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.
The prognostic role of cardiac magnetic resonance (CMR) and late gadolinium enhancement (LGE) has not been clarified in acute myocarditis (AM) with preserved left ventricular (LV) ejection fraction ...(EF).
This study sought to evaluate the role of CMR and LGE in the prognosis of AM with preserved LVEF.
This study analyzed data from ITAMY (ITalian multicenter study on Acute MYocarditis) and evaluated CMR results from 386 patients (299 male; mean age 35 ± 15 years) with AM and preserved LVEF. Clinical follow-up was performed for a median of 1,572 days. A clinical combined endpoint of cardiac death, appropriate implantable cardioverter-defibrillator firing, resuscitated cardiac arrest, and hospitalization for heart failure was used.
Among the 374 patients with suitable images, LGE involved the subepicardial layer inferior and lateral wall in 154 patients (41%; IL group), the midwall layer of the anteroseptal wall in 135 patients (36%; AS anteroseptal group), and other segments in 59 patients (16%; other-LGE group), and it was absent in 26 patients (no-LGE group). The AS group had a greater extent of LGE and a higher LV end-diastolic volume index than other groups, but levels of inflammatory markers were lower than in the other groups. Kaplan-Meier curve analysis indicated that the AS group had a worse prognosis than the other groups (p < 0.0001). Finally, in multivariable analysis, AS LGE was the best independent CMR predictor of the combined endpoint (odds ratio: 2.73; 95% confidence interval: 1.2 to 5.9; p = 0.01).
In patients with AM and preserved LVEF, LGE in the midwall layer of the AS myocardial segment is associated with a worse prognosis than other patterns of presentation.
Cardiac magnetic resonance (CMR) is widely used to confirm the diagnosis of acute myocarditis (AM) in the acute setting. CMR is often repeated after 6 months to assess the evolution of myocardial ...involvement. However, the clinical and prognostic role of 6-month CMR is unknown.
This multicenter study aimed to evaluate the clinical and prognostic role of 6-month repetition of CMR in patients with AM.
In a subgroup of 187 patients from the ITAMY (ITAlian study in MYocarditis) registry, CMR was performed within the first week after symptom onset (CMR-I) and repeated after 6 months (CMR-II).
Myocardial edema was detected in all the patients at CMR-I and persisted in 31 (16%) at CMR-II. LGE was detected in 182 (96%) patients at CMR-I and in 164 (86%) at CMR-II. At CMR-II, 20 (11%) patients presented a complete recovery from edema and LGE, 30 (16%) patients had edema with LGE, and 137 (73%) presented LGE without edema. LGE disappeared completely in 18 (10%) patients, the number of LGE segments decreased in 87 (46%), unchanged in 58 (31%), and increased in 26 (14%). During a median clinical follow-up of 7 years (25th to 75th percentile: 6 to 8 years) cardiac events occurred in 22 patients. At Kaplan-Meier curves, patients with LGE and without edema had worse prognosis than others (p < 0.0001). Patients with increased extent of LGE (p = 0.02) had a worse prognosis than those with decreased/unchanged LGE. At multivariate Cox regression analysis, the midwall septal pattern of LGE and the presence of LGE without edema at CMR-II were independent predictors of a cardiac event.
In the acute setting, LGE does not mean definite fibrosis, and it may disappear at 6 months. The presence of LGE without edema at 6-month CMR is associated with worse prognosis, particularly when distributed with a midwall septal pattern. LGE without edema could represent definite fibrosis whereas the presence of edema suggests a residual chance of recovery.
Display omitted
BACKGROUND—Mitral valve prolapse (MVP) may present with ventricular arrhythmias and sudden cardiac death (SCD) even in the absence of hemodynamic impairment. The structural basis of ventricular ...electric instability remains elusive.
METHODS AND RESULTS—The cardiac pathology registry of 650 young adults (≤40 years of age) with SCD was reviewed, and cases with MVP as the only cause of SCD were re-examined. Forty-three patients with MVP (26 females; age range, 19–40 years; median, 32 years) were identified (7% of all SCD, 13% of women). Among 12 cases with available ECG, 10 (83%) had inverted T waves on inferior leads, and all had right bundle-branch block ventricular arrhythmias. A bileaflet involvement was found in 70%. Left ventricular fibrosis was detected at histology at the level of papillary muscles in all patients, and inferobasal wall in 88%. Living patients with MVP with (n=30) and without (control subjects; n=14) complex ventricular arrhythmias underwent a study protocol including contrast-enhanced cardiac magnetic resonance. Patients with either right bundle-branch block type or polymorphic complex ventricular arrhythmias (22 females; age range, 28–43 years; median, 41 years), showed a bileaflet involvement in 70% of cases. Left ventricular late enhancement was identified by contrast-enhanced cardiac magnetic resonance in 93% of patients versus 14% of control subjects (P<0.001), with a regional distribution overlapping the histopathology findings in SCD cases.
