Mitral Annular Disjunction in the Context of Mitral Valve Prolapse: Identifying the At-Risk Patient.

Mitral annular disjunction (MAD), a separation between the left atrium/mitral valve annulus and the left ventricular myocardium, is frequently seen in patients with arrhythmic mitral valve prolapse. Although an association exists between MAD and ventricular arrhythmias, little is known regarding the identification of individuals at high risk. Multimodality imaging including echocardiography, computed tomography, cardiac magnetic resonance, and positron emission tomography can play an important role in both the diagnosis and risk stratification of MAD. Due to a paucity of data, clinical decision making in a patient with MAD is challenging and remains largely empirical. Although MAD itself can be corrected surgically, the prevention and treatment of associated arrhythmias may require medical therapy, catheter ablation, and an implantable cardioverter-defibrillator. Prospective data are required to define the role of implantable cardioverter-defibrillators, targeted catheter ablation, and surgical correction in selected, at-risk patients.

Epidemiology, pathophysiology, diagnosis and management of chronic right-sided heart failure and tricuspid regurgitation. A clinical consensus statement of the Heart Failure Association (HFA) and the European Association of Percutaneous Cardiovascular Interventions (EAPCI) of the ESC.

Right-sided heart failure and tricuspid regurgitation are common and strongly associated with poor quality of life and an increased risk of heart failure hospitalizations and death. While medical therapy for right-sided heart failure is limited, treatment options for tricuspid regurgitation include surgery and, based on recent developments, several transcatheter interventions. However, the patients who might benefit from tricuspid valve interventions are yet unknown, as is the ideal time for these treatments given the paucity of clinical evidence. In this context, it is crucial to elucidate aetiology and pathophysiological mechanisms leading to right-sided heart failure and tricuspid regurgitation in order to recognize when tricuspid regurgitation is a mere bystander and when it can cause or contribute to heart failure progression. Notably, early identification of right heart failure and tricuspid regurgitation may be crucial and optimal management requires knowledge about the different mechanisms and causes, clinical course and presentation, as well as possible treatment options. The aim of this clinical consensus statement is to summarize current knowledge about epidemiology, pathophysiology and treatment of tricuspid regurgitation in right-sided heart failure providing practical suggestions for patient identification and management.

Multimodality Imaging Evaluation to Detect Subtle Right Ventricular Involvement in Patients with Acute Myocarditis and Preserved Left Ventricular Ejection Fraction.

Background: Evaluation of the right ventricle (RV) in patients with acute myocarditis (MY) remains challenging with both 2D transthoracic echocardiography (TTE) and cardiovascular magnetic resonance (CMR). We examined the incremental diagnostic value of CMR feature tracking (FT) to evaluate RV involvement in patients with myocarditis. Methods: We enrolled 54 patients with myocarditis and preserved left ventricle (LV) ejection fraction (EF). The CMR protocol included T2-weighted images for edema detection and late gadolinium enhancement (LGE) images. Global longitudinal strain (GLS) of the left ventricle (LV) and RV free wall strain (CMR-FWS) were obtained with CMR-FT. We identified 34 patients (62%) with inferior and lateral segment (IL-MY) involvement and 20 (38%) noIL-MY in case of any other myocardial segment involved. Here, 20 individuals who underwent CMR for suspected cardiac disease, which was not confirmed thereafter, were considered as the control population. Results: TTE and CMR showed normal RV function in all patients without visible RV involvement at the LGE or T2-weighted sequences. At CMR, LV-GLS values were significantly lower in patients with MY compared to the control group (median -19.0% vs. -21.0%, p = 0.029). Overall, CMR RV-FWS was no different between MY patients and controls (median -21.2% vs. -23.2 %, p = 0.201) while a significant difference was found between RV FWS in IL-MY and noIL-MY (median -18.17% vs. -24.2%, p = 0.004). Conclusions: CMR-FT has the potential to unravel subclinical RV involvement in patients with acute myocarditis, specifically in those with inferior and lateral injuries that exhibit lower RV-FWS values. In this setting, RV deformation analysis at CMR may be effectively implemented for a comprehensive functional assessment.

Multimodality imaging for patient selection, procedural guidance, and follow-up of transcatheter interventions for structural heart disease: a consensus document of the EACVI Task Force on Interventional Cardiovascular Imaging: part 1: access routes, transcatheter aortic valve implantation, and transcatheter mitral valve interventions.

Transcatheter therapies for the treatment of structural heart diseases (SHD) have expanded dramatically over the last years, thanks to the developments and improvements of devices and imaging techniques, along with the increasing expertise of operators. Imaging, in particular echocardiography, is pivotal during patient selection, procedural monitoring, and follow-up. The imaging assessment of patients undergoing transcatheter interventions places demands on imagers that differ from those of the routine evaluation of patients with SHD, and there is a need for specific expertise for those working in the cath lab. In the context of the current rapid developments and growing use of SHD therapies, this document intends to update the previous consensus document and address new advancements in interventional imaging for access routes and treatment of patients with aortic stenosis and regurgitation, and mitral stenosis and regurgitation.

