scholarly journals Arrhythmogenic Left Ventricular Cardiomyopathy: Genotype-Phenotype Correlations and New Diagnostic Criteria

2021 ◽  
Vol 10 (10) ◽  
pp. 2212
Author(s):  
Giulia Mattesi ◽  
Alberto Cipriani ◽  
Barbara Bauce ◽  
Ilaria Rigato ◽  
Alessandro Zorzi ◽  
...  
Keyword(s):  
Task Force ◽  
Current Knowledge ◽  
Critical Evaluation ◽  
Original Description ◽  
Ventricular Myocardium ◽  
Left Ventricular ◽  
Muscle Disease ◽  
Dominant Form ◽  
Consensus Document ◽  
The Right

Arrhythmogenic cardiomyopathy (ACM) is an inherited heart muscle disease characterized by loss of ventricular myocardium and fibrofatty replacement, which predisposes to scar-related ventricular arrhythmias and sudden cardiac death, particularly in the young and athletes. Although in its original description the disease was characterized by an exclusive or at least predominant right ventricle (RV) involvement, it has been demonstrated that the fibrofatty scar can also localize in the left ventricle (LV), with the LV lesion that can equalize or even overcome that of the RV. While the right-dominant form is typically associated with mutations in genes encoding for desmosomal proteins, other (non-desmosomal) mutations have been showed to cause the biventricular and left-dominant variants. This has led to a critical evaluation of the 2010 International Task Force criteria, which exclusively addressed the right phenotypic manifestations of ACM. An International Expert consensus document has been recently developed to provide upgraded criteria (“the Padua Criteria”) for the diagnosis of the whole spectrum of ACM phenotypes, particularly left-dominant forms, highlighting the use of cardiac magnetic resonance. This review aims to offer an overview of the current knowledge on the genetic basis, the phenotypic expressions, and the diagnosis of left-sided variants, both biventricular and left-dominant, of ACM.

scholarly journals Myocardial infarction remodeling that progresses to heart failure: a signaling misunderstanding

2018 ◽  
Vol 315 (1) ◽  
pp. H71-H79 ◽  
Author(s):  
Alan J. Mouton ◽  
Osvaldo J. Rivera ◽  
Merry L. Lindsey
Keyword(s):  
Myocardial Infarction ◽  
Heart Failure ◽  
Wound Healing ◽  
Current Knowledge ◽  
Healing Process ◽  
Cell Types ◽  
Left Ventricular ◽  
Wound Healing Process ◽  
The Status ◽  
The Right

After myocardial infarction, remodeling of the left ventricle involves a wound-healing orchestra involving a variety of cell types. In order for wound healing to be optimal, appropriate communication must occur; these cells all need to come in at the right time, be activated at the right time in the right amount, and know when to exit at the right time. When this occurs, a new homeostasis is obtained within the infarct, such that infarct scar size and quality are sufficient to maintain left ventricular size and shape. The ideal scenario does not always occur in reality. Often, miscommunication can occur between infarct and remote spaces, across the temporal wound-healing spectrum, and across organs. When miscommunication occurs, adverse remodeling can progress to heart failure. This review discusses current knowledge gaps and recent development of the roles of inflammation and the extracellular matrix in myocardial infarction remodeling. In particular, the macrophage is one cell type that provides direct and indirect regulation of both the inflammatory and scar-forming responses. We summarize current research efforts focused on identifying biomarker indicators that reflect the status of each component of the wound-healing process to better predict outcomes.

scholarly journals Title：Early assessment of ventricular synchronization and function after left bundle-branch-area pacing with right bundle-branch block

2021 ◽  
Author(s):  
Ruohan Zhao ◽  
Feng Xiong ◽  
Xiaoqi Deng ◽  
Shuzhen Wang ◽  
Chunxia Liu ◽  
...  
Keyword(s):  
Standard Deviation ◽  
Ventricular Function ◽  
Right Bundle Branch Block ◽  
Longitudinal Strain ◽  
Ventricular Myocardium ◽  
Left Ventricular ◽  
Peak Strain ◽  
Bundle Branch Block ◽  
The Right ◽  
And Function

Aim To evaluate ventricular synchronization and function in patients with right bundle-branch block after left bundle-branch-area pacing (LBBAP) by echocardiography. Methods Forty patients who successfully received LBBAP were selected and divided into the right bundle-branch block group (RBBB group) and the non-RBBB group by pre-operation ECG. Echocardiography and follow-up were performed 1 month after operation. Interventricular synchronization was evaluated by tissue Doppler (TDI), tissue mitral annular displacement (TMAD), and interventricular mechanical delay (IVMD). The ventricular longitudinal strain and the standard deviation of peak time of longitudinal strain were analyzed by two-dimensional speckle tracking imaging (2D-STI) to evaluate intraventricular synchronization and ventricular function. Results (1) The deviation of systolic time to the peak of the tricuspid and mitral valves, namely ΔPTTV-MV measured by TMAD and ΔTsTV-MV measured by TDI, were statistically different between the two groups (P < 0.05). (2) Compared with the non-RBBB group, there were no statistically significant differences in longitudinal strain (LS), peak strain time, standard deviation of peak strain time (SDt), and global longitudinal strain (GLS) in the right and left ventricle in the RBBB group (P > 0.05). Conclusion Echocardiography technology including 2D-STI, TDI, and TMAD can effectively analyze interventricular synchronization, intraventricular synchronization, and ventricular function. Although the movement of the right ventricular myocardium in the RBBB group treatment was slightly later than that of the left ventricular myocardium after LBBAP, LBBAP is still an effective pacing therapy for RBBB patients with pacing indication.

