Ventricular Dysrhythmias During Long-Term Follow-Up in Patients With Inherited Cardiac Arrhythmia

Sitorus, Gustaf D.S.; Ragab, Ahmed; Houck, Charlotte A.; Lanters, Eva A.H.; Heida, Annejet; Gastel, Virgilla E. van; Muskens, Agnes J.Q.M.; Groot, Natasja M.S. de

doi:10.1016/j.amjcard.2019.07.050

Cited by 4 publications

(3 citation statements)

References 29 publications

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“…Ventricular arrhythmias are a common occurrence amongst patients with so-called inherited cardiac arrhythmias, including ARVC/D, Brugada syndrome, long QT-syndrome and short QT-syndrome, amongst which ARVC/D cohorts have shown to present with the highest rates of frequent ventricular premature complexes, (non)sustained ventricular tachycardias, and malignant ventricular tachyarrhythmias [ 31 ]. Several large-scale studies have reported on the clinical characteristics and predictors of ventricular arrhythmias in ARVC/D patients.…”

Section: Discussionmentioning

confidence: 99%

A Territory-Wide Study of Arrhythmogenic Right Ventricular Cardiomyopathy Patients from Hong Kong

Lakhani¹,

Zhou²,

Lee³

et al. 2022

Rev. Cardiovasc. Med.

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Background: Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a hereditary disease characterized by fibrofatty infiltration of the right ventricular myocardium that predisposes affected patients to malignant ventricular arrhythmias, dual-chamber cardiac failure and sudden cardiac death (SCD). The present study aims to investigate the risk of detrimental cardiovascular events in an Asian population of ARVC/D patients, including the incidence of malignant ventricular arrhythmias, new-onset heart failure with reduced ejection fraction (HFrEF), as well as long-term mortality. Methods and Results: This was a territory-wide retrospective cohort study of patients diagnosed with ARVC/D between 1997 and 2019 in Hong Kong. This study consisted of 109 ARVC/D patients (median age: 61 [46-71] years; 58% male). Of these, 51 and 24 patients developed incident VT/VF and new-onset HFrEF, respectively. Five patients underwent cardiac transplantation, and 14 died during follow-up. Multivariate Cox regression identified prolonged QRS duration as a predictor of VT/VF (p < 0.05). Female gender, prolonged QTc duration, the presence of epsilon waves and T-wave inversion (TWI) in any lead except aVR/V1 predicted new-onset HFrEF (p < 0.05). The presence of epsilon waves, in addition to the parameters of prolonged QRS duration and worsening ejection fraction predicted all-cause mortality (p < 0.05). Clinical scores were developed to predict incident VT/VF, new-onset HFrEF and all-cause mortality, and all were significantly improved by machine learning techniques. Conclusions: Clinical and electrocardiographic parameters are important for assessing prognosis in ARVC/D patients and should in turn be used in tandem to aid risk stratification in the hospital setting.

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Section: Discussionmentioning

confidence: 99%

A Territory-Wide Study of Arrhythmogenic Right Ventricular Cardiomyopathy Patients from Hong Kong

Lakhani¹,

Zhou²,

Lee³

et al. 2022

Rev. Cardiovasc. Med.

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“…Strain imaging by speckle-tracking echocardiography has been used to risk-stratify patients in heart failure (28) and recent work has shown that incorporation of mechanical dispersion can further improve risk prediction (29), as heart failure is typically underrecognized in ARVC/D (30). Ventricular arrhythmias are a common occurrence amongst patients with so-called inherited cardiac arrhythmias, including ARVC/D, Brugada syndrome, long QT-syndrome and short QTsyndrome, amongst which ARVC/D cohorts have shown to present with the highest rates of frequent ventricular premature complexes, (non)sustained ventricular tachycardias, and malignant ventricular tachyarrhythmias (31). Several large-scale studies have reported on the clinical characteristics and predictors of ventricular arrhythmias in ARVC/D patients.…”

Section: Discussionmentioning

confidence: 99%

A territory-wide study of arrhythmogenic right ventricular cardiomyopathy patients from Hong Kong

