Pre-excitation syndrome

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Please Take Over This Page and Apply to be Editor-In-Chief for this topic: There can be one or more than one Editor-In-Chief. You may also apply to be an Associate Editor-In-Chief of one of the subtopics below. Please mail us [2] to indicate your interest in serving either as an Editor-In-Chief of the entire topic or as an Associate Editor-In-Chief for a subtopic. Please be sure to attach your CV and or biographical sketch.

Overview[edit | edit source]

Pre-excitation syndrome is a condition where the the ventricles of the heart become depolarized too early, which leads to their partially premature contraction. Normally, the atria (chambers taking venous blood) and the ventriculi (chambers pro-pulsing blood towards organs) are electrically isolated, and only electrical passage exists at "atrioventricular node". In all pre-excitation syndromes, there is at least one more conductive pathway is present. Physiologically, the electrical depolarization wave 'waits' in atrioventricular node to allow atria contract before ventriculi. However, there is no such property exists in abnormal pathway, so electrical stimulus passes to ventricle by this tracts far before normal atrioventricular-his system, and ventricles are depolarized (excited) before (pre-) normal conduction system. The term pre-excitation derives from this condition.

It is usually caused by a secondary conduction pathway (other than the bundle of His)

Classification[edit | edit source]

Type	Conduction pathway	PR interval	QRS interval	Delta wave?
Wolff-Parkinson-White syndrome	Bundle of Kent (atria to ventricles)	short	long	yes
Lown-Ganong-Levine syndrome	"James bundle" (atria to bundle of His)	short	normal	no
Mahaim-type	Mahaim fibers	normal	long

WPW Syndrome

WPW is a combination of presence of congenital accessory pathways along with episodic tachyarrhythmias.

The features of pre excitation are subtle, intermittent and are aggravated by increase in vagal tone ( Valsalva maneuver, AV blockage by drugs).

• ECG Features of WPW
  • Shortened PR interval (Less than 120ms)
  • Delta wave – slow/slurring in the rise of initial portion of the QRS
  • Widening of QRS complex
  • ST Segment and T wave discordant changes – i.e. in the opposite direction to the major component of the QRS complex
  • WPW is mainly categorized as type A or B.
    • Type A: positive delta wave in all precordial leads with R/S > 1 in V1
    • Type B: negative delta wave in leads V1 and V2

Pathophysiology [edit | edit source]

WPW Syndrome

Pathophysiology of Pre-Excitation syndromes

• Pre-excitation refers to the early activation of the ventricles as a result of impulses bypassing the AV node via an accessory pathway. The latter are abnormal conduction pathways formed during cardiac development. These can conduct impulses either towards ventricles (Anterograde conduction, rarely seen) , Away from the ventricles (Retrograde conduction, in approx 15%), or in both the directions ( Majority of cases).

• In WPW syndrome which is a type of pre-excitation syndrome the abnormal conduction pathways are called Bundle of Kent or AV bypass tract. These accessory pathways facilitates formation of Tachyarrhythmias by mainly forming reentry circuit , termed as AVRT. Even the direct conduction through the accessory pathways from A to V ( Bypassing AV node) can result in the formation of Tachyarrhythmias, seen most frequently in A. Fib with RVR.

Atrioventricular Reentry Tachycardia's (AVRT)

AVRT is a form of PSVT. Reentry circuit results from the combination of signal transduction from normal conduction system and accessory pathway.

• During tachyarrythmias, the accessory pathway forms part of the reentry circuit that results in the disappearance of features of tachyarrythmias..
• AVRT are further divided into
  • Orthodromic or Antidromic conduction based on ECG morphology and direction of formation of re-entry circuit.

AVRT ( Orthodromic and Antidromic)

AVRT with Orthodromic Conduction

In this the anterograde conduction occurs via the AV node and retrograde conduction occurs via accessory pathway.

ECG features of AVRT with orthodromic conduction

• Rate usually 200 – 300 bpm

• P waves may be buried in QRS complex or retrograde
• QRS Complex usually <120 ms unless pre-existing bundle branch block, or rate-related aberrant conduction
• QRS Alternans – phasic variation in QRS amplitude associated with AVNRT and AVRT, distinguished from electrical alterns by a normal QRS amplitude
• T wave inversion common
• ST segment depression

Treatment Of Orthodromic AVRT:- Hemodynamically Unstable patients( Low BP, Altered mental state, pulmonary edema)- Synchronized DC Cardioversion. In patients who are hemodynamically stable- Vagal maneuvers, Adenosine, CCB and DC cardioversion, if patient not responding to medical therapy.

AVRT with Antidromic Conduction

In this the anterograde conduction occurs via the accessory pathway and retrograde conduction via the AV node. Occurring only in app. 5% of patients with WPW.

ECG features are:

• Rate usually 200 – 300 bpm.
• Wide QRS complexes due to abnormal accessory pathway ventricular depolarisation.
• Due to wide complex, Commonly mistaken for Ventricular Tachycardia.

Treatment of antidromic AVRT: Hemodynamically stable patients:- Amiodarone, procainamide or ibutilide. Hemodynamically unstable patients:- Urgent synchronized DC cardio version

Atrial Fib/Atrial Flutter in WPW

• In 20% of the patients WPW Atrial fibrillation can occur and in approx 7% of patients with WPW atrial flutter can occur. Accessory pathways plays major role by allowing the rapid conduction of impulses directly to the ventricles without involving AV node, in extreme cases may lead to VT or VF.

