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

• Asymptomatic

  32 Male − − PS 200 − 22 3 Asymptomatic 32 Female − − LFW 200 + 15 4 Asymptomatic 10 Male − − PS 220 + 25 5 Asymptomatic 10 Male − + LFW+PS 220 + 31 6 Asymptomatic 12 Male − + LFW+PS 210 + 15 7 Asymptomatic 8 Male − − PS 220 + 22 8 Asymptomatic 10 Male [circ.ahajournals.org]

  The risk for asymptomatic pediatric patients with WPW syndrome is not well defined. [pediatrics.aappublications.org]

  […] recommended. 1 We recently reported that a particular subgroup of asymptomatic patients may be at risk for an arrhythmic event during follow-up. 2 We also demonstrated the value of electrophysiological testing for stratifying asymptomatic patients into [content.nejm.org]

• Weakness

  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]

  Palpitations (sensation of rapid or irregular beating of the heart), weakness, and shortness of breath may occur. [web.archive.org]

  However some families may have more than one affected individual and there does appear to be a weak hereditary tendency, with an increased incidence in the children of affected individuals (4-5 per 1000). [betterhealth.vic.gov.au]

• Fatigue

  These arrhythmias are typically not life-threatening but can lead to symptoms such as palpitations, shortness of breath, chest discomfort, fatigue, dizziness, or fainting spells. [houstoncardiovascular.com]

  We ask about general symptoms (anxious mood, depressed mood, fatigue, pain, and stress) regardless of condition. Last updated: May 13, 2019 [patientslikeme.com]

  They include palpitations, lightheadedness or dizziness, and extreme fatigue. Episodes usually last from a few minutes to several hours. [verywellhealth.com]

  At the onset of her sophomore year under a new coach, the patient occasionally experienced fatigue, light-headedness, dyspnea, and chest discomfort with athletic practice. [journals.lww.com]

  If you are symptomatic, symptoms of mild to moderate Wolff-Parkinson-White syndrome can include: Heart palpitations (rapid heartbeat) Fainting Fatigue Anxiety Shortness of breath The rapid heartbeat associated with Wolff-Parkinson-White syndrome can last [mercy.com]

• Congestive Heart Failure

  Chest X-ray showed heart enlargement and pulmonary congestion. [ncbi.nlm.nih.gov]

  Prenatal and infantile presentation of SVT is often associated with signs of congestive heart failure, perhaps due in part to the relatively prolonged interval … View Full Text Request Permissions If you wish to reuse any or all of this article please [heart.bmj.com]

  Patients with WPW syndrome may experience palpitations, dizziness, syncope, congestive heart failure or sudden cardiac death (SCD). [journals.lww.com]

  heart failure. we spent a few days in the icu, then went home feeling confident in the diagnosis and knowing we would just have to wait and see what the genetic test had to say. well, a week and a half later, he began acting not quite right again so [inspire.com]

  Class Ic drugs should not be given if the patient has structural heart disease (coronary artery disease, myocardial infarction, congestive heart failure, left ventricular hypertrophy). [emedicine.medscape.com]

• Unconsciousness

  This can precede unconsciousness or syncope. WPW can only be diagnosed with the help of an ECG. This shows the heart rhythm and the pathway of electrical excitation in the form of a curve. [cardiosecur.com]

  This man became unconscious during jogging. In the emergency department, he was found to be in cardiac arrest due to ventricular fibrillation that was successfully cardioverted only after six shocks of up to 300 J. [content.nejm.org]

  These signs included coughing, gasping, colour, twitching, and movement.[51] However, in face of evidence that these guidelines were ineffective, the current recommendation of ILCOR is that cardiac arrest should be diagnosed in all casualties who are unconscious [en.wikipedia.org]

• Nausea

  Following administration of diltiazem by EMS, he felt worse and had nausea and vomiting. This intense vomiting then led to conversion to normal sinus rhythm with obvious pre-excitation. Troponin on admission was elevated. [onlinejacc.org]

  For example, verapamil and diltiazem can cause dizziness, nausea, headaches, and fatigue. All of these side effects can significantly diminish one's execution during physical activity. [journals.lww.com]

  "Depending on where you were you could have problems like nausea and vomiting or diarrhea," she said. "In an earthquake, suppose someone had a crushed foot or a finger amputation. Suppose you were the one with knowledge and had to direct everyone." [pleasantonweekly.com]

