Gene Editing as the Future of Cardiac Amyloidosis Therapeutics

Sethi, Yashendra; Mahtani, Arun Umesh; Khehra, Nimrat; Padda, Inderbir; Patel, Neil; Sebastian, Sneha Annie; Malhi, Gurnaaz; Kaiwan, Oroshay; Saith, Sunil E.; Johal, Gurpreet

doi:10.1016/j.cpcardiol.2023.101741

Cited by 2 publications

(1 citation statement)

References 43 publications

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“…No data are available for [115,118,121] SCD risk stratification in ALVC patients without a causative gene variant; however, the ICD implantation in primary prevention may be considered in AVLC patients with nsVT, high burden of PVCs, syncope, family history of SCD, or significant LGE on CMR [7]. Patients should be informed about gaps in evidence and a shared decision-making process accounting for individual preferences for ICD implantation in primary prevention, evaluating the benefit/risk ratio [38,[122][123][124][125][126][127][128][129][130][131][132]. Table 3 summarizes genotype-specific reccommendatios for ICD implantation.…”

Section: Genotype-phenotype Correlation For Scd Prevention Strategymentioning

confidence: 99%

Arrhythmogenic Left Ventricular Cardiomyopathy: From Diagnosis to Risk Management

Mauriello,

Roma,

Ascrizzi

et al. 2024

JCM

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Purpose of Review: Left ventricular arrhythmogenic cardiomyopathy (ALVC) is a rare and poorly characterized cardiomyopathy that has recently been reclassified in the group of non-dilated left ventricular cardiomyopathies. This review aims to summarize the background, diagnosis, and sudden cardiac death risk in patients presenting this cardiomyopathy. Recent Findings: Although there is currently a lack of data on this condition, arrhythmogenic left ventricular dysplasia can be considered a specific disease of the left ventricle (LV). We have collected the latest evidence about the management and the risks associated with this cardiomyopathy. Summary: Left ventricular arrhythmogenic cardiomyopathy is still poorly characterized. ALVC is characterized by fibrofatty replacement in the left ventricular myocardium, with variable phenotypic expression. Diagnosis is based on a multiparametric approach, including cardiac magnetic resonance (CMR) and genetic testing, and is important for sudden cardiac death (SCD) risk stratification and management. Recent guidelines have improved the management of left ventricular arrhythmogenic cardiomyopathy. Further studies are necessary to improve knowledge of this cardiomyopathy.

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