Lakhani¹,

Zhou

Lee³

et al. 2021

Preprint

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BackgroundArrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a hereditary disease characterized by fibrofatty infiltration of the right ventricular myocardium that predisposes affected patients to malignant ventricular arrhythmias, dual-chamber cardiac failure and sudden cardiac death (SCD). The present study aims to investigate the risk of detrimental cardiovascular events in an Asian population of ARVC/D patients, including the incidence of malignant ventricular arrhythmias, new-onset heart failure with reduced ejection fraction (HFrEF), as well as long-term mortality.Methods and ResultsThis was a territory-wide retrospective cohort study of patients diagnosed with ARVC/D between 1997 and 2019 in Hong Kong. This study consisted of 109 ARVC/D patients (median age: 61 [46-71] years; 58% male). Of these, 51 and 24 patients developed incident VT/VF and new-onset HFrEF, respectively. Five patients underwent cardiac transplantation, and 14 died during follow-up. Multivariable Cox regression identified prolonged QRS duration as a predictor of VT/VF (p < 0.05). Female gender, prolonged QTc duration, the presence of epsilon waves and T-wave inversion (TWI) in any lead except aVR/V1 predicted new-onset HFrEF (P < 0.05. The presence of epsilon waves, in addition to the parameters of prolonged QRS duration and worsening ejection fraction predicted all-cause mortality (p<0.05). Clinical scores were developed to predict incident VT/VF, new-onset HFrEF and all-cause mortality, and all were significantly improved by machine learning techniques.ConclusionClinical and electrocardiographic parameters are important for assessing prognosis in ARVC/D patients and should in turn be used in tandem to aid risk stratification in the hospital setting.

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“…Apart from the pathophysiology, it should be noted that leading cycle theory and spiral wave theory have, in their origins, a premature contraction that may be caused by abnormal impulse formation. This phenomenon often occurs in patients with channelopathies, with a greater incidence [20,[47][48][49][50][51][52].…”

Section: Reentrymentioning

confidence: 99%

Primary Electrical Heart Disease—Principles of Pathophysiology and Genetics

Badura,

Buławska,

Dąbek

et al. 2024

IJMS

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Primary electrical heart diseases, often considered channelopathies, are inherited genetic abnormalities of cardiomyocyte electrical behavior carrying the risk of malignant arrhythmias leading to sudden cardiac death (SCD). Approximately 54% of sudden, unexpected deaths in individuals under the age of 35 do not exhibit signs of structural heart disease during autopsy, suggesting the potential significance of channelopathies in this group of age. Channelopathies constitute a highly heterogenous group comprising various diseases such as long QT syndrome (LQTS), short QT syndrome (SQTS), idiopathic ventricular fibrillation (IVF), Brugada syndrome (BrS), catecholaminergic polymorphic ventricular tachycardia (CPVT), and early repolarization syndromes (ERS). Although new advances in the diagnostic process of channelopathies have been made, the link between a disease and sudden cardiac death remains not fully explained. Evolving data in electrophysiology and genetic testing suggest previously described diseases as complex with multiple underlying genes and a high variety of factors associated with SCD in channelopathies. This review summarizes available, well-established information about channelopathy pathogenesis, genetic basics, and molecular aspects relative to principles of the pathophysiology of arrhythmia. In addition, general information about diagnostic approaches and management is presented. Analyzing principles of channelopathies and their underlying causes improves the understanding of genetic and molecular basics that may assist general research and improve SCD prevention.

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Ventricular Dysrhythmias During Long-Term Follow-Up in Patients With Inherited Cardiac Arrhythmia

Cited by 4 publications

References 29 publications

A Territory-Wide Study of Arrhythmogenic Right Ventricular Cardiomyopathy Patients from Hong Kong

A Territory-Wide Study of Arrhythmogenic Right Ventricular Cardiomyopathy Patients from Hong Kong

A territory-wide study of arrhythmogenic right ventricular cardiomyopathy patients from Hong Kong

Primary Electrical Heart Disease—Principles of Pathophysiology and Genetics

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