ECG features

• Rate > 200 bpm
• Irregular rhythm
• Wide QRS complexes due to abnormal ventricular depolarisation via accessory pathway
• QRS Complexes change in shape and morphology
• Axis remains stable unlike Polymorphic VT
• Atrial Flutter presents with same features as atrial fibrillation in WPW except rhythm is regular and commonly mistaken for VT

Treatment of AF with WPW:- Hemodynamically unstable patients: Urgent synchronized DC cardioversion. Hemodynamically stable patients:- Procainamide or ibutilide. Caution: Adenosine, CCB, Beta blockers results in increase in conduction via accessory pathway with possible degeneration into VT or VF

Clinical Features[edit | edit source]

• People with Pre- Excitation syndromes are usually asymptomatic , however the individual may experience following symptoms
  • Palpitations
  • Dizziness or lightheadedness.
  • Shortness of breath.
  • Chest pain
  • Fatigue.
  • Anxiety.
  • Fainting
  • Difficulty breathing
    

Differentiating [disease name] from other Diseases[edit | edit source]

• Pre-Excitation syndrome must be differentiated from other diseases. The conditions that needs to be ruled out while making the diagnosis are:

• [Differential dx1]
• [Differential dx2]
• [Differential dx3]

Epidemiology and Demographics[edit | edit source]

• The prevalence of [disease name] is approximately [number or range] per 100,000 individuals worldwide.
• In [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location].

Age[edit | edit source]

• Patients of all age groups may develop [disease name].

• [Disease name] is more commonly observed among patients aged [age range] years old.
• [Disease name] is more commonly observed among [elderly patients/young patients/children].

Gender[edit | edit source]

• [Disease name] affects men and women equally.

• [Gender 1] are more commonly affected with [disease name] than [gender 2].
• The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.

Race[edit | edit source]

• There is no racial predilection for [disease name].

• [Disease name] usually affects individuals of the [race 1] race.
• [Race 2] individuals are less likely to develop [disease name].

Risk Factors[edit | edit source]

• Common risk factors in the development of [disease name] are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].

Natural History, Complications and Prognosis[edit | edit source]

• The majority of patients with [disease name] remain asymptomatic for [duration/years].
• Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
• If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
• Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
• Prognosis is generally [excellent/good/poor], and the [1/5/10­year mortality/survival rate] of patients with [disease name] is approximately [#%].

Diagnosis[edit | edit source]

Diagnostic Criteria[edit | edit source]

• The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:

• [criterion 1]
• [criterion 2]
• [criterion 3]
• [criterion 4]

Symptoms[edit | edit source]

• [Disease name] is usually asymptomatic.
• Symptoms of [disease name] may include the following:

• [symptom 1]
• [symptom 2]
• [symptom 3]
• [symptom 4]
• [symptom 5]
• [symptom 6]

Physical Examination[edit | edit source]

• Patients with [disease name] usually appear [general appearance].
• Physical examination may be remarkable for:

• [finding 1]
• [finding 2]
• [finding 3]
• [finding 4]
• [finding 5]
• [finding 6]

Laboratory Findings[edit | edit source]

• There are no specific laboratory findings associated with [disease name].

• A [positive/negative] [test name] is diagnostic of [disease name].
• An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
• Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

Imaging Findings[edit | edit source]

• There are no [imaging study] findings associated with [disease name].

• [Imaging study 1] is the imaging modality of choice for [disease name].
• On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
• [Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].

Other Diagnostic Studies[edit | edit source]

• [Disease name] may also be diagnosed using [diagnostic study name].
• Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].

Treatment[edit | edit source]

Medical Therapy[edit | edit source]

• There is no treatment for [disease name]; the mainstay of therapy is supportive care.

• The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
• [Medical therapy 1] acts by [mechanism of action 1].
• Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

Surgery[edit | edit source]

• Surgery is the mainstay of therapy for [disease name].
• [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
• [Surgical procedure] can only be performed for patients with [disease stage] [disease name].

Prevention[edit | edit source]

• There are no primary preventive measures available for [disease name].

• Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

• Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].

Pre-excitation syndrome is a condition where the the ventricles of the heart become depolarized too early, which leads to their partially premature contraction. Normally, the atria (chambers taking venous blood) and the ventriculi (chambers pro-pulsing blood towards organs) are electrically isolated, and only electrical passage exists at "atrioventricular node". In all pre-excitation syndromes, there is at least one more conductive pathway is present. Physiologically, the electrical depolarization wave 'waits' in atrioventricular node to allow atria contract before ventriculi. However, there is no such property exists in abnormal pathway, so electrical stimulus passes to ventricle by this tracts far before normal atrioventricular-his system, and ventricules are depolarized (excited) before (pre-) normal conduction system. The term pre-excitation derives from this condition.

It is usually caused by a secondary conduction pathway (other than the bundle of His):

Type	Conduction pathway	PR interval	QRS interval	Delta wave?
Wolff-Parkinson-White syndrome	Bundle of Kent (atria to ventricles)	short	long	yes
Lown-Ganong-Levine syndrome	"James bundle" (atria to bundle of His)	short	normal	no
Mahaim-type	Mahaim fibers	normal	long	yes

See also

• Electrical conduction system of the heart