophic cardiomyopathy (n = 7) and other cardiomyopathies (n = 3). We found LGE patterns showing chronic myocardial infarction (n = 4), suggesting chronic myocarditis (n = 4) and aspecific nonischemic scar formation (n = 14). In 7 cases aspecific structural alterations without scar formation were detected. Overall, myocardial scar was found in 52%, with an average extent of 12 ± 8% of the LV myocardium. The CMR examination changed the clinical diagnosis in 55% of the patients.  During a median follow-up at 2 years, 6 patients died and 42 experienced appropriate ICD therapy. We found an association between cardiac events and the presence of structural abnormality and myocardial scar (logrank: 4,553, p &lt; 0.05 and 8.375, p &lt;0.01).  On Cox proportional-hazards modell LV ejection fraction, LV stroke volume index, the presence of structural abnormality, the presence and extent of  myocardial scar, global LV strain parameters including longitudinal and circumferential strain, and a global left ventricular dssynchrony parameter (mechanical dispersion) were univariate predictors of the combined endpoint of all-cause-mortality and appropriate ICD therapy(p &lt; 0.05).  Conclusion CMR performed in patients after malignant VA and nonobstructed coronary arteries not only establishes the diagnosis in a high proportion of patients, but may also provide additional prognostic factors. This may indicate that CMR could play a complementary role in the risk stratification in this patient population. Abstract Figure.

scholarly journals Left ventricular noncompaction—a rare cause of triad: heart failure, ventricular arrhythmias, and systemic embolic events: a case report 

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Despina Toader ◽  
Alina Paraschiv ◽  
Petrișor Tudorașcu ◽  
Diana Tudorașcu ◽  
Constantin Bataiosu ◽  
...  
Keyword(s):  
Heart Failure ◽  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Left Ventricle ◽  
Ventricular Arrhythmias ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Left Ventricular Noncompaction ◽  
Ventricular Noncompaction ◽  
The Right

Abstract Background Left ventricular noncompaction is a rare cardiomyopathy characterized by a thin, compacted epicardial layer and a noncompacted endocardial layer, with trabeculations and recesses that communicate with the left ventricular cavity. In the advanced stage of the disease, the classical triad of heart failure, ventricular arrhythmia, and systemic embolization is common. Segments involved are the apex and mid inferior and lateral walls. The right ventricular apex may be affected as well. Case presentation A 29-year-old Caucasian male was hospitalized with dyspnea and fatigue at minimal exertion during the last months before admission. He also described a history of edema of the legs and abdominal pain in the last weeks. Physical examination revealed dyspnea, pulmonary rales, cardiomegaly, hepatomegaly, and splenomegaly. Electrocardiography showed sinus rhythm with nonspecific repolarization changes. Twenty-four-hour Holter monitoring identified ventricular tachycardia episodes with right bundle branch block morphology. Transthoracic echocardiography at admission revealed dilated left ventricle with trabeculations located predominantly at the apex but also in the apical and mid portion of lateral and inferior wall; end-systolic ratio of noncompacted to compacted layers > 2; moderate mitral regurgitation; and reduced left ventricular ejection fraction. Between apical trabeculations, multiple thrombi were found. The right ventricle had normal morphology and function. Speckle-tracking echocardiography also revealed systolic left ventricle dysfunction and solid body rotation. Abdominal echocardiography showed hepatomegaly and splenomegaly. Abdominal computed tomography was suggestive for hepatic and renal infarctions. Laboratory tests revealed high levels of N-terminal pro-brain natriuretic peptide and liver enzymes. Cardiac magnetic resonance evaluation at 1 month after discharge confirmed the diagnosis. The patient received anticoagulants, antiarrhythmics, and heart failure treatment. After 2 months, before device implantation, he presented clinical improvement, and echocardiographic evaluation did not detect thrombi in the left ventricle. Coronary angiography was within normal range. A cardioverter defibrillator was implanted for prevention of sudden cardiac death. Conclusions Left ventricular noncompaction is rare cardiomyopathy, but it should always be considered as a possible diagnosis in a patient hospitalized with heart failure, ventricular arrhythmias, and systemic embolic events. Echocardiography and cardiac magnetic resonance are essential imaging tools for diagnosis and follow-up.

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