Pre-excitation syndrome

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Pre-excitation syndrome Microchapters

Overview

Historical Perspective

Classification

Pathophysiology

Differentiating Pre-excitation Syndrome from other Diseases

Epidemiology and Demographics

Risk Factors

Natural History, Complications, and Prognosis

Diagnosis

Treatment

Prevention

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2] Associate Editor(s)-in-Chief: Shivam Singla, M.D.[3]

Synonyms and Keywords: Pre Excitation Syndromes; Lown-Ganong-Levine Syndrome; Pre-Excitation, Mahaim-Type Pre-excitation; Wolff-Parkinson-White Syndrome

Overview

Pre-excitation syndrome is a condition in which the ventricles of heart depolarize earlier than expected via some accessory pathway conduction, leading to a premature contraction. Normally, atria and ventricles are interconnected with each other via the AV node (atrioventricular node). However, in all the pre-excitation syndromes, an accessory pathway is present that conducts impulses to ventricles besides the AV node. The accessory pathway passes the electrical impulses to the ventricles before the normal impulse of depolarization passes through the AV node. The phenomenon of depolarizing ventricles through the accessory pathway earlier than the usual depolarization is supposed to happen through the AV node is referred to as "Pre- Excitation". WPW syndrome was described in 1930 and named after John Parkinson, Paul Dudley White, and Louis Wolff. The accessory pathways are named depending upon the regions of atria and ventricles they are connecting such as Bundle of His, Mahaim fibers, and James fibers. The typical ECG findings associated with WPW syndrome are shortened PR interval, widened QRS complex and Delta wave which is a slurring in the upstroke of QRS complex due to preexcitation of ventricles via the accessory pathway. ECG findings along with symptomatic tachyarrhythmias are referred to as Wolff-Parkinson-White syndrome. Although it is more common in adults, males have an incidence rate of 0.1-0.3 %, WPW can be considered as a congenital anomaly in some cases where it is usually present since the birth and in others, it is considered as a developmental anomaly. Studies have proven its lower prevalence in children aged between 6-13 than those in the age group of 14-15 years of age. Hemodynamically unstable patients should be managed with a direct cardioversion. For the stable patients, medical management should be used first before using acceptable options of catheter ablation or surgical intervention. Although catheter ablation has widely replaced the surgical option, as it is a less invasive technique with better outcomes, in cases where catheter ablation cannot be done or doesn't prove to be effective, the surgical option is worth considering with a curative rate of nearly 100%.

Historical Perspective

WPW Syndrome was given its name in 1930 by Wolf, Parkinson, and White. Source: Ecgpedia.org

Classification

Type Conduction pathway QRS interval PR interval Delta wave
Wolff-Parkinson-White syndrome Bundle of Kent Wide/long Usually short Yes
Lown-Ganong-Levine syndrome "James bundle" (atria to bundle of His) Normal/Unaffected Short No
Mahaim-type Pre-excitation Mahaim fibers Long Normal No

Pathophysiology

Normal electrical conduction pathway of heart

Pre-excitation pathway

Differentiating Pre-excitation Syndrome from other Diseases

Arrhythmia Rhythm Rate P wave PR Interval QRS Complex Response to Maneuvers Epidemiology Co-existing Conditions
Atrial Fibrillation (AFib)[6][7]
  • Absent
Atrial Flutter[8]
  • Varies depending upon the magnitude of the block, but is short
Atrioventricular nodal reentry tachycardia (AVNRT)[9][10][11]
  • Regular
Multifocal Atrial Tachycardia[12][13]
Paroxysmal Supraventricular Tachycardia
  • Regular
  • 150 and 240 bpm
  • Absent
  • Hidden in QRS
  • Absent
Premature Atrial Contractrions (PAC)[14][15]
  • Upright
  • Usually narrow (< 0.12 s)
Wolff-Parkinson-White Syndrome[16][17]
  • Regular
Ventricular Fibrillation (VF)[18][19][20]
  • Absent
  • Absent
  • Absent (R on T phenomenon in the setting of ischemia)
Ventricular Tachycardia[21][22]
  • Regular
  • > 100 bpm (150-200 bpm common)
  • Absent

Epidemiology and Demographics

Risk Factors

Natural History, Complications, and Prognosis

Natural History

Complications

Prognosis

Diagnosis

WPW Syndrome

WPW ECG changes with significant 1) Delta wave 2) PR interval shortening 3) Wide QRS complexes. [1]

Lown-Ganong-Levine (LGL) Syndrome

Mahaim-Type Pre-excitation

History and Symptoms

Treatment

Medical Treatment

GENERAL PROTOCOL

General protocol includes the following:[26]

IN CASE OF ACUTE AVRT/AVNRT

ATRIAL FLUTTER/FIBRILLATION

RADIOFREQUENCY ABLATION

Surgical management

Prevention

References

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