scholarly journals Noncompaction of the Left Ventricular Myocardium: A Cause of Syncope in a Young Patient

2013 ◽  
Vol 1 (3) ◽  
pp. 32
Author(s):  
Deephak Swaminath ◽  
Ragesh Panikkath ◽  
Roshni Narayanan ◽  
Sam Copeland

Noncompaction of the left ventricular myocardium (LVNC) is a rare cardiomyopathywith a spectrum of clinical presentations. The pathogenesis of this type of cardiomyopathyinvolves an arrest in the process of compaction during embryonic life. Identificationof high risk individuals with this syndrome is challenging; it can cause presentationsranging from an asymptomatic state to malignant ventricular arrhythmias and suddendeath. We present a 19-year-old man who presented with syncope and was later diagnosedwith left ventricular noncompaction.

Author(s):  
V. I. Skidan ◽  
Kh. A. Bsharat ◽  
Yu. I. Aseeva ◽  
G. P. Nartsissova ◽  
E. N. Pavlyukova

There is a presentation of the results of a two-year follow-up after surgical treatment of a patient with chronic heart failure and a permanent form of atrial fibrillation, in which isolated apex hypoplasia and non-compaction of left ventricular myocardium were revealed.


2018 ◽  
Vol 69 (8) ◽  
pp. 2209-2212
Author(s):  
Alexandru Radu Mihailovici ◽  
Vlad Padureanu ◽  
Carmen Valeria Albu ◽  
Venera Cristina Dinescu ◽  
Mihai Cristian Pirlog ◽  
...  

Left ventricular noncompaction is a primary cardiomyopathy with genetic transmission in the vast majority of autosomal dominant cases. It is characterized by the presence of excessive myocardial trabecularities that generally affect the left ventricle. In diagnosing this condition, echocardiography is the gold standard, although this method involves an increased risk of overdiagnosis and underdiagnosis. There are also uncertain cases where echocardiography is inconclusive, a multimodal approach is needed, correlating echocardiographic results with those obtained by magnetic resonance imaging. The clinical picture may range from asymptomatic patients to patients with heart failure, supraventricular or ventricular arrhythmias, thromboembolic events and even sudden cardiac death. There is no specific treatment of left ventricular noncompaction, but the treatment is aimed at preventing and treating the complications of the disease. We will present the case of a young patient with left ventricular noncompactioncardiomyopathy and highlight the essential role of transthoracic echocardiography in diagnosing this rare heart disease.


Medicina ◽  
2010 ◽  
Vol 46 (3) ◽  
pp. 193 ◽  
Author(s):  
Sigita Glaveckaitė ◽  
Kęstutis Ručinskas ◽  
Jelena Čelutkienė ◽  
Vytė Maneikienė ◽  
Diana Zakarkaitė ◽  
...  

Isolated noncompaction of the ventricular myocardium is defined as a rare cardiomyopathy caused by intrauterine arrest of compaction of the myocardial fibers and meshwork, an important process in myocardial development, in absence of any coexisting congenital heart lesions. A lot of controversies exist about diagnostic criteria, nomenclature, origin, pathogenesis, and prognosis of this disease. Here, we describe an adult patient with isolated left ventricular noncompaction who presented with worsening congestive heart failure and was successfully treated with heart transplantation.


Heart Rhythm ◽  
2017 ◽  
Vol 14 (2) ◽  
pp. 166-175 ◽  
Author(s):  
Daniele Muser ◽  
Jackson J. Liang ◽  
Walter RT Witschey ◽  
Rajeev K. Pathak ◽  
Simon Castro ◽  
...  

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Despina Toader ◽  
Alina Paraschiv ◽  
Petrișor Tudorașcu ◽  
Diana Tudorașcu ◽  
Constantin Bataiosu ◽  
...  

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.


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
D M Adamczak ◽  
M Bednarski ◽  
A Rogala ◽  
M Antoniak ◽  
T Kiebalo ◽  
...  

