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.

Abstract

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.

Related Concept Videos

Dysrhythmias IV: Characteristics of Bradyarrhythmias01:18

Dysrhythmias IV: Characteristics of Bradyarrhythmias

Bradyarrhythmias are cardiac rhythm disorders characterized by a slower-than-normal heart rate, typically defined as fewer than 60 beats per minute. Some of which are discussed here:Sinus BradycardiaSinus bradycardia presents a heart rate lower than 60 beats per minute, with a regular rhythm originating from the SA node. The ECG typically shows normal P waves preceding each QRS complex, a normal PR interval (0.12 to 0.20 seconds), and a normal QRS duration (0.06 to 0.10 seconds).First-Degree AV...
175
Electrophysiology of Normal Cardiac Rhythm01:19

Electrophysiology of Normal Cardiac Rhythm

The normal cardiac rhythm is a synchronized electrical activity that facilitates the regular and coordinated contraction of the heart muscle. This process is essential for efficient blood circulation throughout the body. The fundamental elements involved in establishing and maintaining this rhythm include the unique electrical properties of cardiac muscle cells, the sinoatrial (SA) node's pacemaker function, the specialized conducting system, and the ionic mechanisms underlying each phase...
7.5K
Bode Plots Construction01:24

Bode Plots Construction

The Bode plot is an essential tool in control system analysis, mapping the frequency response of a system through a magnitude plot and a phase plot, both against a logarithmic frequency axis. To construct a Bode plot, consider the transfer function H(ω):
851
Dysrhythmias V: Evaluating Dysrhythmias01:30

Dysrhythmias V: Evaluating Dysrhythmias

Dysrhythmias, also known as arrhythmias, are disturbances in the heart's rhythm that range from benign to life-threatening. A thorough evaluation is crucial for appropriate management and involves a comprehensive medical history, physical examination, and various diagnostic tests.Medical HistorySymptoms: Collect detailed information on palpitations, dizziness, syncope, chest pain, and fatigue. Note their onset, frequency, and triggers.Previous Cardiac Issues: Document any history of heart...
148