  Exophthalmos (bulging eyes) Eye cancer Eye floaters Eye infection (herpes) Eye injuries Eye tests for children Eyelid problems Back to top F Fabricated or induced illness Face blindness Fainting Falls Farting (flatulence) Febrile seizures Feeling sick (nausea [nhs.uk]

  Cardiac arrest is a sudden loss of blood flow resulting from the failure of the heart to pump effectively.[11] Signs include loss of consciousness and abnormal or absent breathing.[1][2] Some individuals may experience chest pain, shortness of breath, or nausea [en.wikipedia.org]

• Choking

  "It's exciting because, especially after I teach a CPR class, I will invariably have someone come back and say, 'Someone was choking and I did the Heimlich maneuver or knew what to do.'" [pleasantonweekly.com]

• Tachycardia

  […] that leads to periods of rapid heart rate (tachycardia). [mountsinai.org]

  This is called re-entry tachycardia or SVT (supraventricular tachycardia). [my.clevelandclinic.org]

  WPW syndrome is a supraventricular tachycardia – a fast heart rhythm, with a rate of more than 100 beats per minute. It begins above the ventricles, the heart’s two lower chambers. [aurorahealthcare.org]

  Supraventricular tachycardia refers to a group of heart rhythm disorders that causes a rapid heartbeat and originates from one of the top chambers of the heart. [houstoncardiovascular.com]

  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]

• Palpitations

  Palpitations are, however, the main symptom of WPW. Some people may find these palpitations untroubling, but in others they may cause chest pain, light-headedness and even blackouts. [c-r-y.org.uk]

  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]

  Her palpitations only came when she exercised. Is this normal? When teenagers or people in their early 20s first experience an arrhythmia due to this syndrome, palpitations can begin suddenly, often during exercise. [irishtimes.com]

  These arrhythmias are typically not life-threatening but can lead to symptoms such as palpitations, shortness of breath, chest discomfort, fatigue, dizziness, or fainting spells. [houstoncardiovascular.com]

  He complained of paroxysmal palpitations, dizziness, and shortness of breath followed by presyncope and syncope. He has had subtle and infrequent palpitation and shortness of breath since childhood. [hindawi.com]

• Heart Disease

  heart disease–1 case; 4) familial cardiomegaiy–2 cases; and 5) disease of the central nervous system–5 cases. [pediatrics.aappublications.org]

  Causes and symptoms Congenital heart disease may contribute to this and other arrhythmias. [encyclopedia.com]

  Type A Wolff-Parkinson-White syndrome was most common (49%), with heart disease occurring in only 5% of these patients. In contrast, heart disease was identified in 45% of those with type B syndrome. [ncbi.nlm.nih.gov]

  One of the larger studies1 looking at the association between WPW and acquired or congenital heart disease was performed in France. [scielo.br]

• 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]

  Treatment is needed for fainting, dizziness or chest pains. A very rapid heartbeat can be life-threatening. Treatments include drugs called antiarrhythmics. Medicines may need to be taken indefinitely. [carle.org]

• Heart Failure

  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]

  In the past 9 months, she began to show signs of heart failure in the form of New York Heart Association functional class II effort dyspnea. Physical examination was normal, but during exercise testing she only achieved 6 METs. [revespcardiol.org]

• Sweating

  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]

  Typical symptoms include: rapid or irregular pounding heartbeats (which can last anywhere from several seconds to several hours) shortness of breath dizziness lightheadedness fainting (syncope) sweating chest pain or tightness heart palpitations What [nicklauschildrens.org]

  During an episode, you may experience symptoms such as: a pounding or fluttering heartbeat ( heart palpitations ) feeling light-headed or dizzy shortness of breath chest pain sweating feeling anxious finding physical activity exhausting passing out [nhs.uk]

  However, the individual may experience • palpitations, • dizziness, • shortness of breath, • syncope • sweating 4. Pathophysiology 5. [slideshare.net]

  In mild cases, this can cause: a pounding heart (palpitations) dizziness or light-headedness sweating a feeling of anxiety Young children might not be able to describe how they are feeling, but you might notice they are pale and restless, with a low appetite [healthdirect.gov.au]

• Prolapse

  […] biphasic or negative delta wave in leads V1-V3 - common types of arrhythmias at presentation: reciprocating tachycardia (80%), atrial fibrillation(15%), atrial flutter (5%) - associations : Hypertrophic cardiomyopathy (HCM), Ebstein's anomaly, mitral valve prolapse [mrcpart1revision.blogspot.com]

