WOLFF-PARKINSON-WHITE SYNDROME: CAUSES, COMPLICATIONS, ABLATION, AND MANAGEMENT
Medically Reviewed by Dr. Sony Sherpa (MBBS) - August 14, 2024
Wolff-Parkinson-White syndrome (WPW) is a rare heart condition that affects approximately 1-3 people out of every 1,000.[1]
It is an abnormal heart rhythm (arrhythmia) driven by an additional electrical pathway in the heart. This extra pathway can cause a rapid heart rate, known as tachycardia, defined as a pulse rate of over 100 beats per minute. Episodes of tachycardia can lead to severe complications if left untreated.
This article will explore the causes, complications, and treatment options for Wolff-Parkinson-White syndrome.
What Causes Wolff-Parkinson-White Syndrome?
WPW syndrome, sometimes known as pre-excitation syndrome, is a congenital heart defect, meaning it is present at birth. It occurs when there is an extra electrical route between the heart's atria (upper section) and ventricles (lower section). This extra pathway is known as an accessory pathway, while the standard pathway is known as the AV node.[2]
Under usual circumstances, the upper chambers of the heart contract in response to electrical impulses. The impulses travel to the lower chambers at a rate slow enough to allow them to fill with blood first. In WPW syndrome, the extra electrical pathway is quicker than the standard pathway, causing the lower chambers to contract faster and more frequently than in a healthy heart. This heightened activity is known as pre-excitation.
The exact cause of the accessory pathway has yet to be fully understood. It is likely the result of abnormal development of the heart during fetal development.
Not everyone with WPW syndrome has tachycardia. Some people have a less active accessory pathway or may only develop symptoms later. This is known as a WPW pattern and is visible on an electrocardiogram (ECG).
The most prevalent type of tachycardia in WPW patients is atrioventricular reentrant tachycardia (AVRT), the most common type of supraventricular tachycardia[3].
In this type, an electric pulse moves down either the AV node or the accessory path and then "re-enters" the chamber by moving up the other pathway, forming a revolving loop. This signal loop causes the ventricles to contract at an exceedingly high speed, increasing the heart rate to 140 and 250 beats per minute.
Risk Factors
Risk factors of Wolff-Parkinson-White syndrome (WPW) include:
- Congenital Heart Defects: WPW syndrome is often associated with other congenital heart defects, such as Ebstein anomaly.
- Genetic Conditions: WPW syndrome is more common in individuals with certain genetic conditions.
- Heart Conditions: While rare, those with heart conditions, such as hypertrophic cardiomyopathy or heart damage arising from surgery, can acquire WPW syndrome.
- Family History: A family history of WPW syndrome increases the risk of developing the condition.
- Age: WPW syndrome is typically present at birth. For some, symptoms may only appear later during childhood or young adulthood.
- Gender: WPW syndrome is slightly more common in males than females.
These risk factors do not guarantee WPW syndrome, yet they might make it more likely.
If you have WPW syndrome, it is best to speak to a doctor and opt for an accurate diagnosis and advice.
WPW Syndrome Symptoms
The most common symptom of WPW syndrome is a rapid heart rate, also known as tachycardia.
Symptoms of tachycardia include:
- Heart palpitations (a fluttering or pounding sensation in the chest or neck)
- Erratic heartbeats
- Dizziness or lightheadedness
- Fainting or near-fainting episodes
- Shortness of breath
- Chest pain
- Fatigue
- Anxiety or panic attacks
- Profuse sweating
Signs of WPW in infants might include[4]:
- Bluish or grayish hue in skin, lips, and nails (skin tone may affect the visibility)
- Agitation or fussiness
- Rapid breathing
- Subpar feeding habits
Tachycardia is not usually continuous and tends to manifest in episodes. It can start and stop abruptly, persisting for several seconds to hours. Some experience episodes daily, while others only get them a few times a year.
There are no triggers specific to the syndrome, yet exercise, caffeine, stress, or alcohol may trigger tachycardia in those with WPW syndrome.
People with WPW syndrome need to monitor their pulse frequently to ensure it remains within a manageable range.
In some instances, treatment can terminate the tachycardia and restore a normal rhythm.
Complications
If left untreated, WPW syndrome can lead to severe complications.
Atrial fibrillation: In atrial fibrillation, the atria contract at a rapid rate of 350 to 600 beats per minute. If left untreated, this rapid, chaotic heartbeat can lead to cardiac arrest, which can be imminently life-threatening.
Factors associated with developing atrial fibrillation include[5]:
- Aging
- Genetic lineage from Europe
- Elevated blood pressure
- Being overweight
- Other heart conditions like cardiomyopathy, heart failure, and coronary artery disease
- Diabetes
- Overactive thyroid
- Chronic kidney disease
- High alcohol intake and smoking
Cardiac Arrest: In rare cases, the rapid heart rate caused by WPW syndrome can induce cardiac arrest, where suddenly the heart stops completely. Cardiac arrest can be lethal within a matter of minutes and demands immediate help from emergency services.
Ebstein Anomaly: WPW syndrome is often associated with other congenital heart defects, such as Ebstein anomaly.[6] These defects can cause other symptoms and complications, such as slow weight gain, leg and abdominal swelling, and exercise intolerance. They may require additional treatment, including corrective heart surgery.
When to See a Doctor
Visit your doctor if you consistently experience a rapid or noticeable heartbeat. It is crucial to have it examined, as it may indicate a severe condition.[7]
If you have WPW syndrome and you face an episode, initially attempt the procedures taught to you or consume the medication prescribed to you.
