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 usefulness of the electrocardiogram and cardiac magnetic resonance to differentiate tachycardia induced cardiomyopathy from dilated cardiomyopathy in patients admitted for heart failure

2021 ◽  
Vol 22 (Supplement_2) ◽  
Author(s):  
A Vera Sainz ◽  
A Cecconi ◽  
P Martinez-Vives ◽  
MJ Olivera ◽  
S Hernandez ◽  
...  
Keyword(s):  
Heart Failure ◽  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Dilated Cardiomyopathy ◽  
Left Ventricular ◽  
High Rate ◽  
Left Ventricular Ejection ◽  
Qrs Duration ◽  
Tachycardia Induced Cardiomyopathy

Abstract Funding Acknowledgements Type of funding sources: None. Background In patients admitted for heart failure (HF) with reduced left ventricular ejection fraction (LVEF) and a concomitant high-rate supraventricular tachyarrhythmia (SVT) it is challenging to predict LVEF recovery after heart rate control and distinguish tachycardia-induced cardiomyopathy (TIC) from dilated cardiomyopathy (DC). The role of cardiac magnetic resonance (CMR) and the electrocardiogram (ECG) in this setting remains unsettled. Methods Forty-three consecutive patients admitted for HF due to high-rate SVT and LVEF <50% undergoing CMR in the acute phase were retrospectively included. Those who had LVEF >50% at follow up were classified as TIC and those with LVEF <50% were classified as DC. Clinical, laboratory, CMR and ECG findings were analyzed to predict LVEF recovery. Results Twenty-five (58%) patients were classified as TIC. Patients with DC had wider QRS (121.2 ± 26 vs 97.7 ± 17.35 ms; p = 0.003). On CRM the TIC group presented with higher LVEF (33.4 ± 11 vs 26.9 ± 6.4% p = 0.019) whereas late gadolinium enhancement (LGE) was more frequent in DC group (61 vs 16% p = 0.004). On multivariate analysis, QRS duration ≥100 ms (p = 0.027), LVEF < 40% on CMR (p = 0.047) and presence of LGE (p = 0.03) were identified as independent predictors of lack of LVEF recovery. Furthermore, during clinical follow-up (median 60 months) DC patients were admitted more frequently for HF (44% vs 0%; p < 0.001) than TIC patients (Figure 1). Conclusion In patients with reduced LVEF admitted for HF due to high-rate SVT, QRS duration ≥100 ms, LVEF <40% on CMR and presence of LGE are independently associated with lack of LVEF recovery and worse clinical outcome.

scholarly journals Left Ventricular Noncompaction—A Systematic Review of Risk Factors in the Pediatric Population

2021 ◽  
Vol 10 (6) ◽  
pp. 1232
Author(s):  
Katarzyna Łuczak-Woźniak ◽  
Bożena Werner
Keyword(s):  
Risk Factors ◽  
Systematic Review ◽  
Magnetic Resonance ◽  
Clinical Manifestations ◽  
Left Ventricular ◽  
Diagnostic Methods ◽  
Left Ventricular Ejection ◽  
Left Ventricular Noncompaction ◽  
Ventricular Ejection Fraction ◽  
Ventricular Noncompaction

Left ventricular noncompaction (LVNC) is a heterogeneous, often hereditary group of diseases, which may have diverse clinical manifestations. This article reviews the risk factors for unfavorable outcomes of LVNC in children, as well as discuss the diagnostic methods and the differences between pediatric and adult LVNC. Through a systematic review of the literature, a total of 1983 articles were outlined; 23 of them met the inclusion criteria. In echocardiography the following have been associated with adverse outcomes in children: Left ventricular ejection fraction, end-diastolic dimension, left ventricular posterior wall compaction, and decreased strains. T-wave abnormalities and increased spatial peak QRS-T angle in ECG, as well as arrhythmia, were observed in children at greater risk. Cardiac magnetic resonance is a valuable tool to identify those with systolic dysfunction and late gadolinium enhancement. Genetic testing appears to help identify children at risk, because mutations in particular genes have been associated with worse outcomes. ECG and imaging tests, such as echocardiography and magnetic resonance, help outline risk factors for unfavorable outcomes of LVNC in children and in identifying outpatients who require more attention. Refining the current diagnostic criteria is crucial to avoid inadequate restrain from physical activity.