Additional value of cardiac magnetic resonance feature tracking parameters for the evaluation of the arrhythmic risk in patients with mitral valve prolapse.

OBJECTIVES: The identification of patients with mitral valve prolapse (MVP) presenting high arrhythmic risk remains challenging. Cardiovascular Magnetic Resonance (CMR) feature tracking (FT) may improve risk stratification. We analyzed the role of CMR-FT parameters in relation to the incidence of complex ventricular arrhythmias (cVA) in patients with MVP and mitral annular disjunction (MAD). METHODS: 42 patients with MVP and MAD who underwent 1.5 T CMR were classified as MAD-cVA (n = 23, 55%) in case of cVA diagnosed on a 24-h Holter monitoring and as MAD-noVA in the absence of cVA (n = 19, 45%). MAD length, late gadolinium enhancement (LGE), basal segments myocardial extracellular volume (ECV) and CMR-FT were assessed. RESULTS: LGE was more frequent in the MAD-cVA group in comparison with the MAD-noVA group (78% vs 42%, p = 0.002) while no difference was observed in terms of basal ECV. Global longitudinal strain (GLS) was reduced in MAD-cVA compared to MAD-noVA (- 18.2% ± 4.6% vs - 25.1% ± 3.1%, p = 0.004) as well as global circumferential strain (GCS) at the mid-ventricular level (- 17.5% ± 4.7% vs - 21.6% ± 3.1%, p = 0.041). Univariate analysis identified as predictors of the incidence of cVA: GCS, circumferential strain (CS) in the basal and mid infero-lateral wall, GLS, regional longitudinal strain (LS) in the basal and mid-ventricular inferolateral wall. Reduced GLS [Odd ratio (OR):1.56 (confidence interval (CI) 95%: 1.45-2.47; p < 0.001)] and regional LS in the basal inferolateral wall [OR: 1.62 (CI 95%: 1.22-2.13; p < 0.001)] remained independent prognostic factors in multivariate analysis. CONCLUSION: In patients with MVP and MAD, CMR-FT parameters are correlated with the incidence of cVA and may be of interest in arrhythmic risk stratification.

Three-dimensional transoesophageal echocardiography: how to use and when to use-a clinical consensus statement from the European Association of Cardiovascular Imaging of the European Society of Cardiology.

Three-dimensional transoesophageal echocardiography (3D TOE) has been rapidly developed in the last 15 years. Currently, 3D TOE is particularly useful as an additional imaging modality for the cardiac echocardiographers in the echo-lab, for cardiac interventionalists as a tool to guide complex catheter-based procedures cardiac, for surgeons to plan surgical strategies, and for cardiac anaesthesiologists and/or cardiologists, to assess intra-operative results. The authors of this document believe that acquiring 3D data set should become a 'standard part' of the TOE examination. This document provides (i) a basic understanding of the physic of 3D TOE technology which enables the echocardiographer to obtain new skills necessary to acquire, manipulate, and interpret 3D data sets, (ii) a description of valvular pathologies, and (iii) a description of non-valvular pathologies in which 3D TOE has shown to be a diagnostic tool particularly valuable. This document has a new format: instead of figures randomly positioned through the text, it has been organized in tables which include figures. We believe that this arrangement makes easier the lecture by clinical cardiologists and practising echocardiographers.

The Role of Cardiovascular Magnetic Resonance in Patients with Mitral Regurgitation.

The 2019 Global Burden of Disease (GBD) study estimated that there were approximately 24.2 million people affected worldwide by degenerative mitral regurgitation (MR), resulting in 34,200 deaths. After aortic stenosis, MR is the most prevalent VHD in Europe and the second-most common VHD to pose indications for surgery in western countries. Current ESC and AHA/ACC guidelines for the management of VHD emphasize the importance of an integrative approach for the assessment of MR severity, which is of paramount importance in dictating the timing for surgery. Transthoracic echocardiography (TTE) and transesophageal echocardiography (TEE) are the first-line imaging modalities; however, despite the technological advancement, sometimes, the final diagnosis on the degree of the disease may still be challenging. In the last 20 years, CMR has emerged as a robust technique in the assessment of patients with cardiac disease, and, recently, its role is gaining more and more importance in the field of VHD. In fact, CMR is the gold standard in the assessment of cardiac volumes, and it is possible to accurately evaluate the regurgitant volume. The purpose of this review is to outline the current state-of-the-art management of MR by using Cardiac Magnetic Resonance (CMR).

