scholarly journals Noncompaction of Ventricular Wall - A Threat to Life

2012 ◽  
Vol 7 (1) ◽  
pp. 39-41
Author(s):  
Nilufar Fatema ◽  
Ahmed Saiful Bari ◽  
Naznin Sultana ◽  
Mohammad Al Mamun ◽  
SM Mustafa Sultan ◽  
...  

Ventricular noncompaction is a kind of cardiomyopathy which is called Noncompaction Cardiomyopathy (NCC). In this condition in which the muscular wall of the main pumping chamber of the heart -the left ventricle (LV) appears to be spongy and “non-compacted”, consisting of a meshwork of numerous muscle bands called trabeculations. Here, anatomically LV wall has deep trabeculations. This condition is associated major clinical problems like systolic and diastolic dysfunction, arrhythmia and even systemic embolism. Sudden cardiac death may occur in this group. Early detection of this condition may help to plan the management. DOI: http://dx.doi.org/10.3329/uhj.v7i1.10209 UHJ 2011; 7(1): 39-41

Author(s):  
Marianna Leopoulou ◽  
Jo Ann LeQuang ◽  
Joseph V. Pergolizzi ◽  
Peter Magnusson

Dilated cardiomyopathy (DCM) is characterized by the phenotype of a dilated left ventricle with systolic dysfunction. It is classified as hereditary when it is deemed of genetic origin; more than 50 genes are reported to be related to the condition. Symptoms include, among others, dyspnea, fatigue, arrhythmias, and syncope. Unfortunately, sudden cardiac death may be the first manifestation of the disease. Risk stratification regarding sudden death in hereditary DCM as well as preventive management poses a challenge due to the heterogeneity of the disease. The purpose of this chapter is to present the epidemiology, risk stratification, and preventive strategies of sudden cardiac death in hereditary DCM.


Author(s):  
R. W. Roudijk ◽  
K. Taha ◽  
M. Bourfiss ◽  
P. Loh ◽  
L. van den Heuvel ◽  
...  

AbstractIn relatives of index patients with dilated cardiomyopathy and arrhythmogenic cardiomyopathy, early detection of disease onset is essential to prevent sudden cardiac death and facilitate early treatment of heart failure. However, the optimal screening interval and combination of diagnostic techniques are unknown. The clinical course of disease in index patients and their relatives is variable due to incomplete and age-dependent penetrance. Several biomarkers, electrocardiographic and imaging (echocardiographic deformation imaging and cardiac magnetic resonance imaging) techniques are promising non-invasive methods for detection of subclinical cardiomyopathy. However, these techniques need optimisation and integration into clinical practice. Furthermore, determining the optimal interval and intensity of cascade screening may require a personalised approach. To address this, the CVON-eDETECT (early detection of disease in cardiomyopathy mutation carriers) consortium aims to integrate electronic health record data from long-term follow-up, diagnostic data sets, tissue and plasma samples in a multidisciplinary biobank environment to provide personalised risk stratification for heart failure and sudden cardiac death. Adequate risk stratification may lead to personalised screening, treatment and optimal timing of implantable cardioverter defibrillator implantation. In this article, we describe non-invasive diagnostic techniques used for detection of subclinical disease in relatives of index patients with dilated cardiomyopathy and arrhythmogenic cardiomyopathy.


2021 ◽  
Vol 9 (11) ◽  
pp. 643-648
Author(s):  
Nava Udaya N.R ◽  
◽  
Ramiah Rajesh Kannan ◽  
Srikanth Moorthy ◽  
Resmi Sekhar ◽  
...  

