Myocardial Noncompaction

2018 ◽  
Vol 69 (8) ◽  
pp. 2209-2212
Author(s):  
Alexandru Radu Mihailovici ◽  
Vlad Padureanu ◽  
Carmen Valeria Albu ◽  
Venera Cristina Dinescu ◽  
Mihai Cristian Pirlog ◽  
...  
Keyword(s):  
Ventricular Arrhythmias ◽  
Specific Treatment ◽  
Left Ventricular ◽  
Left Ventricular Noncompaction ◽  
Ventricular Noncompaction ◽  
Genetic Transmission ◽  
Asymptomatic Patients ◽  
Increased Risk ◽  
Patients With Heart Failure

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.

Ventricular arrhythmias associated with left ventricular noncompaction: Electrophysiologic characteristics, mapping, and ablation

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

scholarly journals Left ventricular noncompaction—a rare cause of triad: heart failure, ventricular arrhythmias, and systemic embolic events: a case report 

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Despina Toader ◽  
Alina Paraschiv ◽  
Petrișor Tudorașcu ◽  
Diana Tudorașcu ◽  
Constantin Bataiosu ◽  
...  
Keyword(s):  
Heart Failure ◽  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Left Ventricle ◽  
Ventricular Arrhythmias ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Left Ventricular Noncompaction ◽  
Ventricular Noncompaction ◽  
The Right

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.

scholarly journals P1441 Predictors of systemic embolisms in a large cohort of left ventricular noncompaction patients

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
G Casas ◽  
G Oristrell ◽  
J Limeres ◽  
R Barriales ◽  
J R Gimeno ◽  
...  
Keyword(s):  
Risk Factors ◽  
Atrial Fibrillation ◽  
Oral Anticoagulation ◽  
Systolic Dysfunction ◽  
Left Ventricular ◽  
Left Ventricular Noncompaction ◽  
Severe Phenotype ◽  
Ventricular Noncompaction ◽  
Increased Risk

Abstract BACKGROUND Left ventricular noncompaction (LVNC) is associated with an increased risk of systemic embolisms (SE). However, incidence and risk factors are not well established. PURPOSE To evaluate the rate of SE in LVNC and describe risk factors. METHODS LNVC patients were included in a multicentric registry. Those with SE were considered for the analysis. RESULTS 514 patients with LVNC from 10 Spanish centres were recruited from 2000 to 2018. During a median follow-up of 4.2 years (IQR 1.9-7.1), 23 patients (4.5%) had a SE. Patients with SE (Table 1) were older at diagnosis, with no differences in gender and had similar cardiovascular risk factors. They were more frequently under oral anticoagulation (OAC). Besides, they had a more reduced LVEF, and more dilated LV and left atrium (LA). Late gadolinium enhancement (LGE) was more frequent, altogether suggesting a more severe phenotype. Patients with SE had non-significantly higher rates of hospitalization for heart failure (33% Vs 24%, p = 0.31) and atrial fibrillation (35% Vs 19%, p = 0.10). In multivariate analysis, only LA diameter was an independent predictor of SE (OR 1.04, p = 0.04). A LA diameter > 45 mm had an independent 3 fold increased risk of SE (OR 3.04, p = 0.02) (Image 1). CONCLUSIONS LVNC carries a moderate mid-term risk of SE, which appears to be irrespective of atrial fibrillation and associated with age, LV dilatation and systolic dysfunction and mainly LA dilatation. This subgroup of patients should be considered for oral anticoagulation in primary prevention. Table 1 Systemic embolisms (n = 23) No systemic embolisms (n = 491) p Men, n (%) 15 (65) 289 (56) 0.52 Median age at diagnosis (IQR) - yr 60 (48-76) 48 (30-64) 0.02 Median follow up (IQR) - yr 5.9 (3.1-7.8) 4.2 (1.8-7.1) 0.18 OAC, n (%) 19 (83) 118 (24) 0.01 LVEF (SD) - % 37 (15) 48 (17) 0.01 LVEDD (SD) - mm 58 (11) 54 (10) 0.04 LA diameter (SD) - mm 46 (9) 39 (9) 0.01 Characteristics of patients with and without systemic embolisms Abstract P1441 Figure. Image 1

scholarly journals Left Ventricular Myocardial Noncompaction with Advanced Atrioventricular Conduction Disorder and Ventricular Arrhythmias in a Young Patient: Role of MIB1 Gene

2021 ◽  
Vol 8 (9) ◽  
pp. 109
Author(s):  
Cristina Balla ◽  
Martina De Raffele ◽  
Maria Angela Deserio ◽  
Mariabeatrice Sanchini ◽  
Marianna Farnè ◽  
...  
Keyword(s):  
Ventricular Arrhythmias ◽  
Left Ventricular ◽  
Structural Abnormality ◽  
Ventricular Noncompaction ◽  
Patient Role ◽  
Conduction Disorders ◽  
Pathogenic Variation ◽  
Av Conduction ◽  
Conduction Disorder

Left ventricular noncompaction (LVNC) is a structural abnormality of the left ventricle, usually described as an isolated condition, or sometimes associated with other structural cardiac diseases. LVNC is generally asymptomatic, although it may present conduction disorders, arrhythmias, and heart failure. Here, we present the case of a patient who came to our attention with a severe LVNC phenotype associated with advanced AV conduction disorder, and supraventricular and ventricular arrhythmias at young age, in which a novel MIB1, likely pathogenic, variation has been identified.

