Risk Stratification In Arrhythmogenic Right Ventricular Cardiomyopathy: An Integrative Approach Towards Gene-Specific Insights

Alessio Gasperetti

doi:10.33540/1800

Risk Stratification In Arrhythmogenic Right Ventricular Cardiomyopathy: An Integrative Approach Towards Gene-Specific Insights

Alessio Gasperetti

Research output: Thesis › Doctoral thesis 2 (Research NOT UU / Graduation UU)

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Abstract

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy associated with an increased risk of ventricular arrhythmias (VA) and sudden cardiac death. The current cornerstone of ARVC management is the placement of implantable cardioverter defibrillators (ICDs). The ARVC risk calculator is a modern risk stratification tool that can be used in patients with ARVC and no previous sustained arrhythmias to estimate the 5-yr risk of sustained VA events and to help guiding ICD placement at the time of disease diagnosis. This thesis discusses the process of external validation, subsequent algorithm refinement, and longitudinal follow-up recalibration of the ARVC Risk Calculator tool. Specific insights on its use in left-dominant ARVC phenotypes and in patients with a desmoplakin (DSP) pathogenic variants are discussed, as well as its integration with results retrieved from programmed ventricular stimulation procedures.

Original language	English
Awarding Institution	University Medical Center (UMC) Utrecht
Supervisors/Advisors	Asselbergs, Folkert, Primary supervisor van Tintelen, Peter, Supervisor te Riele, Anneline, Co-supervisor James, C.A., Co-supervisor, External person
Award date	8 Jun 2023
Place of Publication	Utrecht
Publisher	Utrecht University
DOIs	https://doi.org/10.33540/1800
Publication status	Published - 8 Jun 2023
Externally published	Yes

Keywords

ARVC
ACM
ICD
Risk stratification

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10.33540/1800

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https://dspace.library.uu.nl/handle/1874/428758

Cite this

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title = "Risk Stratification In Arrhythmogenic Right Ventricular Cardiomyopathy: An Integrative Approach Towards Gene-Specific Insights",

abstract = "Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy associated with an increased risk of ventricular arrhythmias (VA) and sudden cardiac death. The current cornerstone of ARVC management is the placement of implantable cardioverter defibrillators (ICDs). The ARVC risk calculator is a modern risk stratification tool that can be used in patients with ARVC and no previous sustained arrhythmias to estimate the 5-yr risk of sustained VA events and to help guiding ICD placement at the time of disease diagnosis. This thesis discusses the process of external validation, subsequent algorithm refinement, and longitudinal follow-up recalibration of the ARVC Risk Calculator tool. Specific insights on its use in left-dominant ARVC phenotypes and in patients with a desmoplakin (DSP) pathogenic variants are discussed, as well as its integration with results retrieved from programmed ventricular stimulation procedures.",

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doi = "10.33540/1800",

language = "English",

publisher = "Utrecht University",

type = "Doctoral thesis 2 (Research NOT UU / Graduation UU)",

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TY - THES

T1 - Risk Stratification In Arrhythmogenic Right Ventricular Cardiomyopathy

T2 - An Integrative Approach Towards Gene-Specific Insights

AU - Gasperetti, Alessio

PY - 2023/6/8

Y1 - 2023/6/8

N2 - Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy associated with an increased risk of ventricular arrhythmias (VA) and sudden cardiac death. The current cornerstone of ARVC management is the placement of implantable cardioverter defibrillators (ICDs). The ARVC risk calculator is a modern risk stratification tool that can be used in patients with ARVC and no previous sustained arrhythmias to estimate the 5-yr risk of sustained VA events and to help guiding ICD placement at the time of disease diagnosis. This thesis discusses the process of external validation, subsequent algorithm refinement, and longitudinal follow-up recalibration of the ARVC Risk Calculator tool. Specific insights on its use in left-dominant ARVC phenotypes and in patients with a desmoplakin (DSP) pathogenic variants are discussed, as well as its integration with results retrieved from programmed ventricular stimulation procedures.

AB - Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy associated with an increased risk of ventricular arrhythmias (VA) and sudden cardiac death. The current cornerstone of ARVC management is the placement of implantable cardioverter defibrillators (ICDs). The ARVC risk calculator is a modern risk stratification tool that can be used in patients with ARVC and no previous sustained arrhythmias to estimate the 5-yr risk of sustained VA events and to help guiding ICD placement at the time of disease diagnosis. This thesis discusses the process of external validation, subsequent algorithm refinement, and longitudinal follow-up recalibration of the ARVC Risk Calculator tool. Specific insights on its use in left-dominant ARVC phenotypes and in patients with a desmoplakin (DSP) pathogenic variants are discussed, as well as its integration with results retrieved from programmed ventricular stimulation procedures.

KW - ARVC

KW - ACM

KW - ICD

KW - Risk stratification

U2 - 10.33540/1800

DO - 10.33540/1800

M3 - Doctoral thesis 2 (Research NOT UU / Graduation UU)

PB - Utrecht University

CY - Utrecht

ER -

Risk Stratification In Arrhythmogenic Right Ventricular Cardiomyopathy: An Integrative Approach Towards Gene-Specific Insights

Abstract

Keywords

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