Abstract BACKGROUND Hypertrophic cardiomyopathy (HCM) is a heart disease characterized by hypertrophy of the left ventricular myocardium. The disease is the most common cause of sudden cardiac death (SCD) in young people and competitive athletes due to fatal ventricular arrhythmias, but in most patients, however, HCM has a benign course. Therefore, it is of the utmost importance to properly evaluate patients and identify those who would benefit from a cardioverter-defibrillator (ICD) implantation. The HCM SCD-Risk Calculator is a useful tool for estimating the 5-year risk of SCD. Parameters included in the model at evaluation are: age, maximum left ventricular wall thickness, left atrial dimension, maximum gradient in left ventricular outflow tract, family history of SCD, non-sustained ventricular tachycardia and unexplained syncope. Patients’ risk of SCD is classified as low (<4%), intermediate (4-<6%) or high (≥6%). Those in the high-risk group should have an ICD implantation. It can also be considered in the intermediate-risk group. However, the calculator still needs improvement and machine learning (ML) has the potential to fulfill this task. ML algorithm creates a model for solving a specific problem without explicit programming - instead it relies only on available data - by discovering patterns and relations. METHODS 252 HCM patients (aged 20-88 years, 49,6% were men) treated in our Department from 2005 to 2018, have been enrolled. The follow-up lasted 0-13 years (average: 3.8 years). SCD was defined as sudden cardiac arrest (SCA) or an appropriate ICD intervention. All parameters from HCM SCD-Risk Calculator have been obtained and the risk of SCD has been calculated for all patients during the first echocardiographic evaluation. ML model with variables from HCM SCD-Risk Calculator has been created. Both methods have been compared. RESULTS 20 patients reached an SCD end-point. 1 patient died due to SCA and 19 had an appropriate ICD intervention. Among them, there were respectively 6, 7 and 7 patients in the low, intermediate and high-risk group of SCD. 1 patient, who died, had a low risk. The ML model correctly assessed the SCD event only in 1 patient. According to ML, the risk of SCD ≤2.07% was a negative predictor. CONCLUSIONS The study did not show an advantage of ML over HCM SCD-Risk Calculator. Because of the characteristic of the dataset (approximately the same number of features and observations), the selection of machine learning algorithms was limited. Best results (evaluated using LOOCV) were achieved with a decision tree. We expect that bigger dataset would allow improving model performance because of strong regularization need in the current setup.


Heart Rhythm ◽  
2020 ◽  
Author(s):  
Juan José Sánchez Muñoz ◽  
Carmen Muñoz Esparza ◽  
Pablo Peñafiel Verdú ◽  
Juan Martínez Sánchez ◽  
Francisco García Almagro ◽  
...  

2011 ◽  
Vol 5 ◽  
pp. CMC.S6240 ◽  
Author(s):  
Tariq Bhat ◽  
James Lafferty ◽  
Sumaya Teli ◽  
Georges Abou Rjaili ◽  
Yefim Olkovsky ◽  
...  

Isolated noncompaction of the ventricular myocardium has often been misdiagnosed as other cardiomyopathies because it is a relatively recently described cardiomyopathy with literature limited to case reports and case series and little awareness among physicians. We are reporting a case of isolated left ventricular noncompaction cardiomyopathy that was misdiagnosed for over two decades.


2017 ◽  
Vol 44 (4) ◽  
pp. 260-263 ◽  
Author(s):  
Kyriacos Papadopoulos ◽  
Petros M. Petrou ◽  
Demos Michaelides

Isolated ventricular noncompaction, a rare genetic cardiomyopathy, is thought to be caused by the arrest of normal myocardial morphogenesis. It is characterized by prominent, excessive trabeculation in a ventricular wall segment and deep intertrabecular recesses perfused from the ventricular cavity. The condition can present with heart failure, systematic embolic events, and ventricular arrhythmias. Two-dimensional echocardiography is the typical diagnostic method. We report a case of heart failure in a 35-year-old man who presented with palpitations. Two-dimensional echocardiograms revealed left ventricular noncompaction, which markedly improved after standard heart failure therapy.


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