scholarly journals Arrhythmogenic Cardiomyopathy

2019 ◽  
Vol 87 (11-12) ◽  
pp. 599-618
Author(s):  
Blaž Podgoršek ◽  
Gregor Poglajen ◽  
Andraž Cerar ◽  
Matjaž Šinkovec ◽  
Bojan Vrtovec
Keyword(s):  
Task Force ◽  
Current Knowledge ◽  
Diagnostic Yield ◽  
Treatment Strategies ◽  
Arrhythmogenic Cardiomyopathy ◽  
Icd Implantation ◽  
Fatty Replacement ◽  
Current Task ◽  
Advanced Stages ◽  
The Right

Arrhythmogenic cardiomyopathy (AC) is a genetic disease of the myocardium characterized by fibro-fatty replacement of the apoptotic myocardium. It primarily affects the right ventricle, however in advanced stages of the disease the left ventricle can also be significantly affected. AC is a challenging diagnosis, especially in the early stages of the disease, and should be considered in all patients presenting with palpitations, syncope or sudden cardiac death when other, more common causes of these symptoms/signs are excluded. In patients with suspected AC, evaluation according to the current Task Force Criteria should be applied to achieve optimal diagnostic yield. The main therapeutic concern in AC patients is the prevention of SCD, and thus all patients with established diagnosis have to be evaluated for potential ICD implantation, which is indicated in the majority of symptomatic patients. In this narrative review we aim to outline current knowledge on the pathophysiology, diagnosis and treatment strategies of AC.

scholarly journals Possibly Late-Onset Arrhythmogenic Right Ventricular Cardiomyopathy: Unique Triglyceride Deposition by Analysis of Lipid Contents

2019 ◽  
Vol 12 ◽  
pp. 117954761982871
Author(s):  
Kentaro Yamamoto ◽  
Xin Guo ◽  
Ken-ichi Mizutani ◽  
Nozomu Kurose ◽  
Motona Kumagai ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Late Onset ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Ventricular Myocardium ◽  
Cholesterol Content ◽  
Left Ventricular ◽  
Left Ventricular Myocardium ◽  
Ventricular Cardiomyopathy ◽  
Lipid Contents ◽  
The Right

We presented an unusual arrhythmogenic right ventricular cardiomyopathy (ARVC) case of a late-60s elderly man’s death, due to severe pericardial/pleural effusion and ascites, and arrhythmic events, with unique pathological features. The hypertrophic heart grossly displayed yellowish to yellow-whitish predominantly in the variably thinned wall of the dilated right ventricle. Microscopic findings showed diffuse fatty/fibrofatty replacement in not only the right but left ventricular myocardium, together with an outer lymphoplasmacytic infiltrate. According to the lipid contents analysis, the triglyceride content, but not the cholesterol content, in our patient’s right and left ventricular cardiac muscle was much higher than that in the control subject. We propose that this unique triglyceride deposition in our possibly late-onset ARVC case might be one of new clues to understand its enigmatic cause. Further prospective studies are needed to validate the presence and significance of a greater volume of triglyceride deposit, after collecting and investigating a larger number of early and late-onset ARVC cases examined.

Noncompaction of the myocardium associated with Roifman syndrome

2001 ◽  
Vol 11 (2) ◽  
pp. 240-243 ◽  
Author(s):  
Karen Mandel ◽  
Eyal Grunebaum ◽  
Lee Benson
Keyword(s):  
Interventricular Septum ◽  
Retinal Dystrophy ◽  
Ventricular Myocardium ◽  
Left Ventricular ◽  
Antibody Deficiency ◽  
Left Ventricular Myocardium ◽  
Facial Dysmorphism ◽  
Ventricular Wall ◽  
Immune Deficiencies ◽  
The Right

Noncompaction of the ventricular myocardium, sometimes referred to as “spongy myocardium”, appears as excessive and prominent trabeculations and deep intratrabecular recesses within the ventricular wall, usually involving the left ventricle, although the right ventricle and interventricular septum can also be affected. It may occur with or without additional heart malformations. Roifman syndrome is a constellation of antibody deficiency, spondyloepiphyseal dysplasia, facial dysmorphism, growth retardation, and retinal dystrophy. We report a patient with Roifman syndrome who presented with noncompaction of the left ventricular myocardium. Our findings expand the spectrum of diseases associated with noncompaction. The recognition of noncompaction among patients with Roifman syndrome is important, as the immune deficiencies may be subtle and undiagnosed until adulthood. Thus, some patients may first present with cardiac failure.