Background: There are many causes of left ventricular hypertrophy which can result in arrhythmias and sudden cardiac death. Hypertrophic cardiomyopathy (HCM) is one of the commonly encountered cause of sudden cardiac death in young adults. Aim: Identifying the role of Cardiac MRI in characterising the diagnostic parameters of HCM. Materials and methods: 125 patients with clinical suspicion or genetic evidence of HCM referred for cardiac MRI between June 2013 to June 2021 were included under the study. Image interpretations were done by fellowship qualified cardiac imaging radiologist. Categorical variables were expressed using frequency and percentage. Numerical variables were presented using mean and standard deviation. Results: Out of the total population, 119 patients (95 %) had LV wall thickness > 13 mm, 48 patients (38.4%) had Left ventricle outflow tract obstruction (LVOTO) and 32 patients (25.6 %) had mid cavity obstruction, 39 patients (37.9 %) had myocardial scar > 15 % and asymmetric septal hypertrophy was the most frequently encountered left ventricle morphology Conclusion: Cardiac MRI detected HCM has a statistically significant association with the final diagnosis (histopathological and genetic correlation). CMRI hence serves as a reliable tool in identifying and characterising the various diagnostic and non- diagnostic parameters of HCM.


2006 ◽  
Vol 134 (11-12) ◽  
pp. 482-487 ◽  
Author(s):  
Branislav Milovanovic ◽  
Mirjana Krotin ◽  
Dejana Vukovic ◽  
Vesna Bisenic ◽  
Tijana Mirjanic ◽  
...  

Introduction: It has been shown that depolarization disorders, autonomic dysfunction, and systolic dysfunction of the left ventricle are associated with sudden cardiac death after myocardial infarction. Objective: The Objective of study was to examine the prognostic value of the most important predictors in the first week after myocardial infarction. Method: Study included 881 patients who were followed up from 1 to 60 months. During the first week after myocardial infarction, following examination were performed: ECG with standard leads and X, Y, Z orthogonal leads, vectorcardiogram, QT interval, late potentials, short-time spectral analysis of RR variability, nonlinear (Poincare plot) analysis and echocardiogram. Results: In univariate analysis, the following parameters measured on the first day were important predictors of sudden cardiac death: lower LF/HF ratio(<1.5) (p=0.000), T wave inversion in X lead (p=0.000), high P wave in D2 lead (p=0.030), and diminished systolic function (p=0.000). In multivariate analysis, the following parameters were significant risk predictors: T wave inversion in X lead, lower LF/HF ratio, positive late potentials and the left ventricle systolic dysfunction. Conclusion: The parameters of the left ventricle systolic dysfunction with sympathicovagal imbalance and electric instability are the key risk predictors in the first few days after myocardial infarction.


2021 ◽  
Author(s):  
Enkhsaikhan Purevjav ◽  
Michelle Chintanaphol ◽  
Buyan-Ochir Orgil ◽  
Nelly R. Alberson ◽  
Jeffrey A. Towbin

Cardiomyopathy or disease of the heart muscle involves abnormal enlargement and a thickened, stiff, or spongy-like appearance of the myocardium. As a result, the function of the myocardium is weakened and does not sufficiently pump blood throughout the body nor maintain a normal pumping rhythm, leading to heart failure. The main types of cardiomyopathies include dilated hypertrophic, restrictive, arrhythmogenic, and noncompaction cardiomyopathy. Abnormal trabeculations of the myocardium in the left ventricle are classified as left ventricular noncompaction cardiomyopathy (LVNC). Myocardial noncompaction most frequently is observed at the apex of the left ventricle and can be associated with chamber dilation or muscle hypertrophy, systolic or diastolic dysfunction, or both, or various forms of congenital heart disease. Animal models are incredibly important for uncovering the etiology and pathogenesis involved in this disease. This chapter will describe the clinical and pathological features of LVNC in humans and present the animal models that have been used for the study of the genetic basis and pathogenesis of this disease.


Cor et Vasa ◽  
2017 ◽  
Vol 59 (5) ◽  
pp. e457-e459
Author(s):  
Oleg Libryk ◽  
Igor Vakaljuk ◽  
Elina Warenytsia ◽  
Andriy Vytryhovskiy

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