Catheter Ablation of Ventricular Arrhythmias in Left Ventricular NonCompaction Cardiomyopathy

Heart Rhythm ◽  
2020 ◽  
Author(s):  
Juan José Sánchez Muñoz ◽  
Carmen Muñoz Esparza ◽  
Pablo Peñafiel Verdú ◽  
Juan Martínez Sánchez ◽  
Francisco García Almagro ◽  
...  
Keyword(s):  
Catheter Ablation ◽  
Ventricular Arrhythmias ◽  
Left Ventricular ◽  
Left Ventricular Noncompaction ◽  
Ventricular Noncompaction ◽  
Noncompaction Cardiomyopathy ◽  
Left Ventricular Noncompaction Cardiomyopathy

LVAD as a Bridge to Heart Transplantation in a Patient with Left Ventricular Noncompaction Cardiomyopathy and Advanced Heart Failure

2016 ◽  
Vol 19 (3) ◽  
pp. 128 ◽  
Author(s):  
Andraž Cerar ◽  
Juš Kšela ◽  
Gregor Poglajen ◽  
Bojan Vrtovec ◽  
Ivan Kneževič
Keyword(s):  
Heart Failure ◽  
Heart Transplantation ◽  
Specific Treatment ◽  
Left Ventricular ◽  
Advanced Heart Failure ◽  
Circulatory Support ◽  
Left Ventricular Noncompaction ◽  
Ventricular Noncompaction ◽  
Noncompaction Cardiomyopathy ◽  
Left Ventricular Noncompaction Cardiomyopathy

Left ventricular noncompaction cardiomyopathy (LVNC) is a rare hereditary cardiomyopathy characterized by the formation of an outer compacted and inner noncompacted layer of the myocardium. The latter is characterized by prominent trabeculations and deep intertrabecular recesses and is functionally inferior to the compacted myocardium. As there is no specific treatment for patients with LVNC who develop heart failure, the management of these patients is limited and many patients progress to advanced stages of the disease. For LVNC patients with advanced heart failure, the data regarding the use of mechanical circulatory support are scarce. We report a case of a 29-year-old patient with LVNC and advanced refractory heart failure, who was successfully bridged to heart transplantation using a long-term continuous-flow left ventricular assist device.

scholarly journals Left Ventricular Noncompaction Cardiomyopathy Presenting with Heart Failure in a 35-Year-Old Man

2017 ◽  
Vol 44 (4) ◽  
pp. 260-263 ◽  
Author(s):  
Kyriacos Papadopoulos ◽  
Petros M. Petrou ◽  
Demos Michaelides
Keyword(s):  
Heart Failure ◽  
Ventricular Arrhythmias ◽  
Left Ventricular ◽  
Two Dimensional ◽  
Left Ventricular Noncompaction ◽  
Heart Failure Therapy ◽  
Ventricular Noncompaction ◽  
Dimensional Echocardiography ◽  
Two Dimensional Echocardiography ◽  
Left Ventricular Noncompaction Cardiomyopathy

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.

P5555Predictors of systemic embolisms in a large cohort of left ventricular noncompaction patients

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
G Casas ◽  
G Oristrell ◽  
J Limeres ◽  
A Sao-Aviles ◽  
R Barriales ◽  
...  
Keyword(s):  
Risk Factors ◽  
Atrial Fibrillation ◽  
Oral Anticoagulation ◽  
Systolic Dysfunction ◽  
Left Ventricular ◽  
Left Ventricular Noncompaction ◽  
Severe Phenotype ◽  
Ventricular Noncompaction ◽  
Increased Risk

Abstract Background Left ventricular noncompaction (LVNC) is associated with an increased risk of systemic embolisms (SE). However, incidence and risk factors are not well established. Purpose To evaluate the rate of SE in LVNC and describe risk factors. Methods LNVC patients were included in a multicentric registry. Those with SE were considered for the analysis. Results 514 patients with LVNC from 10 Spanish centres were recruited from 2000 to 2018. During a median follow-up of 4.2 years (IQR 1.9–7.1), 23 patients (4.5%) had a SE. Patients with SE (Table 1) were older at diagnosis, with no differences in gender and had similar cardiovascular risk factors. They were more frequently under oral anticoagulation (OAC). Besides, they had a more reduced LVEF, and more dilated LV and left atrium (LA). Late gadolinium enhancement (LGE) was more frequent, altogether suggesting a more severe phenotype. Patients with SE had non-significantly higher rates of hospitalization for heart failure (33% vs 24%, p=0.31) and atrial fibrillation (35% vs 19%, p=0.10). In multivariate analysis, only LA diameter was an independent predictor of SE (OR 1.04, p=0.04). A LA diameter>45 mm had an independent 3 fold increased risk of SE (OR 3.04, p=0.02) (Image 1). Table 1 Systemic embolisms (n=23) No systemic embolisms (n=491) p Men, n (%) 15 (65) 289 (56) 0.52 Median age at diagnosis (IQR), yr 60 (48–76) 48 (30–64) 0.02 Median follow up (IQR), yr 5.9 (3.1–7.8) 4.2 (1.8–7.1) 0.18 Hypertension, % 8 (33) 118 (24) 0.31 Diabetes mellitus, % 3 (14) 39 (8) 0.41 OAC, % 19 (83) 118 (24) 0.01 LVEF (SD), % 37 (15) 48 (17) 0.01 LVEDD (SD), mm 58 (11) 54 (10) 0.04 LVESD (SD), mm 45 (13) 38 (11) 0.01 LA diameter (SD), mm 46 (9) 39 (9) 0.01 LVEDV CMR (SD), mL 193 (75) 163 (70) 0.12 LVESV CMR (SD), mL 121 (64) 85 (64) 0.04 LGE, % 9 (40) 88 (18) 0.04 Conclusions LVNC carries a moderate mid-term risk of SE, which appears to be irrespective of atrial fibrillation and associated with age, LV dilatation and systolic dysfunction and mainly LA dilatation. This subgroup of patients should be considered for oral anticoagulation in primary prevention.

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