CONCLUSIONS—MVP is an underestimated cause of arrhythmic SCD, mostly in young adult women. Fibrosis of the papillary muscles and inferobasal left ventricular wall, suggesting a myocardial stretch by the prolapsing leaflet, is the structural hallmark and correlates with ventricular arrhythmias origin. Contrast-enhanced cardiac magnetic resonance may help to identify in vivo this concealed substrate for risk stratification.
The original designation of “Arrhythmogenic right ventricular (dysplasia/) cardiomyopathy”(ARVC) was used by the scientists who first discovered the disease, in the pre-genetic and pre-cardiac ...magnetic resonance era, to describe a new heart muscle disease predominantly affecting the right ventricle, whose cardinal clinical manifestation was the occurrence of malignant ventricular arrhythmias. Subsequently, autopsy investigations, genotype-phenotype correlations studies and the increasing use of contrast-enhancement cardiac magnetic resonance showed that the fibro-fatty replacement of the myocardium represents the distinctive phenotypic feature of the disease that affects the myocardium of both ventricles, with left ventricular involvement which may parallel or exceed the severity of right ventricular involvement. This has led to the new designation of “Arrhythmogenic Cardiomyopathy” (ACM), that represents the evolution of the original term of ARVC. The present International Expert Consensus document proposes an upgrade of the criteria for diagnosis of the entire spectrum of the phenotypic variants of ACM. The proposed “Padua criteria” derive from the diagnostic approach to ACM, which has been developed over 30 years by the multidisciplinary team of basic researchers and clinical cardiologists of the Medical School of the University of Padua. The Padua criteria are a working framework to improve the diagnosis of ACM by introducing new diagnostic criteria regarding tissue characterization findings by contrast-enhanced cardiac magnetic resonance, depolarization/repolarization ECG abnormalities and ventricular arrhythmia features for diagnosis of the left ventricular phenotype. The proposed diagnostic criteria need to be further validated by future clinical studies in large cohorts of patients.
•Fibro-fatty myocardial replacement is the distinctive phenotypic feature of Arrhythmogenic cardiomyopathy (ACM);•ACM can affect both the right and left ventricle;•The 2010 International Task Force diagnostic criteria lacked specific criteria for diagnosis of left-sided variants of ACM;•The proposed Padua diagnostic criteria encompass dominant-right, biventricular and dominant-left ACM.
Prediction of major arrhythmic events (MAEs) in dilated cardiomyopathy represents an unmet clinical goal. Computational models and artificial intelligence (AI) are new technological tools that could ...offer a significant improvement in our ability to predict MAEs. In this proof-of-concept study, we propose a deep learning (DL)-based model, which we termed Deep ARrhythmic Prevention in dilated cardiomyopathy (DARP-D), built using multidimensional cardiac magnetic resonance data (cine videos and hypervideos and LGE images and hyperimages) and clinical covariates, aimed at predicting and tracking an individual patient's risk curve of MAEs (including sudden cardiac death, cardiac arrest due to ventricular fibrillation, sustained ventricular tachycardia lasting ≥30 s or causing haemodynamic collapse in <30 s, appropriate implantable cardiac defibrillator intervention) over time. The model was trained and validated in 70% of a sample of 154 patients with dilated cardiomyopathy and tested in the remaining 30%. DARP-D achieved a 95% CI in Harrell's C concordance indices of 0.12-0.68 on the test set. We demonstrate that our DL approach is feasible and represents a novelty in the field of arrhythmic risk prediction in dilated cardiomyopathy, able to analyze cardiac motion, tissue characteristics, and baseline covariates to predict an individual patient's risk curve of major arrhythmic events. However, the low number of patients, MAEs and epoch of training make the model a promising prototype but not ready for clinical usage. Further research is needed to improve, stabilize and validate the performance of the DARP-D to convert it from an AI experiment to a daily used tool.
PDF
