scholarly journals Prognostic role of cardiac magnetic resonance in left ventricular noncompaction

2021 ◽  
Vol 22 (Supplement_2) ◽  
Author(s):  
G Casas ◽  
J Limeres ◽  
R Barriales-Villa ◽  
P Garcia-Pavia ◽  
E Zorio ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: None. Background Left ventricular noncompaction (LVNC) is a heterogeneous entity with a wide phenotypic expression. Risk factors have not been well established and prognostic stratification remains challenging. Purpose Describe prognostic role of CMR on long term outcomes of LVNC patients.  Methods   Retrospective multicentric longitudinal cohort study of consecutive patients fulfilling imaging diagnostic criteria for LVNC (Jenni echo criteria and Petersen and Jacquier CMR criteria). Demographic, ECG, genetic, family and treatment variables were recorded. Baseline CMR was used for the analysis. LV ejection fraction (LVEF) was categorized according to heart failure (HF) guidelines and late gadolinium enhancement (LGE) was visually assessed in a binary way. End points were HF, ventricular arrhythmias (VA), systemic embolisms (SE) and all-cause death. Major adverse cardiovascular events (MACE) were the combination of the four previous end points. In patients with initially preserved LVEF (≥ 50%), LV adverse remodelling (LVAR) was defined as an LVEF < 50% and/or absolute decrease of ≥10% in LVEF at last follow-up. Results 585 patients from 12 referral centres were included from 2000 to 2018. Age at diagnosis was 45 ± 20 years, 334 (57%) were male, baseline LVEF was 48 ± 17% and 18% presented LGE. During a median follow-up of 5.1 years (IQR 2.3-8.1), 110 (19%) patients presented HF, 87 (15%) VA, 18 (3%) SE and 34 (6%) died. MACE occurred in 223 (38%) patients. LVEF was independently associated with HF, VA, SE and MACE: HR were 1.08, 1.02, 1.04 and 1.02 respectively (all p < 0.05). LGE was more frequent in patients with reduced LVEF (39 Vs 53%, p < 0.001) and was associated with higher HF and VA risk in patients with an LVEF > 35% (HR 2.69 and 2.48 respectively, p < 0.05) (Figure 1). No MACE (0%) occurred during long-term follow-up in patients with preserved LVEF, no LGE as well as no ECG abnormalities and no family aggregation. 305 (52%) patients presented with initially preserved LVEF, and 230 (75%) of those had LVEF available at last follow-up. LVAR occurred in 50 (22%) patients: 22 (10%) had an LVEF < 50% and 41 (18%) an absolute ≥ 10% decrease in LVEF. LGE was independently associated with LVAR (HR 3.51, p = 0.045) (Figure 2).  Conclusions Cardiac magnetic resonance has an important prognostic role in LVNC. LVEF is the most powerful predictor of events. Myocardial fibrosis is associated with worse outcomes in patients without severe systolic dysfunction, as well as with left ventricular adverse remodelling in those with initially preserved LVEF. Besides, CMR may identify a low-risk subgroup of LVNC patients. Therefore, CMR should be used in risk stratification in LVNC.

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
G Casas ◽  
J Limeres ◽  
G Oristrell ◽  
L Gutierrez ◽  
R Barriales ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: None. Background Left ventricular noncompaction (LVNC) is a heterogeneous entity with a wide phenotypic expression. Risk factors have not been well established and prognostic stratification remains challenging. Objectives Describe long term outcomes of LVNC patients and determine predictors of cardiovascular events.  Methods   Prospective multicentric study of consecutive patients fulfilling imaging diangostic criteria for LVNC (Jenni echo criteria and Petersen CMR criteria). Demographic, ECG, imaging and genetic variables were collected. End points were heart failure (HF), ventricular arrhythmias (VA), systemic embolisms (SE) and all-cause death. Major adverse cardiovascular events (MACE) was the combination of the four previous end points. Results 585 patients from 12 referral centres were included from 2000 to 2018. Age at diagnosis was 45 ± 20 years, 334 (57%) were male, baseline LVEF was 48 ± 17% and 18% presented late gadolinium enhancement (LGE). During a median follow-up of 5.1 years (IQR 2.3-8.1), 110 (19%) patients presented HF, 87 (15%) VA, 18 (3%) SE and 34 (6%) died. MACE occurred in 223 (38%) patients. LVEF was independently associated with HF, VA, SE and MACE: HR were 1.08, 1.02, 1.04 and 1.02 respectively (all p < 0.05). LGE was more frequent in patients with reduced LVEF (39 Vs 53%, p < 0.001) and was associated with higher HF and VA risk in patients with LVEF > 35% (HR 2.69 and 2.48 respectively, p < 0.05) (Figure 1). Patients with a normal ECG, LVEF≥50%, no LGE and no family aggregation presented no MACE (0%) at long term follow-up. Among patients who underwent genetic testing (354, 61%), TTN variants and complex genotype (more than one variant) presented lower LVEF and higher HF risk. ACTC1 variants were associated with VA. Conclusions LVNC carries a high long term risk of heart faliure and ventricular arrhythmias. LVEF is the most important predictor and myocardial fibrosis is associated with increased risk in patients without severe systolic dysfunction. Genotype is a modifier of outcomes. These factors might be used to risk stratify LVNC patients. Abstract Figure. Kaplan Meier survival curves


