scholarly journals Prognostic significance of cardiac magnetic resonance-based markers in patients with hypertrophic cardiomyopathy

Author(s):  
Zsofia Dohy ◽  
Liliana Szabo ◽  
Attila Toth ◽  
Csilla Czimbalmos ◽  
Rebeka Horvath ◽  
...  

AbstractThe prognosis of patients with hypertrophic cardiomyopathy (HCM) varies greatly. Cardiac magnetic resonance (CMR) is the gold standard method for assessing left ventricular (LV) mass and volumes. Myocardial fibrosis can be noninvasively detected using CMR. Moreover, feature-tracking (FT) strain analysis provides information about LV deformation. We aimed to investigate the prognostic significance of standard CMR parameters, myocardial fibrosis, and LV strain parameters in HCM patients. We investigated 187 HCM patients who underwent CMR with late gadolinium enhancement and were followed up. LV mass (LVM) was evaluated with the exclusion and inclusion of the trabeculae and papillary muscles (TPM). Global LV strain parameters and mechanical dispersion (MD) were calculated. Myocardial fibrosis was quantified. The combined endpoint of our study was all-cause mortality, heart transplantation, malignant ventricular arrhythmias and appropriate implantable cardioverter defibrillator (ICD) therapy. The arrhythmia endpoint was malignant ventricular arrhythmias and appropriate ICD therapy. The LVM index (LVMi) was an independent CMR predictor of the combined endpoint independent of the quantification method (p < 0.01). The univariate predictors of the combined endpoint were LVMi, global longitudinal (GLS) and radial strain and longitudinal MD (MDL). The univariate predictors of arrhythmia events included LVMi and myocardial fibrosis. More pronounced LV hypertrophy was associated with impaired GLS and increased MDL. More extensive myocardial fibrosis correlated with impaired GLS (p < 0.001). LVMi was an independent CMR predictor of major events, and myocardial fibrosis predicted arrhythmia events in HCM patients. FT strain analysis provided additional information for risk stratification in HCM patients.

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
L Szabo ◽  
V Bodi ◽  
CS Czimbalmos ◽  
ZS Dohy ◽  
V Horvath ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: Public grant(s) – National budget only. Main funding source(s): Development and Innovation Fund of Hungary, National Research, Development and Innovation Office Background In case of malignant ventricular arrhythmias (VA) and nonobstructed coronary arteries, the differential diagnosis of the  underlying diseases is still challenging, due to the board spectrum of possible causes. Cardiac magnetic resonance (CMR) provides functional, morphological and tissue specific information, including necrotic and scar-tissue.  Aims   We aimed to assess the diagnostic and prognostic implications of CMR parameters including global strain values and myocardial scar in patients after ventricular fibrillation (VF) or sustained ventricular tachycardia (SVT) and nonobstructed coronary arteries. Methods Between 2011 and 2019, 99 patients (42 ±17 years, 54 male) presenting with VF or SVT and nonobstructed coronary arteries, who underwent CMR examination before secondary prevention implantable cardioverter defibrillator (ICD) implantation were included in our study. Post-processing included feature-tracking strain analysis and left ventricular (LV) scar quantification. Patients were followed for the combined endpoint of all-cause-mortality and appropriate ICD therapy. Results CMR examination proved structural myocardial disease in 72%: dilated (n = 21), arrhythmogenic (n = 11), hypertrophic cardiomyopathy (n = 7) and other cardiomyopathies (n = 3). We found LGE patterns showing chronic myocardial infarction (n = 4), suggesting chronic myocarditis (n = 4) and aspecific nonischemic scar formation (n = 14). In 7 cases aspecific structural alterations without scar formation were detected. Overall, myocardial scar was found in 52%, with an average extent of 12 ± 8% of the LV myocardium. The CMR examination changed the clinical diagnosis in 55% of the patients.  During a median follow-up at 2 years, 6 patients died and 42 experienced appropriate ICD therapy. We found an association between cardiac events and the presence of structural abnormality and myocardial scar (logrank: 4,553, p &lt; 0.05 and 8.375, p &lt;0.01).  On Cox proportional-hazards modell LV ejection fraction, LV stroke volume index, the presence of structural abnormality, the presence and extent of  myocardial scar, global LV strain parameters including longitudinal and circumferential strain, and a global left ventricular dssynchrony parameter (mechanical dispersion) were univariate predictors of the combined endpoint of all-cause-mortality and appropriate ICD therapy(p &lt; 0.05).  Conclusion CMR performed in patients after malignant VA and nonobstructed coronary arteries not only establishes the diagnosis in a high proportion of patients, but may also provide additional prognostic factors. This may indicate that CMR could play a complementary role in the risk stratification in this patient population. Abstract Figure.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
A R Barbosa ◽  
C M O'neill ◽  
C Ruivo ◽  
I Cruz ◽  
O Sousa ◽  
...  