Early Detection of Cardiotoxicity From Systemic and Radiation Therapy in Patients With Breast Cancer: Protocol for a Multi-Institutional Prospective Study.

BACKGROUND: The incidence of breast cancer is rising worldwide. Recent advances in systemic and local treatments have significantly improved survival rates of patients having early breast cancer. In the last decade, great attention has been paid to the prevention and early detection of cardiotoxicity induced by breast cancer treatments. Systemic therapy-related cardiac toxicities have been extensively studied. Radiotherapy, an essential component of breast cancer treatment, can also increase the risk of heart diseases. Consequently, it is important to balance the expected benefits of cancer treatment with cardiovascular risk and to identify strategies to prevent cardiotoxicity and improve long-term outcomes and quality of life for these patients. OBJECTIVE: This CardioTox Breast study aims to investigate the use of cardiac imaging, based on cardiac magnetic resonance and echocardiography, and to identify associated circulating biomarkers to assess early tissue changes in chemo-induced and radiation-induced cardiotoxicity in the time window of 12 months after the end of radiotherapy in patients with breast cancer. METHODS: The CardioTox Breast trial is a multicenter observational prospective longitudinal study. We aim to enroll 150 women with stage I-III unilateral breast cancer, treated with breast conserving surgery, who planned to receive radiotherapy with or without systemic therapy. Baseline and follow-up data include cardiac measurements based on cardiac magnetic resonance imaging, echocardiography, and circulating biomarkers of cardiac toxicity. RESULTS: This study details the protocol of the CardioTox Breast trial. Recruitment started in September 2020. The results of this study will not be published until data are mature for the final analysis of the primary study end point. CONCLUSIONS: The CardioTox Breast study is designed to investigate the effects of systemic and radiation therapy on myocardial function and structure, thus providing additional evidence on whether cardiac magnetic resonance is the optimal screening imaging for cardiotoxicity. TRIAL REGISTRATION: ClinicalTrials.gov NCT04790266; https://clinicaltrials.gov/ct2/show/NCT04790266. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/31887.

Imaging assessment of the right atrium: anatomy and function.

The right atrium (RA) is the cardiac chamber that has been least well studied. Due to recent advances in interventional cardiology, the need for greater understanding of the RA anatomy and physiology has garnered significant attention. In this article, we review how a comprehensive assessment of RA dimensions and function using either echocardiography, cardiac computed tomography, and magnetic resonance imaging may be used as a first step towards a better understanding of RA pathophysiology. The recently published normative data on RA size and function will likely shed light on RA atrial remodelling in atrial fibrillation (AF), which is a complex phenomenon that occurs in both atria but has only been studied in depth in the left atrium. Changes in RA structure and function have prognostic implications in pulmonary hypertension (PH), where the increased right ventricular (RV) afterload first induces RV remodelling, predominantly characterized by hypertrophy. As PH progresses, RV dysfunction and dilatation may begin and eventually lead to RV failure. Thereafter, RV overload and increased RV stiffness may lead to a proportional increase in RA pressure. This manuscript provides an in-depth review of RA anatomy, function, and haemodynamics with particular emphasis on the changes in structure and function that occur in AF, tricuspid regurgitation, and PH.

Morphology of Mitral Annular Disjunction in Mitral Valve Prolapse.

Mitral annular disjunction (MAD) is an abnormal insertion of the hinge line of the posterior mitral leaflet on the atrial wall: the mitral annulus shows a separation or "disjunction" between the leaflet-atrial wall junction and the crest of the left ventricle myocardium. This anomaly is often observed in patients with myxomatous mitral valve prolapse. The anatomical substrate of MAD remains unclear for the following reasons: (1) most studies are focused on the association between MAD and arrhythmias, rather than on pathomorphological aspects of MAD; and (2) the complex anatomic architecture of the posterior mitral annulus is often simply described as the posterior segment of a fibrous ring. The aims of this paper are to review the pertinent normal anatomy of the mitral valve and to propose new hypotheses on the morphological nature of MAD.

Multimodality Imaging of the Anatomy of Tricuspid Valve.

Even though the tricuspid valve is no longer "forgotten", it still remains poorly understood. In this review, we focus on some controversial and still unclear aspects of tricuspid anatomy as illustrated by noninvasive imaging techniques. In particular, we discuss the anatomical architecture of the so-called tricuspid annulus with its two components (i.e., the mural and the septal annulus), emphasizing the absence of any fibrous "ring" around the right atrioventricular junction. Then we discussed the extreme variability in number and size of leaflets (from two to six), highlighting the peculiarities of the septal leaflet as part of the septal atrioventricular junction (crux cordis). Finally, we describe the similarities and differences between the tricuspid and mitral valve, suggesting a novel terminology for tricuspid leaflets.

Multimodality Imaging of the Anatomy of the Aortic Root.