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.

scholarly journals Epicardial Adipose Tissue and Outcome in Heart Failure With Mid-Range and Preserved Ejection Fraction

2021 ◽  
Author(s):  
Gijs van Woerden ◽  
Dirk J. van Veldhuisen ◽  
Olivier C. Manintveld ◽  
Vanessa P.M. van Empel ◽  
Tineke P. Willems ◽  
...  
Keyword(s):  
Heart Failure ◽  
Adipose Tissue ◽  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Ejection Fraction ◽  
Epicardial Adipose Tissue ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Preserved Ejection Fraction ◽  
All Cause Mortality

Background: Epicardial adipose tissue (EAT) accumulation is thought to play a role in the pathophysiology of heart failure (HF) with mid-range and preserved ejection fraction, but its effect on outcome is unknown. We evaluated the prognostic value of EAT volume measured with cardiac magnetic resonance in patients with HF with mid-range ejection fraction and HF with preserved ejection fraction. Methods: Patients enrolled in a prospective multicenter study that investigated the value of implantable loop-recorders in HF with mid-range ejection fraction and HF with preserved ejection fraction were analyzed. EAT volume was quantified with cardiac magnetic resonance. Main outcome was the composite of all-cause mortality and first HF hospitalizations. Hazard ratios (HR) and 95% CI are described per SD increase in EAT. Results: We studied 105 patients (mean age 72±8 years, 50% women, and mean left ventricular ejection fraction 53±8%). During median follow-up of 24 (17–25) months, 31 patients (30%) died or were hospitalized for HF. In univariable analysis, EAT was significantly associated with a higher risk of the composite outcome (HR, 1.76 [95% CI, 1.24–2.50], P =0.001), and EAT remained associated with outcome after adjustment for age, sex, and body mass index (HR, 1.61 [95% CI, 1.13–2.31], P =0.009), and after adjustment for New York Heart Association functional class and N-terminal of pro-brain natriuretic peptide (HR, 1.53 [95% CI, 1.04–2.24], P =0.03). Furthermore, EAT was associated with all-cause mortality alone (HR, 2.06 [95% CI, 1.26–3.37], P =0.004) and HF hospitalizations alone (HR, 1.54 [95% CI, 1.04–2.30], P =0.03). Conclusions: EAT accumulation is associated with adverse prognosis in patients with HF with mid-range ejection fraction and HF with preserved ejection fraction. This finding supports the importance of EAT in these patients with HF. REGISTRATION: URL: https://www.clinicaltrials.gov ; Unique identifier: NCT01989299.

scholarly journals Left ventricular hypertrabeculation in Ebstein anomaly – a clinically significant or irrelevant problem?

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
A Cieplucha ◽  
O Trojnarska ◽  
J Rajewska-Tabor ◽  
A Katarzynska-Szymanska ◽  
P Mitkowski ◽  
...  
Keyword(s):  
Heart Failure ◽  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Left Ventricle ◽  
Left Ventricular ◽  
Peak Oxygen Consumption ◽  
Ebstein Anomaly ◽  
Funding Sources ◽  
Peak Vo2 ◽  
And Function

Abstract Background Ebstein anomaly (EA) is characterized by anomalous tricuspid valve resulting in the whole right heart's distorted anatomy. Heart failure is the most frequent complication in adults with EA. Recently more attention is also paid to the left ventricle. Some limited echocardiographic data suggest a markedly increased occurrence of the left ventricular hypertrabeculation, by some authors called a non-compacted myocardium. Currently, cardiac magnetic resonance (CMR) is a gold standard in the evaluation of hypertrabeculation. However, different diagnostic criteria and discrepancies in the study outcomes make the clinical interpretation of this phenomenon questionable and challenging. Purpose To determine the frequency of the left ventricular hypertrabeculation among adults with EA; to assess its association with the ventricular size and function and the markers of heart failure. Methods Study group consisted of 35 unoperated adults (mean age 41.9±14.8 years) with Ebstein anomaly. The study protocol included: a) cardiac magnetic resonance (CMR) with the assessment of the left ventricle (LV) and functional right ventricle (fRV), including ejection fraction (EF), end-diastolic, end-systolic, and stroke volumes indexed by body surface area (respectively: EDVind, ESVind, SVind), and presence of hypertrabeculation defined according to Jacquier (1) as trabeculation mass >20% of the LV mass; b) cardiopulmonary tests with the assessment of peak oxygen consumption (peak VO2, % of predicted peak VO2), ventilation/carbon dioxide slope (VE/VCO2 slope) c) brain natriuretic peptide (BNP). Results Left ventricular hypertrabeculation was present in 13 (37.1%) patients. We demonstrated no difference between patients with and without LV hypertrabeculation regarding: s Conclusions Left ventricular hypertrabeculation demonstrated by cardiac magnetic resonance is a phenomenon frequently occurring among adults with Ebstein anomaly. However, its presence is not associated with both ventricles' worse function nor with reduced exercise capacity. Therefore, the clinical relevance of left ventricular hypertrabeculation in this population remains questionable and requires further prospective studies. FUNDunding Acknowledgement Type of funding sources: None.