Call for emergency medical help if you or someone you know has:
- A fluctuating heartbeat that does not stabilize within a few minutes.
- Prolonged chest pain and discomfort in the arms, back, or jaw for longer than 15 minutes.
- Tachycardia coupled with nausea, vomiting, breathlessness, or perspiration.
- Loss of consciousness or fainting with revival failure.
How to Cope During a WPW Episode
Medication and vagal maneuvers may effectively calm the heart rate during an acute episode.
Vagal Maneuvers are methods intended to stimulate the nerve that interrupts the electrical signals in your heart. In the popular "Valsalva maneuver," you plug your nose, seal your mouth, and forcefully exhale.
When to Seek Medical Help
If vagal maneuvers are not effective, medications such as adenosine injections may be indicated in hemodynamically stable patients. Also known as chemical cardioversion [8], these injections can block any unusual signals causing the issue. However, they are not indicated for controlling long-term WPW syndrome as they block the normal AV node pathway.
The heart may need a manual rhythm correction if none of these measures work.
Cardioversion is a form of electric shock therapy that can restore the heart to its normal rhythm. It usually requires hospitalization or emergency medical services to perform.
Diagnosis
If you are experiencing symptoms of WPW syndrome, a doctor will perform a physical investigation and weigh the results against your medical history. In the absence of any known heart condition, they may execute a variety of tests to diagnose the condition.[9]
The first test used to diagnose WPW syndrome is usually an ECG.
- An electrocardiogram, or ECG, is a non-invasive test that measures the heart's electrical activity. It can detect abnormal heart rhythms and indicate an additional electrical pathway in the heart by observing pre-excitation or a delta wave.
Other tests may include:
- A Holter monitor is a small transportable device that uses removable skin sensors to register the user’s heart electrical activity for 24-48 hours. This device can help your doctor identify abnormal heart rhythms during daily activities.
- An electrophysiology study is a more invasive test that involves threading thin, flexible wires called catheters through a blood vessel and into the heart. This test allows your doctor to map the electrical pathways in your heart and identify any abnormalities.
How is Wolff-Parkinson-White Syndrome Treated?
The main goal of WPW syndrome treatment is to prevent episodes of rapid heart rate and reduce the risk of complications. The standard approach involves a combination of medication, lifestyle changes, and procedures such as ablation or heart surgery.
Medications prescribed to help control the rapid heart rate associated with WPW syndrome may include anti-arrhythmic drugs, like procainamide or amiodarone. These medications, along with beta-blockers and calcium channel blockers, tend to block the AV node.[10]
As they can exacerbate symptoms of WPW syndrome, they are usually used only during chemical cardioversion to calm the arrhythmia and end an episode that may lead to cardiac arrest.
They also cause several side effects, such as dizziness, constipation, headaches, and fatigue. If you experience any symptoms, speak to your doctor about your prescription and dose.[11]
Ablation
The enduring solution for WPW syndrome often involves catheter ablation. Ablation is often successful in treating WPW syndrome and can eliminate the need for medication.[12]
It involves a tube (catheter) introduced to the affected area through a tiny incision near the groin towards the heart region. Once in the heart tissue, either cold energy (cryoablation) or heat energy (radiofrequency ablation) ablates or destroys the accessory pathway responsible for tachycardia.
Ablation has a success rate of 85-95% in comparison to surgery, which has a higher complication rate.[13] However, Ablation and surgery may lead to complications such as infection or scarring.
Heart Surgery
In rare cases, open-heart surgery may be necessary to correct the extra electrical pathway in the heart or to insert a pacemaker.[14]
Surgery is considered if other treatments have been unsuccessful or if other underlying heart conditions need addressing.
Lifestyle Changes
Some lifestyle changes your doctor might recommend for those living with WPW syndrome include:
- Quitting smoking
- Following a nutritious diet
- Engaging in regular physical activity
- Avoid caffeine or other stimulating substances
- Keeping your weight within a healthy range
- Taking steps to control emotional stress
These suggestions can strengthen the heart and improve overall health and well-being.
You may also need to avoid the following AV node-blocking medications if you have atrial fibrillation and atrial flutter with Wolff Parkinson White syndrome[15]:
- Adenosine
- Digoxin
- Diltiazem
- Verapamil
- Amiodarone
- Sotalol
- Procainamide
- Quinidine
- Disopyramide
- Flecainide
- Propafenone
Prognosis and Outlook
The prognosis for those living with WPW syndrome is generally favorable, especially when appropriately managed. With advances in medical treatments and interventions, such as ablation, most people with WPW can live fulfilling lives.
However, it is essential to remain vigilant and adhere to recommended treatment plans to minimize risks.
If you have been diagnosed with WPW syndrome, collaborate with your doctor on managing your condition and reducing the risk of complications. A doctor may suggest making lifestyle changes, such as avoiding triggers that can cause a rapid heart rate and taking medication as prescribed.
Monitoring your heart health regularly and seeking medical attention if you experience any new or worsening symptoms is also important. Your doctor may recommend regular check-ups and tests, such as ECGs or Holter monitors, to monitor your heart's electrical activity.
Conclusion
Wolff-Parkinson-White syndrome is an uncommon heart condition that causes rapid heart rate episodes and leads to severe complications if left untreated. With appropriate treatment and management, most people with WPW syndrome can live an everyday, healthy life. If you are experiencing symptoms of WPW syndrome, a healthcare professional can help to manage your condition and lower the risk of potentially life-threatening complications.
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