scholarly journals Electrocardiographic differences between Anderson-Fabry and sarcomeric hypertrophic cardiomyopathy and correlation with cardiac magnetic resonance

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
G Vitale ◽  
F Di Nicola ◽  
I Tanini ◽  
A Camporeale ◽  
F Graziani ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Cardiac Magnetic Resonance ◽  
Hypertrophic Cardiomyopathy ◽  
Magnetic Resonance ◽  
Late Onset ◽  
Lateral Wall ◽  
Left Ventricular ◽  
Maximal Wall Thickness ◽  
Group A ◽  
T Waves

Abstract Background Differential diagnosis between Anderson-Fabry (AF) and sarcomeric hypertrophic cardiomyopathy (HCM) is often very challenging particularly in AF patients with late onset cardiac involvement. Purpose To gain new insights from standard electrocardiogram (ECG) in AF disease for differential diagnosis from sarcomeric HCM. Additionally, to better understand ECG features in AF patients, a correlation substudy ECG-cardiac magnetic resonance (CMR) has been performed. Methods From 162 patients with definite diagnosis of AF disease, 111 [65 males, median age 57 (51–67) years] with pathologic left ventricular hypertrophy (LVH) (Group A) were compared with 111 sarcomeric HCM patients (Group B) sex, age and maximal wall thickness matched by 1:1 propensity score. Results AF patients showed shorter PR interval [155 (140–180) vs 163 (149–184) msec; p=0.005) and wider QRS interval [110 (100–134) vs 100 (90–106) msec; p<0.0001). Additionally AF patients had a higher prevalence of complete (22% vs 3%; p<0.0001) and incomplete (13% vs 1%; p<0.0001) right bundle branch block (RBBB) and a higher percentage of ST segment depression (12% vs 1%; p=0.001) and inferior negative T waves (34% vs 19%; p=0.01). No differences in terms of Sokolow-Lyon and Cornell scores were found whereas total QRS score was higher in Group A [20 (16–27) vs 18 [14–22] mV; p=0.0004). Low QRS voltages and inferior Q waves were not present in AF patients. Among the 69 AF patients who underwent MRI, the 44 with late gadolinium enhancement (LGE) were older [59 (52–66) vs 53 (40–59) years; p=0.017] and had more frequently negative T waves on ECG, particularly in the inferior leads (64% vs 8%; p<0.0001), compared to the 25 without LGE. At multivariate analysis, age and negative T waves were independently associated to the presence of LGE on CMR. Conclusions Compared to matched sarcomeric HCM, AF patients had a shorter PR, wider QRS and a higher percentage of RBBB in relation to to the different aetiology (storage vs “pure” hypertrophy). The higher total QRS score and the absence of inferior Q waves could reflect the more frequent concentric distribution of LVH. Additionally negative T waves, especially in inferior leads, are related to the presence of LGE on CMR (often in the postero-lateral wall). Funding Acknowledgement Type of funding source: None

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

2021 ◽  
Author(s):  
Zsofia Dohy ◽  
Liliana Szabo ◽  
Attila Toth ◽  
Csilla Czimbalmos ◽  
Rebeka Horvath ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Hypertrophic Cardiomyopathy ◽  
Magnetic Resonance ◽  
Myocardial Fibrosis ◽  
Ventricular Arrhythmias ◽  
Prognostic Significance ◽  
Strain Analysis ◽  
Left Ventricular ◽  
Icd Therapy ◽  
Lv Mass

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.

Magnetic Resonance Imaging of Hypertrophic Cardiomyopathy: Beyond Left Ventricular Wall Thickness

2015 ◽  
Vol 66 (1) ◽  
pp. 71-78 ◽  
Author(s):  
Julie Fattal ◽  
Marc-Antoine Henry ◽  
Sopheap Ou ◽  
Simon Bradette ◽  
Konstantin Papas ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Hypertrophic Cardiomyopathy ◽  
Magnetic Resonance ◽  
Left Ventricular ◽  
Resonance Imaging ◽  
The Past ◽  
Radiologic Findings ◽  
Pictorial Essay ◽  
Ventricular Wall Thickness

During the past decade, cardiac magnetic resonance has gained increasing popularity in the diagnosis of hypertrophic cardiomyopathy because of its greater accuracy and better characterization of cardiac morphology compared with other imaging modalities. In this pictorial essay, a global clinical portrait of hypertrophic cardiomyopathy will be drawn. The various radiologic findings associated with each variant of hypertrophic cardiomyopathy, and the clinical edge offered by cardiac magnetic resonance will be discussed.

