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Wolff-Parkinson-White Syndrome

Syndrome Wolf Parkinson White


Presentation

Cardiac

Affected children present with chest pain or chest tightness accompanied with dyspnoea, paroxysmal nocturnal dyspnoea and palpitations. These symptoms may be accompanied by light headedness and dizziness, and sometimes the child may faint during an acute attack.

Other signs and symptoms may include pallor, irritability, tachypnoea and slight behavioural changes. Once the tachycardia episode is over, symptoms may disappear and the affected individual may return to normal

Weight Loss
  • She reported a 90-pound unintentional weight loss, increased appetite, irritability, night sweats and palpitations for 2 months. Physical examination revealed a heart rate (HR) of 269 bpm and a blood pressure of 116/94 mm Hg.[ncbi.nlm.nih.gov]
Sputum
  • At first, the patient sustained respiratory failure and weak cough reflex, thereafter repeated bronchoscopy was used to aspirate the sputum as well as control the pneumonia, which resulted in arrhythmia (AF and AVNRT).[ncbi.nlm.nih.gov]
Increased Appetite
  • She reported a 90-pound unintentional weight loss, increased appetite, irritability, night sweats and palpitations for 2 months. Physical examination revealed a heart rate (HR) of 269 bpm and a blood pressure of 116/94 mm Hg.[ncbi.nlm.nih.gov]
Tachycardia
  • The most common diagnosis found in the survey was ventricular tachycardia/broad QRS-complex tachycardia marked by approximately a half of the participants.[ncbi.nlm.nih.gov]
  • Immediately following this, her rhythm changed to wide QRS irregular tachycardia without hemodynamic compromise. She was suspected to have ventricular tachycardia and was treated with intravenous amiodarone with cardioversion to sinus rhythm.[ncbi.nlm.nih.gov]
  • Orthodromic Tachycardia It is the most common type of tachycardia in WPW syndrome. The reentry circuit passes antegradely through the AV node and retrogradely through the accessory pathway, producing a narrow complex tachycardia like a SVT.[symptoma.com]
  • AV re-entry tachycardia, or circus movement tachycardia: The accessory pathway only conducts in a retrograde manner and the condition remains latent until triggered.[web.archive.org]
  • We report a patient with Wolff-Parkinson-White syndrome who presented with two distinct tachycardias that represented atrioventricular reentrant tachycardia utilizing left lateral accessory pathway (AP) and idiopathic left ventricular tachycardia (ILVT[ncbi.nlm.nih.gov]
Palpitations
  • A 44-year-old woman with no medical history presented to the emergency department with a 2 h history of sudden onset chest pressure, palpitations, diaphoresis and shortness of breath.[ncbi.nlm.nih.gov]
  • We describe a 31-year-old patient without any previous cardiac disorder admitted to our hospital due to palpitations and concomitantly diagnosed as WPW syndrome and treated with radiofrequency catheter ablation.[ncbi.nlm.nih.gov]
  • However, some WPW cases do not have typical findings on ECG and may remain undiagnosed unless palpitations occur.[ncbi.nlm.nih.gov]
  • He suffered episodes of loss of consciousness preceded by palpitations when he performed strenuous physical exercise. The electrocardiogram reveals a Wolff-Parkinson-White syndrome.[ncbi.nlm.nih.gov]
  • A 56-year-old man was admitted because of palpitations and dyspnea. A 12-lead electrocardiogram showed irregular wide QRS complex tachycardia with a slur at the initial portion of the QRS complex.[ncbi.nlm.nih.gov]
Heart Disease
  • Myocardial dysfunction and associated congenital heart disease remain as risks of mortality. Copyright 2014 Elsevier Ireland Ltd. All rights reserved.[ncbi.nlm.nih.gov]
