Characterization of the electrophysiological substrate in patients with Barlow's disease
Pasquale Vergara1, Iside Scarfò2, Antonio Esposito3,4
1Arrhythmia Unit and Electrophysiology Laboratories, IRCCS San Raffaele Scientific Institute, Milano, Italy.
Background:
Myxomatous mitral valve prolapse (MVP) and mitral-annular disjunction (Barlow disease) are at-risk for ventricular arrhythmias (VA). Fibrosis involving the papillary muscles and/or the infero-basal left ventricular (LV) wall was reported at autopsy in sudden cardiac death (SCD) patients with MVP.
Objectives:
We investigated the electrophysiological substrate subtending VA in MVP patients with Barlow disease phenotype.
Methods:
Twenty-three patients with VA were enrolled, including five with syncope and four with a history of SCD. Unipolar (Uni < 8.3 mV) and bipolar (Bi < 1.5 mV) low-voltage areas were analyzed with electro-anatomical mapping (EAM), and VA inducibility was evaluated with programmed ventricular stimulation (PES). Electrophysiological parameters were correlated with VA patterns, electrocardiogram (ECG) inferior negative T wave (nTW), and late gadolinium enhancement (LGE) assessed by cardiac magnetic resonance.
Results:
Premature ventricular complex (PVC) burden was 12 061.9 ± 12 994.6/24 h with a papillary-muscle type (PM-PVC) in 18 patients (68%). Twelve-lead ECG showed nTW in 12 patients (43.5%). A large Uni less than 8.3 mV area (62.4 ± 45.5 cm2 ) was detected in the basal infero-lateral LV region in 12 (73%) patients, and in the papillary muscles (2.2 ± 2.9 cm2 ) in 5 (30%) of 15 patients undergoing EAM. A concomitant Bi less than 1.5 mV area (5.0 ± 1.0 cm2 ) was identified in two patients. A history of SCD, and the presence of nTW, and LGE were associated with a greater Uni less than 8.3 mV extension: (32.8 ± 3.1 cm2 vs. 9.2 ± 8.7 cm2 ), nTW (20.1 ± 11.0 vs. 4.1 ± 3.8 cm2 ), and LGE (19.2 ± 11.7 cm2 vs. 1.0 ± 2.0 cm2 , p = .013), respectively. All patients with PM-PVC had a Uni less than 8.3 mV area. Sustained VA (ventricular tachycardia 2 and VF 2) were induced by PES only in four patients (one with resuscitated SCD).
Conclusions:
Low unipolar low voltage areas can be identified with EAM in the basal inferolateral LV region and in the papillary muscles as a potential electrophysiological substrate for VA and SCD in patients with MVP and Barlow disease phenotype.
Insights
Myxomatous mitral valve prolapse (MVP) and Barlow disease increase risk for ventricular arrhythmias (VA). Low voltage areas in the left ventricle and papillary muscles are identified as potential substrates for VA and sudden cardiac death (SCD).
Area of Science:
- Cardiology
- Electrophysiology
- Cardiac Imaging
Background:
- Myxomatous mitral valve prolapse (MVP) and mitral-annular disjunction (Barlow disease) are associated with increased risk of ventricular arrhythmias (VA) and sudden cardiac death (SCD).
- Autopsy studies in MVP patients with SCD have revealed fibrosis in the papillary muscles and/or infero-basal left ventricular (LV) wall.
Purpose of the Study:
- To investigate the electrophysiological substrate underlying VA in patients with MVP and Barlow disease phenotype.
- To correlate electrophysiological findings with clinical presentation and imaging data.
Main Methods:
- Electro-anatomical mapping (EAM) was used to analyze unipolar (Uni < 8.3 mV) and bipolar (Bi < 1.5 mV) low-voltage areas in 23 patients with VA.
- Programmed ventricular stimulation (PES) assessed VA inducibility.
- Electrophysiological parameters were correlated with VA patterns, ECG inferior negative T wave (nTW), and late gadolinium enhancement (LGE) on cardiac MRI.
Main Results:
- A significant burden of premature ventricular complexes (PVCs), often papillary-muscle type (PM-PVC), was observed.
- Large unipolar low-voltage areas were identified in the basal infero-lateral LV region and papillary muscles.
- The extension of unipolar low-voltage areas correlated with a history of SCD, presence of nTW, and LGE.
Conclusions:
- Low unipolar low-voltage areas in the basal inferolateral LV and papillary muscles represent a potential electrophysiological substrate for VA and SCD in MVP patients with Barlow disease.
- EAM can identify these critical areas, aiding in risk stratification and management.
More Related Videos
06:35In Vivo Electrophysiological Measurement of Compound Muscle Action Potential from the Forelimbs in Mouse Models of Motor Neuron Degeneration
Published on: June 15, 2018
14:39Isolation and Functional Characterization of Human Ventricular Cardiomyocytes from Fresh Surgical Samples
Published on: April 21, 2014
Related Concept Videos
Dysrhythmias IV: Characteristics of Bradyarrhythmias
Electrophysiology of Normal Cardiac Rhythm
Bode Plots Construction
Dysrhythmias V: Evaluating Dysrhythmias
