Unraveling the ventricular arrhythmia substrate and risk stratification in non-ischemic cardiomyopathies

Unraveling the ventricular arrhythmia substrate and risk stratification in non-ischemic cardiomyopathies

Patients with non-ischemic cardiomyopathies are at risk for life-threatening ventricular arrhythmias. Understanding the underlying substrate is the key to developing successful treatment and individualized risk stratification. Over the last decade our research group has provided important insights. 

Projects

Prospective Leiden NICM registry

The Leiden Nonischemic Cardiomyopathy Study is a single-center prospective cohort study designed to identify NICM patients at risk for  ventricular arrhythmias. Patients undergo a comprehensive evaluation including a 24-hour Holter, transthoracic echocardiography, LGE-CMR, exercise testing, blood sampling, invasive EPS, endomyocardial biopsy, and genetic analysis. Clinicaltrials.gov identifier: NCT01940081.

project prospective leiden nicm registry circle wecam

A workflow has been developed at WECAM to accurately integrate in-vivo obtained electroanatomical mapping and imaging data with ex-vivo full heart human histology.  Direct comparison of functional data with 3D histology allows  detailed analysis of the underlying  arrhythmic substrate.  

project full heart histology wecam

Unipolar electroanatomical voltage mapping correlates with fibrosis and can also detect fibrosis not evident on LGE-CMR. We have developed a new parameter, the area and volume weighted UV, as a measure that reflects the total amount of remaining viable myocardium, which identifies patients at risk of rapid deterioration of cardiac function and end-stage HF. The predictive value is under evaluation.

project risk stratification wecam

Sympathetic denervation is known to be an important determinant of arrhythmogenicity. In order to study ventricular arrhythmia substrates that are difficult to control by radiofrequency catheter ablation, this project focuses on the relation between cardiac sympathetic innervation and myocardial fibrosis.

project cardiac innervation wecam
project cardiac innervation 2 wecam

Key reviews

Ventricular Tachycardia Ablation in Nonischemic Cardiomyopathy.
JACC. Clinical electrophysiology
Edition: 2405-500X | Pages: 1123-1140

Key publications

Nonsustained Ventricular Tachycardia Is Independently Associated With Sustained Ventricular Arrhythmias in Nonischemic Dilated Cardiomyopathy.
Edition: 1941-3149 | Pages: e009979
Prevalence and Prognostic Impact of Pathogenic Variants in Patients With Dilated Cardiomyopathy Referred for Ventricular Tachycardia Ablation.
J Innov Card Rhythm Manag
Edition: 2405-500X | Pages: 1103-1114
Whole Heart Histology: A Method for the Direct Integration of Histology With Electrophysiological and Imaging Data.
JACC Clin Electrophysiol
Edition: 2405-500X | Pages: 461-462
Entropy as a Novel Measure of Myocardial Tissue Heterogeneity for Prediction of Ventricular Arrhythmias and Mortality in Post-Infarct Patients.
JACC Clin Electrophysiol
Edition: 2405-500X | Pages: 480-489
Whole human heart histology to validate electroanatomical voltage mapping in patients with non-ischaemic cardiomyopathy and ventricular tachycardia.
Europace
Edition: 0195-668X | Pages: 2867-2875
QRS prolongation after premature stimulation is associated with polymorphic ventricular tachycardia in nonischemic cardiomyopathy: Results from the Leiden Nonischemic Cardiomyopathy Study.
JACC Clin Electrophysiol
Edition: 1547-5271 | Pages: 860-869
Contrast-enhanced MRI-derived scar patterns and associated ventricular tachycardias in nonischemic cardiomyopathy: implications for the ablation strategy.
Eur Heart J
Edition: 1941-3149 | Pages: 875-883
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