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- Cortez, Daniel, et al.
(författare)
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In Hypertrophic Cardiomyopathy, the Spatial Peaks QRS-T Angle Identifies Those With Sustained Ventricular Arrhythmias
- 2016
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Ingår i: Clinical Cardiology. - : Wiley. - 0160-9289. ; 39:8, s. 459-463
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Tidskriftsartikel (refereegranskat)abstract
- Background: The spatial peaks QRS-T (SPQRS-T) angle differentiates hypertrophic cardiomyopathy (HCM) patients from controls. Increased angle confers arrhythmia risk in other populations. Hypothesis: We predict that the SPQRS-T angle will identify HCM patients with sustained ventricular arrhythmias (VAs) and those with New York Heart Association class III/IV heart failure. Methods: Corrected QT interval, QRS duration, and SPQRS-T angle were assessed in HCM patients with VAs (>30seconds) and those without VAs. Results: One hundred HCM patients (mean age, 32.7±17.2years) were assessed. Twenty patients had VAs. The corrected QT interval identified VA (P = 0.018) and at 460ms gave positive and negative predictive values of 28.6% and 83.3%, respectively, and an odds ratio of 2.0 (95% confidence interval: 0.7-5.6). The SPQRS-T angle differentiated VA from no VA (P <0.001) and at 124.1 degrees gave positive and negative predictive values and an odds ratio of 36.7%, 96.1%, and 14.2 (95% confidence interval: 3.1-65.6), respectively. Conclusions: The SPQRS-T angle best differentiated patients with VAs.
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| 3. |
- Cortez, Daniel, et al.
(författare)
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Right precordial-directed electrocardiographical markers identify arrhythmogenic right ventricular cardiomyopathy in the absence of conventional depolarization or repolarization abnormalities
- 2017
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Ingår i: BMC Cardiovascular Disorders. - : Springer Science and Business Media LLC. - 1471-2261. ; 17:1
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Tidskriftsartikel (refereegranskat)abstract
- Background: Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) carries a risk of sudden death. We aimed to assess whether vectorcardiographic (VCG) parameters directed toward the right heart and a measured angle of the S-wave would help differentiate ARVD/C with otherwise normal electrocardiograms from controls. Methods: Task Force 2010 definite ARVD/C criteria were met for all patients. Those who did not fulfill Task Force depolarization or repolarization criteria (-ECG) were compared with age and gender-matched control subjects. Electrocardiogram measures of a 3-dimentional spatial QRS-T angle, a right-precordial-directed orthogonal QRS-T (RPD) angle, a root mean square of the right sided depolarizing forces (RtRMS-QRS), QRS duration (QRSd) and the corrected QT interval (QTc), and a measured angle including the upslope and downslope of the S-wave (S-wave angle) were assessed. Results: Definite ARVD/C was present in 155 patients by 2010 Task Force criteria (41.7 ± 17.6 years, 65.2% male). -ECG ARVD/C patients (66 patients) were compared to 66 control patients (41.7 ± 17.6 years, 65.2% male). All parameters tested except the QRSd and QTc significantly differentiated -ECG ARVD/C from control patients (p < 0.004 to p < 0.001). The RPD angle and RtRMS-QRS best differentiated the groups. Combined, the 2 novel criteria gave 81.8% sensitivity, 90.9% specificity and odds ratio of 45.0 (95% confidence interval 15.8 to 128.2). Conclusion: ARVD/C disease process may lead to development of subtle ECG abnormalities that can be distinguishable using right-sided VCG or measured angle markers better than the spatial QRS-T angle, the QRSd or QTc, in the absence of Taskforce ECG criteria.
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| 4. |
- Cortez, Daniel, et al.
(författare)
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The S-wave angle identifies arrhythmogenic right ventricular cardiomyopathy in patients with electrocardiographically concealed disease phenotype
- 2018
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Ingår i: Journal of Electrocardiology. - : Elsevier BV. - 0022-0736 .- 1532-8430. ; 51:6, s. 1003-1008
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Tidskriftsartikel (refereegranskat)abstract
- Background: Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) carries risk of sudden death. We hypothesize that the S-wave angle differentiates ARVD/C with otherwise normal electrocardiograms from controls. Materials and methods: All patients met Task Force 2010 definite ARVD/C criteria. ARVD/C patients without Task Force depolarization/repolarization criteria (−ECG) were compared to controls. Electrocardiogram measures of QRS duration, corrected QT interval, and measured angle between the upslope and downslope of the S-wave in V2, were assessed. Results: Definite ARVD/C was present in 155 patients (42.7 ± 17.3 years, 68.4%male). −ECG ARVD/C patients (66 patients) were compared to 66 control patients (41.8 ± 17.6 years, 65.2%male). Only the S-wave angle differentiated −ECG ARVD/C patients from controls (<0.001) with AU the ROC curve of 0.77 (95%CI 0.53 to 0.71) and odds ratio of 28.3 (95%CI 6.4 to 125.5). Conclusion: ARVD/C may lead to development of subtle ECG abnormalities distinguishable using the S-wave angle prior to development of 2010 Taskforce ECG criteria.
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| 5. |
- Roberts, Jason D., et al.
(författare)
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Ankyrin-B dysfunction predisposes to arrhythmogenic cardiomyopathy and is amenable to therapy
- 2019
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Ingår i: Journal of Clinical Investigation. - : AMER SOC CLINICAL INVESTIGATION INC. - 0021-9738 .- 1558-8238. ; 129:8, s. 3171-3184
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Tidskriftsartikel (refereegranskat)abstract
- Arrhythmogenic cardiomyopathy (ACM) is an inherited arrhythmia syndrome characterized by severe structural and electrical cardiac phenotypes, including myocardial fibrofatty replacement and sudden cardiac death. Clinical management of ACM is largely palliative, owing to an absence of therapies that target its underlying pathophysiology, which stems partially from our limited insight into the condition. Following identification of deceased ACM probands possessing ANK2 rare variants and evidence of ankyrin-B loss of function on cardiac tissue analysis, an ANK2 mouse model was found to develop dramatic structural abnormalities reflective of human ACM, including biventricular dilation, reduced ejection fraction, cardiac fibrosis, and premature death. Desmosomal structure and function appeared preserved in diseased human and murine specimens in the presence of markedly abnormal beta-catenin expression and patterning, leading to identification of a previously unknown interaction between ankyrin-B and beta-catenin. A pharmacological activator of the WNT/beta-catenin pathway, SB-216763, successfully prevented and partially reversed the murine ACM phenotypes. Our findings introduce what we believe to be a new pathway for ACM, a role of ankyrin-B in cardiac structure and signaling, a molecular link between ankyrin-B and beta-catenin, and evidence for targeted activation of the WNT/beta-catenin pathway as a potential treatment for this disease.
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