Abstract Background Strain techniques, such as feature tracking cardiac magnetic resonance (FT-CMR), have emerged as a promise for more accurate evaluation of cardiac function compared to ejection fraction. In hypertrophic cardiomyopathy (HCM) patients, impaired myocardial deformation measured by FT-CMR has been associated with severity of hypertrophy and presence of late gadolinium enhancement (LGE) but associations with clinical severity and prognosis are scarce. Purpose To analyse the association between left ventricular strain measured by FT-CMR, morphologic features and prognostic markers in patients with HCM. Methods Retrospective analysis of clinical, echocardiography, Holter and CMR data of HCM patients aged ≥16 years followed at two referral centres. Ventricular arrhythmias (VA) were defined as non-sustained or sustained ventricular tachycardia or sudden cardiac arrest. Sudden cardiac death (SCD) risk was evaluated using the score proposed by the European Society of Cardiology. LGE extension was evaluated using the American Heart Association 17-segment model. FT-CMR was used to evaluate global peak systolic longitudinal (GLS), radial (GRS) and circumferential (GCS) strains - GLS was averaged from three standard longitudinal views while GRS and GCS were averaged from the basal, mid and apical LV short-axis planes. Results A total of 109 HCM patients (59.2±16.2 years old; 60.6% males) were included; mean follow-up was 39±25 months. Mean LV mass was 170.6±70.3g, LVEF was 63.7±10.0% and the number of segments with LGE was 3.14±3.32. Mean GLS, GRS and GCS were −14.8±4.0%, 34.4±13.3% and −17.5±4.8%, respectively. Impaired strain was associated with higher LV mass (GLS: r=0.46, GRS: r=−0.46, GCS: r=0.47, p<0.001 for all), reduced LVEF (GLS: r=−0,33, GRS: r=0,44, GCS: r=−0.41, p<0.003 for all) and LGE extension (GLS: r=0.26, GRS: r=−0.38, GCS: r=0.38, p<0.01 for all). SCD risk score was 3.12%±2.98 (8 patients scored as high risk) and VA were documented in 26 patients (26%). Patients with VA had worse strain values than those without (GLS −13.2±4.12 vs −15.5±3.71, p=0.011; GCS −15,8±5.22 vs −18.3±4.24, p=0.017). Patients with high estimated risk of SCD also had worse strain values than those at low/intermediate risk (GLS −12.2±3.57 vs −15.1±3.83, p=0.048; GCS −14.5±4.26 vs −17.9±4.54, p=0.047). A correlation between SCD risk and GLS and GCS was observed (r=0.32, p=0.004; r=0.23, p=0.03, respectively). Conclusions In our population, worse strain measurements were associated with a more severe HCM phenotype, presence of VA and a higher estimated risk of SCD. Strain assessed by FT-CMR may improve risk stratification in HCM patients.


2021 ◽  
Vol 8 ◽  
Author(s):  
Cailing Pu ◽  
Jingle Fei ◽  
Sangying Lv ◽  
Yan Wu ◽  
Chengbin He ◽  
...  

Background: Hypertrophic cardiomyopathy (HCM) is prone to myocardial heterogeneity and fibrosis, which are the substrates of ventricular arrhythmias (VAs). Cardiac magnetic resonance tissue tracking (CMR-TT) can quantitatively reflect global and regional left ventricular strain from different directions. It is uncertain whether the change of myocardial strain detected by CMR-TT is associated with VAs. The aim of the study is to explore the differential diagnostic value of VAs in HCM by CMR-TT.Materials and Methods: We retrospectively included 93 HCM patients (38 with VAs and 55 without VAs) and 30 healthy cases. Left ventricular function, myocardial strain parameters and percentage of late gadolinium enhancement (%LGE) were evaluated.Results: Global circumferential strain (GCS) and %LGE correlated moderately (r = 0.51, P &lt; 0.001). HCM patients with VAs had lower left ventricular ejection fraction (LVEF), global radial strain (GRS), GCS, and global longitudinal strain (GLS), but increased %LGE compared with those without VAs (P &lt; 0.01 for all). %LGE and GCS were indicators of VAs in HCM patients by multivariate logistic regression analysis. HCM patients with %LGE &gt;5.35% (AUC 0.81, 95% CI 0.70–0.91, P &lt; 0.001) or GCS &gt;-14.73% (AUC 0.79, 95% CI 0.70–0.89, P &lt; 0.001) on CMR more frequently had VAs. %LGE + GCS were able to better identify HCM patients with VAs (AUC 0.87, 95% CI 0.79–0.95, P &lt; 0.001).Conclusion: GCS and %LGE were independent risk indicators of VAs in HCM. GCS is expected to be a good potential predictor in identifying HCM patients with VAs, which may provide important values to improve risk stratification in HCM in clinical practice.