The aortic root has long been considered an inert unidirectional conduit between the left ventricle and the ascending aorta. In the classical definition, the aortic valve leaflets (similar to what is perceived for the atrioventricular valves) have also been considered inactive structures, and their motion was thought to be entirely passive-just driven by the fluctuations of ventricular-aortic gradients. It was not until the advent of aortic valve-sparing surgery and of transcatheter aortic valve implantation that the interest on the anatomy of the aortic root again took momentum. These new procedures require a systematic and thorough analysis of the fine anatomical details of the components of the so-called aortic valve apparatus. Although holding and dissecting cadaveric heart specimens remains an excellent method to appreciate the complex "three-dimensional" nature of the aortic root, nowadays, echocardiography, computed tomography, and cardiac magnetic resonance provide excellent images of cardiac anatomy both in two- and three-dimensional format. Indeed, modern imaging techniques depict the aortic root as it is properly situated within the thorax in an attitudinally correct cardiac orientation, showing a sort of "dynamic anatomy", which admirably joins structure and function. Finally, they are extensively used before, during, and after percutaneous structural heart disease interventions. This review focuses on the anatomy of the aortic root as revealed by non-invasive imaging techniques.

From secondary to tertiary mitral regurgitation: the paradigm shifts, but uncertainties remain.

Secondary mitral regurgitation (MR) is the most common and undertreated form of MR, whose contribution to poor prognosis and indications to correction remains under discussion. MR has been characterized into 'proportionate' or 'disproportionate', based on left ventricle (LV) and regurgitant volumes, whereas 'tertiary' MR identifies conditions, in which regurgitation is pathologic per se and actively contributes to LV dysfunction. Echocardiographic and anatomo-pathological studies revealed that secondary MR prompts subtle leaflet maladaptive changes, actively contributing to the dynamic progression of secondary MR. We critically discuss the paradigm shift from secondary to tertiary MR and question the notion that MV leaflets play a passive role in secondary MR. We also review the role of standard transthoracic echocardiography for appraising and quantifying maladaptive MV leaflet changes and LV volumes and call for a more sophisticated and comprehensive imaging framework for classifying MR in future interventional studies.

Transcatheter mitral valve interventions: pre-procedural planning and intra-procedural guidance.

Mitral regurgitation is the most prevalent valve disease worldwide. Percutaneous mitral valve interventions are emerging as alternative options for high-risk patients with severe mitral regurgitation not eligible for conventional surgery. Accurate patient selection is based on a detailed pre-procedural multimodality imaging evaluation. Morphology and functional anatomy of the mitral valve should be evaluated to determine the feasibility of treatments and to identify the best therapeutic approach. The procedures are guided by fluoroscopy, transesophageal echocardiography and fusion imaging, ensuring a continuous communication between the interventionalist and the imaging specialist. The present review will cover the pre-procedural planning and the intra-procedural guidance of the most used transcatheter approaches in the setting of native mitral valve regurgitation: edge to edge repair, percutaneous direct and indirect annuloplasty, transapical beating-heart chordal implantation and transcatheter mitral valve replacement.

The structural heart disease interventional imager rationale, skills and training: a position paper of the European Association of Cardiovascular Imaging.

Percutaneous therapeutic options for an increasing variety of structural heart diseases (SHD) have grown dramatically. Within this context of continuous expansion of devices and procedures, there has been increased demand for physicians with specific knowledge, skills, and advanced training in multimodality cardiac imaging. As a consequence, a new subspecialty of 'Interventional Imaging' for SHD interventions and a new dedicated professional figure, the 'Interventional Imager' with specific competencies has emerged. The interventional imager is an integral part of the heart team and plays a central role in decision-making throughout the patient pathway, including the appropriateness and feasibility of a procedure, pre-procedural planning, intra-procedural guidance, and post-procedural follow-up. However, inherent challenges exist to develop a training programme for SHD imaging that differs from traditional cardiovascular imaging pathways. The purpose of this document is to provide the standard requirements for the training in SHD imaging, as well as a starting point for an official certification process for SHD interventional imager.

Better Take a Second Look: The Fameless Face of Subacute Takotsubo Syndrome.

We have recently published in the journal the case of a 66-year-old female affected by typical Takotsubo syndrome (TTS) with apical ballooning, who presented important novel apical wall thickening despite normalization of left ventricular ejection fraction at a follow-up cardiac magnetic resonance (CMR) 1 month after the acute event. In the absence of significant elevated edema-sensitive T2 values at CMR, this constellation was interpreted as apical hypertrophic cardiomyopathy, initially mimicked by TTS. However, a routine late follow-up echocardiography and CMR after 6 months showed complete resolution of apical wall thickening. "Pseudohypertrophy" caused by transient significant myocardial edema seems to be a more frequent phenotype in the subacute phase of TTS than is yet known, which may cause diagnostic confusion.