Texas man, 54, diagnosed with rare, genetic form of sudden death syndrome

Karl Wiggins was driving with his mother in Liberty, Texas, in March when he suddenly collapsed behind the wheel.

His mother was able to gain control of the car so they didn’t crash and pulled over. A passerby who witnessed the event dialed 911.

EMT officers arrived at the scene and tried to revive the 54-year-old, but were unsuccessful. Later at the hospital, he was revived – only for his heart to stop again.

That’s when doctors discovered Wiggins was living with a rare, genetic form of sudden death syndrome called Brugada syndrome that causes sufferers to go into cardiac arrest with no noticeable symptoms.

Karl Wiggins, 54 (pictured)), was driving in Liberty, Texas, in March when he collapsed behind the wheel. He was revived once but his heart stopped beating, with this pattern occurring repeatedly

At the Memorial Hermann-Texas Medical Center in Houston, doctors diagnosed Wiggins with Brugada syndrome, a rare, genetic form of sudden death syndrome. Pictured: EMT officers working to revive Wiggins

At the Memorial Hermann-Texas Medical Center in Houston, doctors diagnosed Wiggins with Brugada syndrome, a rare, genetic form of sudden death syndrome. Pictured: EMT officers working to revive Wiggins

After collapsing, Wiggins was transported by helicopter to the Memorial Hermann-Texas Medical Center in Houston.

Doctors revived him several times only for his heart to stop, leaving them baffled.

Dr Siddharth Mukerji, a cardiac electrophysiologist at Memorial Hermann, said the diagnosis was clear after he looked at Wiggins’s test results.

‘We evaluated his EKG and we noticed that he had what we called the Brugada pattern,’ he told ABC 13. 

Brugada syndrome, a type of sudden death syndrome, is an inherited heart rhythm disorder that occurs in the heart’s lower chambers.

According to the National Institutes of Health (NIH), it is caused mainly by a mutation of the SCN5A gene, which provides instructions for making a sodium channel.

This channel transports positively charged sodium ions into heart muscle cells, which play a role in controlling heart rhythm.

Many with the condition don’t know they have it and go undiagnosed because there are no noticeable symptoms.

However, there are signs of a ‘Brugada pattern’ including dizziness, fainting, irregular heartbeats and difficulty breathing.  

‘Frequently, sudden death can be the first manifestation of the disease,’ according to the National Organization of Rare Diseases.  

Brugada is an inherited heart rhythm disorder that occurs in the lower chambers but, because symptoms aren't noticeable, the condition often goes undiagnosed. Pictured: Wiggins

Brugada is an inherited heart rhythm disorder that occurs in the lower chambers but, because symptoms aren’t noticeable, the condition often goes undiagnosed. Pictured: Wiggins

Doctors installed an implantable cardioverter-defibrillator, which will deliver electrical shocks when the heartbeat appears irregular. Pictured: Wiggins's surgical scar

Doctors installed an implantable cardioverter-defibrillator, which will deliver electrical shocks when the heartbeat appears irregular. Pictured: Wiggins’s surgical scar

The NIH says that Brugada is estimated to affect around one in 2,000 people worldwide. 

It has earned the nickname ‘The Moaning Death’ in the Philippines because sufferers are often heard moaning at night before they die suddenly. 

To treat Wiggins, doctors installed what is known as an implantable cardioverter-defibrillator (ICD).

Two ends of a flexible, insulated lead wire are attached to the lower pumping chambers of the heart, according to the Mayo Clinic.

The other ends are secured to a shock generator, which is implanted underneath the collarbone. 

The ICD continuously monitors heartbeat and will deliver electrical shocks when the heartbeat appears irregular.

Wiggins said he’s been doing well since getting the ICD and adds he is no longer afraid of death.

‘I think if someone put a gun to my head and threatened to kill me, I would say: “Shoot your best shot. I’ve been dead before”,’ he told ABC 13. 

  

Read more at DailyMail.co.uk