2021 ◽  
Vol 8 ◽  
Author(s):  
Yang Ye ◽  
ZhongPing Ji ◽  
Wenli Zhou ◽  
Cailing Pu ◽  
Ya Li ◽  
...  

Background: Ventricular arrhythmias are associated with sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM). Previous studies have found the late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR) was independently associated with ventricular arrhythmia (VA) in HCM. The risk stratification of VA remains complex and LGE is present in the majority of HCM patients. This study was conducted to determine whether the scar heterogeneity from LGE-derived entropy is associated with the VAs in HCM patients.Materials and Methods: Sixty-eight HCM patients with scarring were retrospectively enrolled and divided into VA (31 patients) and non-VA (37 patients) groups. The left ventricular ejection fraction (LVEF) and percentage of the LGE (% LGE) were evaluated. The scar heterogeneity was quantified by the entropy within the scar and left ventricular (LV) myocardium.Results: Multivariate analyses showed that a higher scar [hazard ratio (HR) 2.682; 95% CI: 1.022–7.037; p = 0.039] was independently associated with VA, after the adjustment for the LVEF, %LGE, LV maximal wall thickness (MWT), and left atrium (LA) diameter.Conclusion: Scar entropy and %LGE are both independent risk indicators of VA. A high scar entropy may indicate an arrhythmogenic scar, an identification of which may have value for the clinical status assessment of VAs in HCM patients.


2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
C Nikolaidou ◽  
C Kotanidis ◽  
J Leal-Pelado ◽  
K Kouskouras ◽  
VP Vassilikos ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Cardiac magnetic resonance (CMR) imaging can identify the underlying substrate in patients with ventricular arrhythmias (VAs) and normal echocardiography. Myocardial strain has emerged as a superior index of systolic performance compared to ejection fraction (EF), with an incremental prognostic value in many cardiac diseases. Purpose To assess myocardial deformation using 2-D feature-tracking CMR strain imaging (CMR-FT) in patients with frequent VAs (≥500 ventricular premature contractions (VPC)/24 hours; and/or non-sustained ventricular tachycardia), and structurally normal hearts on echocardiography without evidence of coronary artery disease. Methods Sixty-eight consecutive patients (mean age 46 ± 16 years; 54% female) and 72 healthy controls matched for age and body surface area were included in the study. CMR imaging was performed on a 1.5T Magnetom Avanto (Siemens, Erlangen, Germany) scanner using a standard cardiac protocol. Results CMR showed normal findings in 30 patients (44%), while 16 (24%) had previous myocarditis, 6 (9%) had a diagnosis of non-ischaemic cardiomyopathy (NICM), 15 (22%) were diagnosed with VPC-related cardiomyopathy, and 1 patient had subendocardial infarction [excluded from strain analysis]. Mean left ventricular EF (LVEF) in patients was 62% ± 6% and right ventricular EF 64% ± 6% (vs. 65% ± 3% and 66% ± 4% in controls, respectively). Compared to control subjects, patients with VAs had impaired peak LV global radial strain (GRS) (28.88% [IQR: 25.87% to 33.97%] vs. 36.65% [IQR:33.19% to 40.2%], p &lt; 0.001) and global circumferential strain (GCS) (-17.73% [IQR: -19.8% to -16.33%] vs. -20.66% [IQR: -21.72% to -19.6%], p &lt; 0.001, Panel A). Peak LV GRS could differentiate patients with previous myocarditis from patients with NICM and those with VPC-related cardiomyopathy (Panel B). Peak LV GCS could differentiate patients with previous myocarditis from patients with NICM (Panel C). Peak LV GRS showed excellent diagnostic accuracy in detecting patients from control subjects (Panel D). In a multivariable regression model, subjects with a low GRS (&lt;29.91%-determined by the Youden’s index) had 5-fold higher odds of having VAs (OR:4.99 [95%CI: 1.2-21.95]), after adjusting for LVEF, LV end-diastolic volume index, age, sex, BMI, smoking, hypertension, and dyslipidaemia. Peak LV global longitudinal strain (GLS) and RV strain indices were not statistically different between patients and controls. Conclusion Peak LV GRS and GCS are impaired in patients with frequent idiopathic VAs and can detect myocardial contractile dysfunction in patients with different underlying substrates. Our findings suggest that LV strain indices on CMR-FT constitute independent markers of myocardial dysfunction on top and independently of EF. Abstract Figure.


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.


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