scholarly journals Prognostic Significance of Left Ventricular Noncompaction

2020 ◽  
Vol 13 (1) ◽  
Author(s):  
Nay Aung ◽  
Sara Doimo ◽  
Fabrizio Ricci ◽  
Mihir M. Sanghvi ◽  
Cesar Pedrosa ◽  
...  
Keyword(s):  
Heart Failure ◽  
Dilated Cardiomyopathy ◽  
Cardiovascular Mortality ◽  
Ventricular Arrhythmias ◽  
Incidence Rates ◽  
Left Ventricular ◽  
Adverse Outcomes ◽  
Left Ventricular Ejection ◽  
Ventricular Ejection Fraction ◽  
Ventricular Noncompaction

Background: Although left ventricular noncompaction (LVNC) has been associated with an increased risk of adverse cardiovascular events, the accurate incidence of cardiovascular morbidity and mortality is unknown. We, therefore, aimed to assess the incidence rate of LVNC-related cardiovascular events. Methods: We systematically searched observational studies reporting the adverse outcomes related to LVNC. The primary end point was cardiovascular mortality. Results: We identified 28 eligible studies enrolling 2501 LVNC patients (mean age, 46 years; male/female ratio, 1.7). After a median follow-up of 2.9 years, the pooled event rate for cardiovascular mortality was 1.92 (95% CI, 1.54–2.30) per 100 person-years. LVNC patients had a similar risk of cardiovascular mortality compared with a dilated cardiomyopathy control group (odds ratio, 1.10 [95% CI, 0.18–6.67]). The incidence rates of all-cause mortality, stroke and systemic emboli, heart failure admission, cardiac transplantation, ventricular arrhythmias, and cardiac device implantation were 2.16, 1.54, 3.53, 1.24, 2.17, and 2.66, respectively, per 100 person-years. Meta-regression and subgroup analyses revealed that left ventricular ejection fraction, not the extent of left ventricular trabeculation, had an important influence on the variability of incidence rates. The risks of thromboembolism and ventricular arrhythmias in LVNC patients were similar to dilated cardiomyopathy patients. However, LVNC patients had a higher incidence of heart failure hospitalization than dilated cardiomyopathy patients. Conclusions: Patients with LVNC carry a similar cardiovascular risk when compared with dilated cardiomyopathy patients. Left ventricular ejection fraction—a conventional indicator of heart failure severity, not the extent of trabeculation—appears to be an important determinant of adverse outcomes in LVNC patients. Registration: https://www.crd.york.ac.uk/PROSPERO/ Unique identifier: CRD42018096313.

scholarly journals Updating the Risk Stratification for Sudden Cardiac Death in Cardiomyopathies: The Evolving Role of Cardiac Magnetic Resonance Imaging. An Approach for the Electrophysiologist

Diagnostics ◽  
2020 ◽  
Vol 10 (8) ◽  
pp. 541 ◽  
Author(s):  
Ourania Kariki ◽  
Christos-Konstantinos Antoniou ◽  
Sophie Mavrogeni ◽  
Konstantinos A. Gatzoulis
Keyword(s):  
Cardiac Magnetic Resonance ◽  
High Risk ◽  
Sudden Cardiac Death ◽  
Magnetic Resonance ◽  
Cardiac Death ◽  
Ventricular Arrhythmias ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
High Risk Patients ◽  
Risk Patients

The prevention of sudden cardiac death (SCD) in cardiomyopathies (CM) remains a challenge. The current guidelines still favor the implantation of devices for the primary prevention of SCD only in patients with severely reduced left ventricular ejection fraction (LVEF) and heart failure (HF) symptoms. The implantation of an implantable cardioverter-defibrillator (ICD) is a protective barrier against arrhythmic events in CMs, but the benefit does not outweigh the cost in low risk patients. The identification of high risk patients is the key to an individualized prevention strategy. Cardiac magnetic resonance (CMR) provides reliable and reproducible information about biventricular function and tissue characterization. Furthermore, late gadolinium enhancement (LGE) quantification and pattern of distribution, as well as abnormal T1 mapping and extracellular volume (ECV), representing indices of diffuse fibrosis, can enhance our ability to detect high risk patients. CMR can also complement electro-anatomical mapping (EAM), a technique already applied in the risk evaluation and in the ventricular arrhythmias ablation therapy of CM patients, providing a more accurate assessment of fibrosis and arrhythmic corridors. As a result, CMR provides a new insight into the pathological substrate of CM. CMR may help identify high risk CM patients and, combined with EAM, can provide an integrated evaluation of scar and arrhythmic corridors in the ablative therapy of ventricular arrhythmias.

Sign in / Sign up

Export Citation Format

Share Document