Guide to Good Health: St. Clair Health Offers Hope for Patients with Cardiac Amyloidosis

Medical research is offering hope for a disease that was once thought to be hopeless, and St. Clair Health cardiologist Christopher Pray, M.D., FACC, is excited about the prospects.

“Cardiac amyloidosis is a rapidly progressing rare disease that was once thought to be a death sentence,” said Dr. Pray, who in addition to being Director of Non-Invasive Cardiology, Cardiac Imaging and Heart Failure Services at St. Clair Health has consulted and presented on the condition all over the country. “That has changed.”

In amyloidosis, Dr. Pray explains, certain proteins that are formed in the liver mutate and fragment. These fragments, known as amyloid fibrils, build up in the body – particularly in and around the heart – which then causes congestive heart failure. Because there is no way to eliminate the fragments once they’ve built up in the body, that’s why it was considered a death sentence.

Although rare, amyloidosis became a bit more familiar in Pennsylvania, particularly western Pennsylvania, when Pittsburgh Mayor Richard Caligiuri, Erie Mayor Louis Tullio, and Pennsylvania Governor Robert Casey all died from the condition between the late 1980s and mid-1990s. And unfortunately, the incidence of amyloidosis is increasing.

“We are actually detecting it in more patients than previously. We seem to have a really large number of patients with this condition,” said Dr. Pray, before adding some great news. “Thanks to advancements in the treatment of this disease, we can manage it as we would any other chronic condition.”

Before it can be treated, the first challenge is diagnosing it. The symptoms are similar to what is found in other forms of heart disease, as well as other conditions. Fatigue, shortness of breath, swelling of the limbs, neuropathy, and unexplained weight loss are just some of the possible symptoms. Even carpal tunnel syndrome and spinal stenosis could be signs of amyloidosis.

“There isn’t one exact sign, but more of a constellation of findings that point to amyloidosis,” said Dr. Pray. “The earlier that this can be diagnosed the better it is for the patients.”

One of the advances that has enabled more accurate diagnoses is the sophistication of cardiac imaging. Dr. Pray says that both nuclear imaging and MRI imaging can detect amyloidosis. Prior to the advances in cardiac imaging, one of the standard ways to diagnose amyloidosis was by taking a biopsy from the patient’s heart.

“We are needing to do fewer and fewer biopsies,” said Dr. Pray. “Thanks to imaging, we are moving toward less invasive procedures.”

Most important, once amyloidosis has been diagnosed in a patient, there are treatments available that didn’t exist a decade ago. There are multiple drug therapies that can prevent the underlying disease process from occurring.

“There are four types of treatment,” Dr. Pray explained. “There are drugs that prevent fragmentation of the proteins, drugs that can turn off the gene that produces the proteins in the liver, and drugs that will scarf up the proteins that are produced. There are also promising advances in CRISPR technology, where the genes that produce the proteins can be modified.”

Although there is currently nothing available that might reverse any damage that has already occurred, once the drug therapy has begun, the disease can be managed in the same manner as anyone else who experiences the beginnings of congestive heart failure would be prescribed: regular exercise in the cardiac rehabilitation gym, a low fat and low sodium diet, and no smoking.

“It’s really rewarding to be on my side of it and see the advances that have been made in such a short time,” said Dr. Pray. “It’s a real testament to the amount of research that has been done on this disease. I’m really proud of the work that we’ve done in western Pennsylvania to recognize amyloidosis and treat it well. Our patients can reap the benefits.”

Dr. Christopher Pray is part of St. Clair Medical Group Cardiology, formerly known as South Hills Cardiology Associates. Each cardiologist has their own area of expertise to offer patients, but they are all driven by a common goal – to provide the best cardiovascular care to their patients and the community.

Dr. Pray sees patients at the following location(s):

1000 Bower Hill Road
Suite 7400
Pittsburgh, PA 15243
(412) 942-7780

3928 Washington Road
Suite 270
McMurray, PA 15317
(412) 942-7780