Laminar structure of the heart: ventricular myocyte arrangement and connective tissue architecture in the dog

1995 ◽  
Vol 269 (2) ◽  
pp. H571-H582 ◽  
Author(s):  
I. J. LeGrice ◽  
B. H. Smaill ◽  
L. Z. Chai ◽  
S. G. Edgar ◽  
J. B. Gavin ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Three Dimensional ◽  
Length Distribution ◽  
Ventricular Myocardium ◽  
Left Ventricular ◽  
Tangential Section ◽  
Connective Tissue Matrix ◽  
The Right ◽  
Tissue Matrix ◽  
Number Of Branches

We have studied the three-dimensional arrangement of ventricular muscle cells and the associated extracellular connective tissue matrix in dog hearts. Four hearts were potassium-arrested, excised, and perfusion-fixed at zero transmural pressure. Full-thickness segments were cut from the right and left ventricular walls at a series of precisely located sites. Morphology was visualized macroscopically and with scanning electron microscopy in 1) transmural planes of section and 2) planes tangential to the epicardial surface. The appearance of all specimens was consistent with an ordered laminar arrangement of myocytes with extensive cleavage planes between muscle layers. These planes ran radially from endocardium toward epicardium in transmural section and coincided with the local muscle fiber orientation in tangential section. Stereological techniques were used to quantify aspects of this organization. There was no consistent variation in the cellular organization of muscle layers (48.4 +/- 20.4 microns thick and 4 +/- 2 myocytes across) transmurally or in different ventricular regions (23 sites in 6 segments), but there was significant transmural variation in the coupling between adjacent layers. The number of branches between layers decreased twofold from subepicardium to midwall, whereas the length distribution of perimysial collagen fibers connecting muscle layers was greatest in the midwall. We conclude that ventricular myocardium is not a uniformly branching continuum but a laminar hierarchy in which it is possible to identify three axes of material symmetry at any point.

Absence of the left ventricular myocardium in a newborn

1996 ◽  
Vol 6 (4) ◽  
pp. 344-347 ◽  
Author(s):  
Angelika Lindinger ◽  
Yvonne Masur ◽  
Hans-Gerhard Limbach
Keyword(s):  
Aortic Valve ◽  
Left Ventricle ◽  
Ventricular Myocardium ◽  
Arrhythmogenic Right Ventricular Dysplasia ◽  
Left Ventricular ◽  
Adolescent And Young Adult ◽  
Left Ventricular Myocardium ◽  
Fatty Replacement ◽  
Myocardial Fibers ◽  
The Right

SummaryAbsence of myocardial fibers in the right ventricle is the essence of so-called Uhl's anomaly, which should be distinguished from the fatty replacement producing arrhythmogenic right ventricular dysplasia of the adolescent and young adult. In this report, we describe a newborn with nearly complete absence of the myocardium of the left ventricle. The infant died on the seventh day because of myocardial incompetence of the left ventricle, which was unable to open the aortic valve.

scholarly journals Arrhythmogenic Cardiomyopathy—Current Treatment and Future Options

2021 ◽  
Vol 10 (13) ◽  
pp. 2750
Author(s):  
Federico Migliore ◽  
Giulia Mattesi ◽  
Alessandro Zorzi ◽  
Barbara Bauce ◽  
Ilaria Rigato ◽  
...  
Keyword(s):  
Ventricular Arrhythmias ◽  
Ventricular Dysfunction ◽  
Original Description ◽  
Current Treatment ◽  
Muscle Disease ◽  
Arrhythmogenic Cardiomyopathy ◽  
The Right ◽  
Selection Of Patients ◽  
Selection Of ◽  
Myocardial Lesions

Arrhythmogenic cardiomyopathy (ACM) is an inheritable heart muscle disease characterised pathologically by fibrofatty myocardial replacement and clinically by ventricular arrhythmias (VAs) and sudden cardiac death (SCD). Although, in its original description, the disease was believed to predominantly involve the right ventricle, biventricular and left-dominant variants, in which the myocardial lesions affect in parallel or even mostly the left ventricle, are nowadays commonly observed. The clinical management of these patients has two main purposes: the prevention of SCD and the control of arrhythmic and heart failure (HF) events. An implantable cardioverter defibrillator (ICD) is the only proven lifesaving treatment, despite significant morbidity because of device-related complications and inappropriate shocks. Selection of patients who can benefit the most from ICD therapy is one of the most challenging issues in clinical practice. Risk stratification in ACM patients is mostly based on arrhythmic burden and ventricular dysfunction severity, although other clinical features resulting from electrocardiogram and imaging modalities such as cardiac magnetic resonance may have a role. Medical therapy is crucial for treatment of VAs and the prevention of negative ventricular remodelling. In this regard, the efficacy of novel anti-HF molecules and drugs acting on the inflammatory pathway in patients with ACM is, to date, unknown. Catheter ablation represents an effective strategy to treat ventricular tachycardia relapses and recurrent ICD shocks. The present review will address the current strategies for prevention of SCD and treatment of VAs and HF in patients with ACM.

Sign in / Sign up

Export Citation Format

Share Document