  • Review of the literature in MEDLINE using the search terms of congenital myotonia or Becker's disease and arrhythmia or heart disease only found 2 articles on Thomsen disease but none on Becker's disease.[ncbi.nlm.nih.gov]
  • heart disease–1 case; 4) familial cardiomegaiy–2 cases; and 5) disease of the central nervous system–5 cases.[pediatrics.aappublications.org]
  • Associated heart disease was present in 40 patients (9%).[ncbi.nlm.nih.gov]
  • The Wolff-Parkinson-White syndrome (WPW) is a benign heart disease with accessory pathways, which can result in cardiac arrhythmias.[ncbi.nlm.nih.gov]
Chest Pain
  • Chest pain is not an uncommon complaint among adolescents; however, it often leads them to seek emergency medical care.[ncbi.nlm.nih.gov]
  • It should be kept in mind if the patient complains of new onset of severe chest pain, an immediate angiography should be performed.[ncbi.nlm.nih.gov]
  • Modes of presentation included supraventricular tachycardia (38%), palpitations (22%), chest pain (5%), syncope (4%), atrial fibrillation (0.4%), sudden death (0.2%), and incidental findings (26%); data were unavailable in 4%.[ncbi.nlm.nih.gov]
  • The diagnosis of Wolff-Parkinson-White syndrome is typically reserved for patients who experience ventricular pre-excitation and symptoms that are related to paroxysmal supraventricular tachycardia, such as chest pain, dyspnea, dizziness, palpitations[ncbi.nlm.nih.gov]
  • Palpitations occurred in 16 patients (88.9%), chest pain in 11 (61.1%), dizziness in 13 (72.2%), syncope in 15 (83.3%), and dyspnea in 13 (72%).[ncbi.nlm.nih.gov]
Left Ventricular Dysfunction
  • Abstract Emerging evidence suggests that significant left ventricular dysfunction may arise in right-sided septal or paraseptal accessory pathways (APs) with Wolff-Parkinson-White syndrome, even in the absence of recurrent or incessant tachycardia.[ncbi.nlm.nih.gov]
  • These abnormal findings might reflect the long-term effects of dyskinesia, suggesting irreversible myocardial injury that ultimately causes left ventricular dysfunction.[ncbi.nlm.nih.gov]
Night Sweats
  • She reported a 90-pound unintentional weight loss, increased appetite, irritability, night sweats and palpitations for 2 months. Physical examination revealed a heart rate (HR) of 269 bpm and a blood pressure of 116/94 mm Hg.[ncbi.nlm.nih.gov]
Blurred Vision
  • The patients were asked to report any palpitations, asthenia, dyspnea (at rest, during effort, or both), dizziness, chest pain, blurred vision, or syncope.[doi.org]
Dizziness
  • Sixty patients (34.6%) complained of different symptoms as palpitations, near syncope and dizziness. Fifty-three patients (30.6%) went in a regular sport activity.[ncbi.nlm.nih.gov]
  • The diagnosis of Wolff-Parkinson-White syndrome is typically reserved for patients who experience ventricular pre-excitation and symptoms that are related to paroxysmal supraventricular tachycardia, such as chest pain, dyspnea, dizziness, palpitations[ncbi.nlm.nih.gov]
  • Palpitations occurred in 16 patients (88.9%), chest pain in 11 (61.1%), dizziness in 13 (72.2%), syncope in 15 (83.3%), and dyspnea in 13 (72%).[ncbi.nlm.nih.gov]
  • ECG and electrophysiological examinations are required for early detection of PSVT and WPW syndrome for prompt resolution of potentially life-threatening complications in all AS patients, especially those presenting with the symptoms of palpitation, dizziness[ncbi.nlm.nih.gov]
  • Surgery If a person starts to experience dizziness or palpitations, they should see a doctor. Sometimes, a person may need more invasive procedures to prevent future episodes.[medicalnewstoday.com]
Gowers Sign
  • From age 27 years, he developed proximal muscle weakness predominantly of the lower limbs, a positive Gower sign, and a waddling gait.[ncbi.nlm.nih.gov]