  Two-dimensional echocardiogram (68 out of 173) was normal in 44 patients, while 24 showed minor cardiac abnormalities with two major disease; mitral valve prolapse was diagnosed in 8 patients. [ncbi.nlm.nih.gov]

  May be associated with congenital cardiac defects, Ebstein's anomaly, mitral valve prolapse, hypertrophic cardiomyopathy or other cardiomyopathies. [web.archive.org]

  Clinical features of associated cardiac defects may be present - eg, mitral valve prolapse, cardiomyopathy. Differential diagnosis [ 6 ] Atrial fibrillation. Atrial flutter. Atrioventricular nodal re-entry tachycardia (AVNRT). [patient.co.uk]

• Osteoporosis

  Obesity Obsessive compulsive disorder (OCD) Occupational therapy Oesophageal atresia and tracheo-oesophageal fistula Oesophageal cancer Oral thrush (mouth thrush) Orf Orthodontics Osteoarthritis Osteomalacia Osteomyelitis Osteopathy Osteophyte (bone spur) Osteoporosis [nhs.uk]

• Excitement

  A significant proportion of responders (50.9%) would use electrical cardioversion to restore sinus rhythm in a patient with pre-excited atrial fibrillation. [ncbi.nlm.nih.gov]

  English Wolff-Parkinson-White syndrome disorder due to a specific type of problem with the electrical system of the heart which has resulted in symptoms Anomalous A-V excitation anomalous atrioventricular excitation WPW syndrome Wolff-Parkinson-White [wikidata.org]

  Convert to ICD-10-CM : 426.7 converts approximately to: 2015/16 ICD-10-CM I45.6 Pre-excitation syndrome Approximate Synonyms ANOMALOUS ATRIOVENTRICULAR EXCITATION Anomalous AV excitation Wolff Parkinson White syndrome Wolff-Parkinson-White pattern Clinical [icd9data.com]

• Dizziness

  These arrhythmias are typically not life-threatening but can lead to symptoms such as palpitations, shortness of breath, chest discomfort, fatigue, dizziness, or fainting spells. [houstoncardiovascular.com]

  Symptoms of WPW may include one or more of the following: Heart palpitations – a sudden pounding, fluttering or Racing feeling in your chest Dizziness – feeling lightheaded or faint Shortness of breath (dyspnea) Anxiety Rarely, cardiac arrest (sudden [my.clevelandclinic.org]

  Surgery Share on Pinterest 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]

  Symptoms can include episodes of tachycardia (rapid heart rhythm), dizziness, chest palpitations, fainting or, rarely, cardiac arrest. [irishtimes.com]

  People with WPW may experience palpitations, dizziness, lightheadedness, and fainting, although some people with WPW have no symptoms. [omicsonline.org]

• Seizure

  A 10-year-old girl presented to the emergency department of a regional hospital with 1 episode of generalized tonic-clonic seizures. [ncbi.nlm.nih.gov]

  A LTE was defined as sudden death, aborted sudden death, or rapid conduction over the accessory pathway (AP) during atrial fibrillation (AF) with symptoms (e.g., syncope or seizure) or with the shortest pre-excited RR interval (SPERRI) during AF ≤250 [acc.org]

  Age at onset of MELAS syndrome was described as the age at which the patient experienced his or her first seizure or strokelike event based on clinical history and medical records. [jamanetwork.com]

  Feeling sick (nausea) Female genital mutilation (FGM) Femoral hernia repair Fever in children Fibroids Fibromyalgia First aid Fits (children with fever) Fits (seizures) Flat feet Flat head syndrome Floaters and flashes in the eyes Flu Fluoride Foetal [nhs.uk]

• Convulsions

  Additional heart rhythm disorders are: Adams-Stokes Disease Adams-Stokes Disease, also called Stokes-Adams disease, refers to a heart block that can slow the heart rate considerably, causing fainting (syncope) or convulsions. [heart.org]

  Prior participation in any clinical trial within 30 days prior to the screening visit 9)Patients predisposed to inflammatory reaction due to use of medical devices such as electroporation within 30 days of screening visit 10)Past history of epilepsy or convulsion [cris.nih.go.kr]

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

  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]

• Short PR Interval

  Delta waves and short PR interval is seen. Click here for a more detailed ECG [metealpaslan.com]

  However, none of the identified short PR interval mechanisms is necessary for the generation of LGL tachycardias. [emedicine.medscape.com]

  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]

  The syndrome is characterized by a short PR interval and a long QRS interval with a delta wave. [fpnotebook.com]

• 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 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].

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].

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.

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.

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.

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.

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.

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.

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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