Episode 58: Wolff-Parkinson-White Syndrome - Extra Connections aren’t always Good - Anatomy Of Illness

Episode Transcript
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(00:00):
As you sit in class, an unsettling sensation begins to wash over you, making you feel strangely

(00:05):
short of breath despite having not moved from your seat.
It's as if you've been running for miles.
Your heart pounds violently, feeling as if it is trying to escape your chest, each beat
echoing in your ears, drowning out the sounds around you.
The dizziness comes next, and the room around you seems to spin uncontrollably.
Just when you think you might snap back into reality, darkness takes hold, and you faint,

(00:28):
falling into an abyss where time and space loses all meaning.
Hello and Welcome to Anatomy Of Illness.
Today's episode is about Wolff-Parkinson-White syndrome.

(00:50):
Before we get into the condition, we are going to start with the history.
So why do we know Wolff-Parkinson-White syndrome exists?
We are going to begin in 1893.
This is where Stanley Kent would discover some of the atrioventricular connections.
So part of the heart's electrical system.
A little while later, we would have the work of both His and Tawara.
They gave us the anatomy of the atrioventricular node and the His-Purkinje system.

(01:13):
This will be important.
Now we move to an article published in August 1930.
This was in the American Heart Journal.
This article was written by three doctors.
Who were these doctors you might ask?
Well, they were doctors Wolff, Parkinson and White.
This article described 11 patients who were having the same symptoms.
They all had short PR intervals and bundle branch blocks.

(01:33):
PR intervals are part of the heart rhythm you will see in an ECG.
These people were also having paroxysmal supraventricular tachycardia.
So sudden short bursts of a faster than normal heart rate above the bottom chambers in the heart.
Or they were experiencing atrial fibrillation,
which is when the upper chambers of the heart beat out of sync with the lower chambers.
However, there was no evidence of structural cardiac disease in these patients that was causing the condition.

(01:59):
Not long after this, we would have the contributions from Mines.
They discussed a concept called circus movement of tachycardia as an explanation for tachycardia.
Tachycardia is a rapid heartbeat and circus movement tachycardia is caused when the electrical impulses move in a circular path in the heart.
This causes a rapid heartbeat and an abnormal heart rhythm.
Now we move to 1952 with Holzmann and Scherf.

(02:22):
They described something called pre-excitation due to anterograde conduction over an accessory AV connection.
Pre-excitation is when an electrical impulse reaches the ventricles too early.
The ventricles are the bottom part of the heart.
Anterograde means something happening forward in time.
So this essentially means the ventricles are being triggered before they are supposed to be due to an extra atrioventricular connection.

(02:43):
So an extra electrical connection in the heart between the top and bottom sections.
In 1981, Scheinman and their colleagues would come up with a surgical intervention that would be important for the treatment of several conditions including Wolff-Parkinson-White syndrome.
What could this be?
Catheter ablation of the AV junction.
What this technique does is use high energy direct current shocks to the area of the AV junction.

(03:04):
This is done to create scar tissue limiting the extra signals.
A few years later in 1984, Morady and Scheinman would demonstrate a 65% success rate in using the catheter ablation technique on accessory pathways in the right posterior septal area.
Essentially the right side of the back of the septum which is the dividing wall of the heart.
This was able to prevent the accessory pathways in Wolff-Parkinson-White syndrome from sending extra signals in many cases.

(03:29):
With more advances we would get radiofrequency catheter ablation which allowed for better mapping and better success rates in the treatment of Wolff-Parkinson-White syndrome.
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So what actually is Wolff-Parkinson-White syndrome?
It is a type of heart condition that is also sometimes known as pre-excitation syndrome.
With this there is an extra pathway in the heart connecting the upper and lower chambers that skips the normal pathway.

(03:53):
This prevents the heart from slowing down as you have both the regular signal and the extra signal telling the lower chambers to beat.
With the two signals telling the lower chamber to beat rather than only one, it can cause the heart rate to speed up leading to tachycardia or a rapid heart rate.
This extra pathway can also cause signals to travel backwards occasionally leading to an uncoordinated heart rhythm or an arrhythmia.

(04:13):
What actually causes Wolff-Parkinson-White syndrome?
Wolff-Parkinson-White syndrome is a congenital heart condition.
This means it is something you are born with that is often found later in life.
Wolff-Parkinson-White syndrome can be inherited or familial.
So there are multiple family members with the condition meaning that it has a genetic cause that is passed down.
When it comes to inherited Wolff-Parkinson-White syndrome, so just this condition, none of the related conditions, it is inherited in an autosomal dominant inheritance pattern.

(04:38):
So you need only one copy from either parent to have the condition.
The gene that will cause this condition in some is a mutated variant of the PRKAG2 gene.
However, in many cases the specific cause is unknown as there is no other known family members with the condition and genetics behind Wolff-Parkinson-White syndrome are currently unknown in many cases.

(04:59):
So these people are born with a condition without a known genetic cause for it.
Speaking of genes, we have some great clothes on our website merchanatomy.com.
How do we diagnose Wolff-Parkinson-White syndrome?
After discussing with a physician the symptoms you are experiencing, they will order several tests that can include an electrocardiogram.
You may know this as an ECG or an EKG.
This is the sticky dot electrodes that are placed on the chest.