Workup

Diagnosis of WPW syndrome can not be made without a complete work up first. After a detailed history and physical examination, the following tests should be conducted to rule out any other cause.

Laboratory Tests

  • Complete blood count
  • Liver Function Tests
  • Thyroid Panel
  • Urea/Creatinine/Electrolytes

Imaging Studies

  • Chest X-ray
  • Echocardiography
  • Series ECG monitoring
  • Electrophysiologic Studies (EPS)

Test Results

If the patient is not having an acute tachycardia episode, physical findings may appear normal. In such cases, routine ECGs such as by a Holter monitor and EPS may help in making a diagnosis

Wide QRS Complex
  • We report a 68-year-old man who presented with heart failure and atrial fibrillation (AF) with rapid ventricular response and wide QRS complexes.[ncbi.nlm.nih.gov]
  • A 12-lead electrocardiogram showed irregular wide QRS complex tachycardia with a slur at the initial portion of the QRS complex. He had preexisting long-standing persistent atrial fibrillation, but early excitation syndrome had never been noted.[ncbi.nlm.nih.gov]
  • Elective cardioversion restored sinus rhythm, and the ECG showed a wide QRS complex, short PR interval, and delta wave, indicating the presence of an accessory pathway and pre-excitation.[ncbi.nlm.nih.gov]
  • The electrocardiogram showed a PR interval of 0.10 second and wide QRS complexes suggestive of an accessory pathway. The case of a variant of Wolff-Parkinson-White syndrome is discussed.[ncbi.nlm.nih.gov]
  • A 56-year-old man with no previous medical history was admitted due to recurrent wide QRS complex tachycardia with hemodynamic collapse. A chest radiograph showed dextrocardia.[ncbi.nlm.nih.gov]
Short PR Interval
  • Wolff-Parkinson-White (WPW) syndrome is defined as the presence of an accessory atrioventricular pathway which is manifested as delta waves and short PR interval on electrocardiography (ECG).[ncbi.nlm.nih.gov]
  • Electrocardiography (ECG) of patients with WPW syndrome portrays a short PR interval and a wide QRS interval with a delta wave.[ncbi.nlm.nih.gov]
  • Elective cardioversion restored sinus rhythm, and the ECG showed a wide QRS complex, short PR interval, and delta wave, indicating the presence of an accessory pathway and pre-excitation.[ncbi.nlm.nih.gov]
  • We identified 173 cases of overt WPW pattern (short PR interval, delta wave, anomalous configuration of QRS complex) with a calculated incidence of 1.48/1000.[ncbi.nlm.nih.gov]
  • Her preoperative 12-lead electrocardiogram showed a sinus rhythm at 82 beats per minute, a delta wave, and a short PR interval. After an uneventful surgery, sugammadex 2mg/kg was administered as a reversal agent instead of neostigmine.[ncbi.nlm.nih.gov]
Electrocardiogram Change
  • The mechanism of the electrocardiogram changes was considered to be caused by the two drugs synergistic sodium channel blocking effects.[ncbi.nlm.nih.gov]

Treatment

Treatment consists of medication with radiofrequency ablation or open heart surgery.

Medications

In acute cases of arrhythmia, procainamide or amiodarone are the drugs of choice. Adenosine may also be given. Digoxin and calcium channel clockers should be avoided because they block the normal AV node and promote abnormal conduction in the accessory pathway.

Radiofrequency Ablation

It is the first line of therapy in symptomatic patients and is curative. A wire (electrode catheter) is advanced into the heart from large blood vessels and positioned within the chambers of the heart using fluoroscopy. After the accessory pathway is localised, radio frequency energy is delivered to the area to destroy or 'ablate' the accessory pathway, preventing it from conducting impulses [8].

Surgical Ablation

An open heart surgery may be performed to disconnect the pathway through which supraventricular excitations pass in WPW syndrome. The success rate for surgical ablation is close to 100% and the complication rate is low. However, radiofrequency ablation is a less invasive option; as a result, surgery is now rarely performed [9].

Prognosis

If correctly diagnosed and appropriately treated, the Wolff-Parkinson-White syndrome has an excellent prognosis. The success rate of treatment is almost 100%.

Asymptomatic patients generally have the best prognosis and symptomatic patients that are appropriately treated also achieve a long healthy life. However, some patients have a family history of arrhythmias or they may have other heart defects in which case, the prognosis becomes negatively affected.

Complications

Although periods of tachycardia are not life threatening, patients can develop serious heart problems [2]. Complications include trauma due to fainting, heart failure, hypotension, surgical complications, other arrhythmias like ventricular fibrillation (VF), etc.

VF is the most severe from of a rapid heart beat. It may rapidly lead to shock and death, and requires emergency treatment (cardioversion) [1] [8].

Etiology

The genesis of reentrant supraventricular tachycardia (SVT) involves the presence of dual conducting pathways between the atria and the ventricles [5]. These accessory pathways such as the bundle of Kent, allow reentry of impulses resulting in arrhythmias.

It may be inherited as a familial trait, with or without associated congenital heart defects [6]. The inheritance pattern is autosomal dominant, but in some extremely rare cases, it can be a result of mitochondrial inheritance. Mutations in the gamma 2 subunits of AMP activated protein kinase (PRKAG2), a protein kinase enzyme, encoded by the PRKAG2 gene [7] result in cardiomyopathies characterised by WPW syndrome, AV blocks and ventricular hypertrophy.

Infantile Pompe disease, Danon disease (an X linked lysosomal myopathy) and other types of glycogen storage diseases may also lead to development of WPW syndrome.

In rare cases, certain tumors present around the AV node or along the AV pathway and may cause WPW syndrome.

Epidemiology

Incidence and Prevalence

The prevalence of Wolff-Parkinson-White syndrome is between 0.1% and 0.3% in the general population [3]. Among these, sudden cardiac death is rare with an incidence of less than 0.6% [4].

Age

WPW syndrome commonly exhibits bimodal presentation, occurring either in infancy or between 5 to 15 years of age. It is rare in old age and in fact, incidence of WPW syndrome decreases with increase in age.

Sex

Studies reveal a predisposition in males, with the male to female ratio somewhere around 2:1. A 3.5 fold higher prevalence in boys and young men was also shown by another study.

Race

WPW syndrome exhibits no known predisposition to any race or demographic.