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 93 (1115) ◽  
pp. 20200514
Author(s):  
Vineeta Ojha ◽  
Rishabh Khurana ◽  
Kartik P Ganga ◽  
Sanjeev Kumar

Takotsubo cardiomyopathy (TC) is a reversible condition in which there is transient left ventricular (LV) dysfunction characterised most commonly by basal hyperkinesis and mid-apical LV ballooning and hypokinesia. It is said to be triggered by stress and mimics, such as acute coronary syndrome (ACS) clinically. Diagnosis is usually suspected on echocardiography due to the characteristic contraction pattern in a patient with symptoms and signs of ACS but normal coronary arteries on catheter angiography. Cardiac magnetic resonance (CMR), with its latest advancements, is the diagnostic modality of choice for diagnosis, prognosis and follow-up of patients. The advances in CMR (including T1, T2, ECV mapping and threshold-based late gadolinium enhancement (LGE) measurements have revolutionised the role of CMR in tissue characterisation and prognostication in patients with TC. In this review, we highlight the current role of CMR in management of TC and enumerate the CMR findings in TC as well the current advances in the field of CMR, which could help in prognosticating these patients.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Jiamin Zhang ◽  
Mengchun Jiang ◽  
Chao Zheng ◽  
Hui Liu ◽  
Yangyu Guo ◽  
...  

AbstractWe used cardiac magnetic resonance tissue tracking (CMR-TT) to quantitatively analyze the global, regional and layer-specific strain of isolated left ventricular noncompaction (ILVNC). Combined with late gadolinium enhancement (LGE), we initially explored the effect of focal myocardial fibrosis on myocardial strain. CMR was performed in 63 patients with ILVNC and 52 patients without ILVNC (i.e., the control group). The ILVNC group was divided into an LGE(+) group (29 patients) and an LGE(−) group (34 patients) according to the presence or absence of late gadalinum enhancement (LGE). CVI42 software was used to measure global and regional (basal, middle, apical) radial strain (RS), circumferential strain (CS), longitudinal strain (LS), subendocardial LS and subepicardial LS. The basal–apical strain gradient was defined as the apical mean strain minus the basal mean strain. We then compared differences between these strain parameters. The subendocardial-subepicardial LS gradient was defined as the maximum subendocardial LS minus the subepicardial LS. Compared with the control group, the global and regional RS, CS, LS and the subendocardial, subepicardial LS of the ILVNC group were significantly diminished (P < 0.01). Compared with the LGE(−) group, the global and regional RS, CS, LS and the subendocardial, subepicardial LS of the LGE(+) group were significantly diminished (P < 0.05). In the ILVNC group, the basal–apical CS and LS gradient, and the subendocardial-subepicardial LS gradient were significantly lower than those in the control group (P < 0.01). There were significant differences in myocardial strain between patients with and without ILVNC. ILVNC revealed a specific pattern in terms of strain change. The myocardial strain of the cardiac apex and endocardium was significantly lower than that of the cardiac base and epicardium, respectively. Myocardial strain reduction was more significant in ILVNC patients with focal myocardial fibrosis.


2019 ◽  
Vol 12 (11) ◽  
pp. 2141-2151 ◽  
Author(s):  
Chrysanthos Grigoratos ◽  
Andrea Barison ◽  
Alexander Ivanov ◽  
Daniele Andreini ◽  
Mihaela-Silvia Amzulescu ◽  
...  

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