(05:21):
Occasionally there are additional ones placed on the arms or legs.
This device reads the electrical activity in the heart.
Sometimes electrocardiograms may be done on the treadmill.
This can be called a stress ECG.
There are some patterns on this that can suggest an extra pathway, but more testing will be needed for a definitive diagnosis.
Holter monitor.
This is a device similar to the electrocardiogram, except it is a portable device that you wear for a longer period of time.

(05:46):
Normally a day or two whilst going about your daily activities.
Event recorder.
This device is similar to a Holter monitor, however you wear it for even longer, for up to 30 days in most cases.
This device only records for a few minutes at a time, whereas a Holter monitor is constantly recording.
With an event recorder, you will push a button when you feel symptoms coming on, or in some cases with some devices, it will start recording when it detects an abnormal rhythm.

(06:09):
Electrophysiology study.
This is a test done through a catheter inserted in the groin, guided to the heart.
The sensors in the catheters are able to record the heart's electrical patterns.
This allows for a diagnosis of Wolff-Parkinson-White syndrome, differentiating it from other similar conditions.
We'll get right into the symptoms and presentation right after this little break.
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(06:42):
What symptoms might you have if you had Wolff-Parkinson-White syndrome?
The symptoms you may have if you had this condition include feelings of anxiety, dizziness and lightheadedness, fainting.
Your doctor may call this syncope. Shortness of breath, chest pain, fatigue, rapid fluttering or pounding heartbeats.
You may know these as palpitations.
Having episodes of rapid heart rates, in many cases, this can be a heart rate above 200 beats per minute. Drops in blood pressure or hypotension.

(07:07):
Infants can also have symptoms of Wolff-Parkinson-White syndrome.
Their symptoms include blue or gray skin, lips and nails, restlessness and irritability, rapid breathing and poor eating.
What complications should a doctor be aware of when it comes to Wolff-Parkinson-White syndrome?
The main complication doctors should be aware of when it comes to this condition is sudden cardiac death.
This is mostly seen in young children and young adults.

(07:28):
Doctors should also be aware of heart failure.
This is due to the strain put on the heart from the periods of rapid heart rate.
How do we treat Wolff-Parkinson-White syndrome?
When treating Wolff-Parkinson-White syndrome, a lot of the time it comes to managing your symptoms when they come up and how severe these symptoms are when they do occur.
There are several options that can be used when it comes to treating the symptoms.
These include vagal maneuvers.

(07:48):
These are actions that can be taken to slow down the heart rate.
This is various things like coughing, putting an ice pack on your face or breathing like you would to blow up a balloon.
This is known as a Valsalva maneuver when you forcefully exhale with a closed or a semi-closed airway.
For those that the vagal and Valsalva type maneuvers don't work on, more interventions may be required.
This can be things like medications.

(08:10):
There are a variety of medications that can be used to slow the heart rate.
Some of these are administered through IV, so in the vein.
So it will depend on the case of a person and their symptoms as to what they are given.
Cardioversion.
This is where the heart rhythm is reset, often through paddles or patches, shocking the heart through electricity.
In some cases, this is also done through medications.

(08:30):
For more long-term management, if there are frequent symptom episodes, doctors may recommend catheter ablation.
This is the surgery that I mentioned in the history section.
This surgery guides a catheter, which is a flexible tube through an artery normally in the groin.
This is guided into the heart.
It uses little bursts of energy to create scars in the heart to block the electrical signals causing the fast heart rate or abnormal heart rhythms.

(08:51):
This is done through causing scar tissue on the little extra pathway that you have in Wolf-Parkinson-White.
Are there any famous people with Wolf-Parkinson-White syndrome?
Yes, there is a singer, Jessie J.
She inherited the condition from her father, who got it from his father.
She claims that she started writing her music as a way to help with being ill.
If you want to check out her social media page, there is @BellaWillWalkAgain on Instagram.

(09:12):
She has Wolf-Parkinson-White syndrome, after an episode of the condition she needed to have surgical intervention.
However, she had a rare reaction to the anesthesia, which led to paralysis.
Currently, she is going through the journey of learning to walk again.
On her Instagram, she takes you along for the journey, which is why I have linked it on the episode description.
If you want to check out an episode of Sonic on TV featuring the condition, there is Grey's Anatomy season 12 episode 13.

(09:35):
All Eyez On Me.
In this episode, we have a group of cheerleaders who are brought into the emergency room department due to a cheerleading accident.
They eventually find out one of the cheerleaders has Wolf-Parkinson-White syndrome and treat her for the condition.
If you would like to check out a foundation, there is, for those in the US, the Sudden Arrhythmia Death Syndrome Foundation.
They aim to increase awareness of conditions that can cause sudden death, increase awareness on the early warning signs and symptoms,

(09:58):
and also provide support to patients and families with these conditions.
For those in the UK, there is the British Heart Foundation.
They raise money for research into treatments and cures for heart conditions.
They also provide an online community to allow those who have been affected by various heart conditions to connect.
For those in Australia, there is the Eloise Gannon Foundation, whose goal is to raise awareness of undiagnosed arrhythmias and sudden cardiac arrests.

(10:19):
This foundation honours the memory of Eloise Gannon, who had Wolf-Parkinson-White syndrome, who passed away suddenly in her sleep at 15 years old.
If you want to check out the sources, social media links or any other links, you can head to anatomyofillness.com.
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(10:54):
Did you know the Heart beats over 100,000 times a day.

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Episode 58: Wolff-Parkinson-White Syndrome - Extra Connections aren’t always Good

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