Sex distribution
Age distribution

Pathophysiology

ECG Changes

  • Shortened PR interval
  • Delta wave- which is the 'slurring' of the QRS complex is a characteristic finding.

According to the type of tachycardia and ECG changes produced and well as the pathway of reentry, WPW syndrome can be subdivided into three types:

Sinus Rhythm

In this type, the ventricles are depolarised through the AV node and accessory pathway. It produces a shortened PR interval and delta waves.

Orthodromic Tachycardia

It is the most common type of tachycardia in WPW syndrome. The reentry circuit passes antegradely through the AV node and retrogradely through the accessory pathway, producing a narrow complex tachycardia like a SVT. Sometimes, the QRS complex may be normal, delta waves may be absent and p waves may be typically inverted on lateral and inferior leads.

Pre-Excitatory Atrial Fibrillation/Antidromic Tachycardia

In this type, ventricles are largely depolarised by the accessory pathway producing an irregular, broad complex tachycardia. It is the most rare type occurring in only 5% of cases and can be easily confused with ventricular tachycardias.

Prevention

WPW syndrome has no known prevention. However, once diagnosed, the episodes of tachycardia may be prevented by the above mentioned treatments like ablation and medication.

Summary

Wolff-Parkinson-White (WPW) syndrome is a heart condition in which there is an abnormal extra electrical pathway of the heart [1]. It is defined as an ECG pattern sometimes associated with paroxysmal tachycardia. It consists of a short PR interval (usually 0.1 second or less;occasionally normal) together with a prolonged QRS complex with a slurred initial component (delta wave) [2]. 

In WPW syndrome, an accessory bundle of impulse pathway known as the bundle of Kent is present which sends preexcitatory impulses down to the ventricles causing arrhythmias.

Patient Information

WPW syndrome is one of the most common causes of tachycardia in infants and children. It is due to a congenital defect in the excitatory circuit pathway of the heart and can be successfully treated with ablation.

If an infant appears to resist feeding, becomes cyanotic or pale with an apparent strain on breathing and becomes irritable, the infant should be immediately checked for WPW syndrome. A child old enough to express himself may complain of chest pain, palpitations, dizziness, lethargy and difficulty in breathing during episodes of tachycardia.

References

Article

  1. Olgin JE, Zipes DP. Specific arrhythmias: diagnosis and treatment. In: Bonow RO, Mann DL, Zipes DP, Libby P, eds. Braunwald's Heart Disease:A Textbook of Cardiovascular Medicine. 9th edition. St. Louis. MO:WB Saunders: 2011; Chap 39.
  2. Nordqvist C. 2011 March 24. What is Wolff-Parkinson-White syndrome? What Causes Wolff-Parkinson-White Syndrome? Medical News Today. Retrieved from http//www.medicalnewstoday.com/articles/220163.php
  3. Sorbo MD, Buja GF, Miorelli M, Nistri S, Perrone C, Manca S, Grasso F, Giordano GM, Nava A 1995. The prevalence of Wolff-Parkinson-White syndrome in a population of 116,542 young males. Giornale Italiano di Cardiologia (in Italian). (25)6:681-7 PMID6749416.
  4. Munger DM, Packer DL, Hammil SC, Feldman BJ, Bailey KR, Ballard JR, Holmes DR Jr, Gersh BJ (1993). A population study of the natural history of Wolff-Parkinson-White syndrome in Olmsted County, Minnesota. 1953-1989. Circulation. 87(3):866-73. doi 10.1161/01.CIR.87.3.866 PMID 8443907.
  5. Sethi KK, Dhall A, Chadha GS, Garg S, Malani SK, Mathew OP. WPW and pre excitation syndromes. J Assoc Physicians India. Apr 2007;57 Suppl:10-5
  6. Ehtisham J, Watkins H. Is Wolff-Parkinson-White syndrome a genetic disease?. J Cardiovasc Electrophysiol. Nov 2005 16(11):1258-62 [Medline]
  7. Gollob MH. Modulating phenotypic expression of the PRKAG2 cardiac syndrome. Circulation 2008 117(2):134-5 
  8. Zimetbaum P. Cardiac arrhythmias with supraventricular origin. In: Goldman L, Schafer AL, eds. Cecil Medicine, 24th edition. Philadelphia. Pa: Saunders Elsevier ;2011 Chap 64
  9. Pappone C, Santinelli V. Should catheter ablation be performed in asymptomatic patients with Wolff-Parkinson-White syndrome? Catheter ablation should be performed in patients with Wolff-Parkinson-White syndrome. Circulation 2005;112:2207
  10. Hogenhuis W, Stevens SK, Wang P, et al. Cost effectiveness of radiofrequency ablation compared with other strategies in Wolff-Parkinson-White syndrome. Circulation 1993:88;437

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Last updated: 2018-06-21 19:11