Heart Inherited Heart Disorders

Researchers at St. Paul’s Hospital to launch first-in-Canada database for this heart condition

Darren Ali's proximity to St. Paul's Hospital after a heart attack was a critical factor in his positive outcome.

It was supposed to be a special mini getaway for Darren Ali and his wife.

In July 2019, the couple, who live 45 minutes outside Vancouver, booked an overnight stay at a downtown hotel. They made dinner reservations at a nearby restaurant.

There, Ali had a steak he recalls as “wonderful.”

But later at their hotel, Ali felt off – sweaty and unwell.

“I knew immediately what was happening,” says Ali, who is now 50.

Lucky to have been near St. Paul’s

Three months earlier, he’d seen his family doctor complaining of pain under his right shoulder blade. But the doctor told him everything was fine.

So, when the discomfort came that summer night, Ali made the connection.

He was rushed St. Paul’s Hospital and diagnosed with the widow maker – the often-fatal type of heart attack in which one of the three main cardiac arteries is blocked.

He was given statins to thin the blood, and doctors inserted a stent into his coronary artery to widen it.  

“I was told I was lucky I’d been so close to the hospital and that if I’d waited any longer, it would have been dangerous,” he recalls.

But what had caused this? He was young and didn’t smoke or drink.  

A genetic condition that most don’t know they have

The diagnosis was elevated levels of lipoprotein (a), a small, sticky particle that carries fats like cholesterol through the blood stream. They can accumulate and stick to the artery walls, causing a heart attack, heart-valve narrowing and stroke.

The condition is almost entirely genetic.

About 20 per cent of Canadians have high levels of Lp(a), or “little a”, as it’s known, but most people don’t know it, says Dr. Liam Brunham, Medical Lead of the Healthy Heart Program Prevention Clinic at St. Paul’s Hospital.

“Little a” not screened in standard cholesterol tests

“It’s common,” adds the doctor. “But it is not included in a standard cholesterol test. Fewer than 10 per cent of Canadians know their Lp(a) levels or whether they’re elevated.”

Ali is one of them. When he visited his doctor before the attack, no blood tests were done. He was told he was too young for heart disease.  

First Lp(a) database in Canada

Researchers at St. Paul’s Hospital want to change that and help people know if they have high Lp(a) or may be at risk of developing it.

Physicians and researchers at the Healthy Heart Program are forming a registry to help identify more patients with elevated Lp(a), learn more about it, and connect patients to the right treatments.

The database will be the first in the country for this specific disorder.

The registry will launch in April at St. Paul’s and at Vancouver General Hospital, with plans to expand to Quebec and Ontario in the first year as well as other sites in the province.

Physicians involved in the registry will enter their patients’ names into it. Family members of those with Lp(a) will be tested too.

Once Ali learned his condition was genetic and that his children could be at risk, all four were tested. It turned out that two of his three daughters have high Lp(a). One of them had higher levels of Lp(a) than he had before his attack. She is taking statins to keep them in the healthy range. His son had normal levels.

“Knowing about high Lp(a) is important as it allows doctors to counsel a patient on risk and how to mitigate it,” says Dr. Brunham.

Dr. Liam Brunham, Medical Lead, St. Paul’s Healthy Heart Program Prevention Clinic

High Lp(a) is treated with drugs that lower levels of low-density lipoprotein (known as “bad cholesterol”) like statins and other drugs. These drugs are effective at reducing the risk of heart disease in patients with high Lp(a).

Drugs that specifically lower Lp(a) levels are currently being tested in clinical trials, and Ali is a participant in one such program. It is hoped that these new drugs will be even more effective at lowering risk of heart disease in patients like him.

One of the goals of the registry is to identify patients with elevated Lp(a) who may benefit from new therapies that become available. 

While Lp(a) is genetic, Ali decided to modify his lifestyle, nonetheless. He became a vegan for a time and has eliminated red meat from his diet. His levels of the lipoprotein, which he gets tested at the Healthy Heart program, are better than ever.

He does confess to missing a good steak dinner every once in awhile, though.

March 24 is Lipoprotein (a) Awareness Day.

Story by Ann Gibbon, Providence Health Care