P900Electrocardiographic findings in Anderson-Fabry disease versus sarcomeric hypertrophic cardiomyopathy

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
G Vitale ◽  
E Biagini ◽  
M Ziacchi ◽  
F Di Nicola ◽  
M Graziosi ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Cardiac Involvement ◽  
Organ Damage ◽  
Left Ventricular ◽  
St Segment ◽  
Replacement Fibrosis ◽  
Group A ◽  
Group B ◽  
Repolarization Abnormalities ◽  
T Waves

Abstract Background Cardiac involvement is one of the most frequent and disabling organ damage in Anderson-Fabry (AF) disease, causing hypertrophic cardiomyopathy (HCM), conduction disturbances, arrhythmias and coronary disease. Differential diagnosis from sarcomeric HCM is often very challenging, especially for patients with exclusive cardiac involvement. Purpose To gain new insights from standard electrocardiogram (ECG) in AF disease, and identify ECG differences from sarcomeric HCM. Methods Sixty-two consecutive patients (27 males, mean age: 62±16 years) with definite diagnosis of AF disease from 2 Italian centres were evaluated for ECG analysis and divided in 2 groups, according to the presence (Group A, N=39) or the absence (Group B, N=26) of cardiac involvement [hypertrophy detected at echocardiogram or cardiac magnetic resonance (CMR)]. All ECGs were analysed by 2 independent investigators. For Group A, when CMR was performed, a correlation between CMR and ECG was assessed. Patients with cardiac involvement were matched with 78 sarcomeric HCM patients according to sex, age and septal wall thickness on echocardiogram. Results Two AF patients out 39 with cardiac involvement (5%) had normal ECG. Short PR and I degree atrio-ventricular (AV) block were both reported in 6 (15%) cases. Twenty-six (67%) patients showed left ventricular hypertrophy and the majority (85%) had abnormal repolarization. CMR was performed in 22 patients (56%); the 11 (50%) patients with replacement fibrosis had a higher mean Sokolow-Lyon score (4.1±1.8 vs 2.9±1.0 mV; p=0.05), more frequent ST segment depression (82 vs 27%; p=0.03) and negative T waves (91 vs 36%; p=0.027; sensitivity: 90%; specificity: 63%), compared with the 11 without replacement fibrosis. When compared with sarcomeric HCM, AF patients with cardiac involvement had a significantly wider QRS (120±30 vs 100±16 msec; p<0.0001), a higher frequency of right bundle branch block (RBBB) (18 vs 3%; p=0.01), ST segment depression (54 vs 20%; p<0.0001) and negative T waves (72 vs 46%; p=0.01), typically in the inferior leads (44 vs 14%; p<0.0001). No significant differences in terms of pseudo-necrosis and QRS voltages were found. Among Group B AF patients (26, mean age: 36±12 years), 4 had short PR and 5 incomplete RBBB. Four had an abnormal ECG (1 left atrial enlargement, 2 unspecific repolarization abnormalities, 1 Sokolow score of 3.5 mV). Conclusions Standard ECG can detect cardiac involvement in AF disease. A good correlation was reported between repolarization abnormalities and replacement fibrosis on CMR. Wide QRS and RBBB were more frequent among AF patients compared to age-sex-matched sarcomeric HCM ones, probably due to the different aetiology of the diseases (infiltrative disease vs pure hypertrophy).

P5270Impaired myocardial deformation assessed by cardiac magnetic resonance is associated with increased arrhythmic risk in hypertrophic cardiomyopathy

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
A R Barbosa ◽  
C M O'neill ◽  
C Ruivo ◽  
I Cruz ◽  
O Sousa ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Hypertrophic Cardiomyopathy ◽  
Magnetic Resonance ◽  
Sudden Cardiac Arrest ◽  
Heart Association ◽  
Left Ventricular ◽  
Myocardial Deformation ◽  
Clinical Severity ◽  
Lv Mass ◽  
Estimated Risk

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.

scholarly journals Post-Mortem Cardiac Magnetic Resonance for the Diagnosis of Hypertrophic Cardiomyopathy

Diagnostics ◽  
2020 ◽  
Vol 10 (11) ◽  
pp. 981
Author(s):  
Giovanni Donato Aquaro ◽  
Benedetta Guidi ◽  
Federico Biondi ◽  
Enrica Chiti ◽  
Alessandro Santurro ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Hypertrophic Cardiomyopathy ◽  
Magnetic Resonance ◽  
Cardiac Death ◽  
Left Ventricular ◽  
Post Mortem ◽  
Cardiac Phase ◽  
Rigor Mortis ◽  
Female Population ◽  
Artery Disease

Background: Post-mortem cardiac magnetic resonance (PMCMR) is an emerging tool supporting forensic medicine for the identification of the causes of cardiac death, such as hypertrophic cardiomyopathy (HCM). We proposed a new method of PMCMR to diagnose HCM despite myocardial rigor mortis. Methods: We performed CMR in 49 HCM patients, 30 non-HCM hypertrophy, and 32 healthy controls. In cine images, rigor mortis was simulated by the analysis of the cardiac phase corresponding to 25% of diastole. Left ventricular mass, mean, and standard deviation (SD) of WT, maximal WT, minimal WT, and their difference were compared for the identification of HCM. These parameters were validated at PMCMR, evaluating eight hearts with HCM, 10 with coronary artery disease, and 10 with non-cardiac death. Results: The SD of WT with a cut-off of > 2.4 had the highest accuracy to identify HCM (AUC 0.95, 95% CI = 0.89–0.98). This was particularly evident in the female population of HCM (AUC=0.998), with 100% specificity (95% CI = 85–100%) and 96% sensitivity (95% CI = 79–99%). Using this parameter, at PMCMR, all of the eight patients with HCM were correctly identified with no false positives. Conclusions: PMCMR allows identification of HCM as the cause of sudden death using the SD of WT > 2.4 as the diagnostic parameter.

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