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Health Matters: Providence St. Joseph Hospital | 7 C ardiac amyloidosis (CA) might be a singularly unfamiliar term, unless you are one of the 100,000 in the United States who have been diagnosed with it. Still, it's a confounding condition, and one that specialists at Providence St. Joseph Hospital have been studying with great success. Brian Kolski, MD, an interventional cardiologist and director of the structural heart program at Providence St. Joseph, is one of those specialists. CA is a type of cardiovascular disease in which an overproduction and dumping of abnormal amyloid protein—a misfolded and insoluble protein—is deposited in the heart's valves, atria or ventricles. The deposits can cause thickening of these sections and decrease the heart's function. "Basically," says Dr. Kolski, "it causes pump failure." This disease has been widely misdiagnosed, and is often not properly diagnosed at all until an autopsy occurs. "We started studying CA out of necessity," Dr Kolski says. "People were experiencing heart failure and we didn't have the proper tools. We began clinical trials to discover ways to detect and treat it, and now have actual therapies that can help arrest it and prevent it from getting worse." Dr. Kolski suspects that the current estimate of 1 in 6,000 heart patients having cardiac amyloidosis might be on the low side: "As we start to understand and are better at diagnosing, the number gets higher." He says there are two kinds of CA: a hereditary or genetic type and a "wild" mutation. SCREENING AND ATTENTION ARE KEY "There are indicators, especially in elderly patients, that point to the need for a nuclear imaging scan, " Dr. Kolski explains. "Those indicators include congestive heart failure, neuropathy and weakness in the legs, and carpal tunnel syndrome." Technicians have found that a specialized type of imaging called 99mTc-pyrophosphate (PYP), which was originally developed as a bone scan, is very effective at finding cardiac amyloidosis. Providence St. Joseph is one of the few places actively screening for CA; the PYP imaging is an important test not many others are offering, says Dr. Kolski. "Once we do the screening, we plug patients into a clinical trial that uses a combination of drugs; we also pair the treatment with genetic screening and the patient with a genetic counselor." Drugs can treat both the hereditary and wild types of the disease. And Dr. Kolski's team takes a multidisciplinary approach, which includes a hematologist and a neurologist and possibly "access to a liver transplant, because that's where the proteins are being produced." Dr. Kolski has come to the conclusion that patients who present with heart failure but normal pumping should be evaluated for a PYP scan, "especially when they have neurological conditions such as carpal tunnel and muscle weakness." To learn about our many screenings, call the Heart and Vascular Center at 714-744-8849. Advances in Cardiac Treatment Providence St. Joseph cardiologists are having success finding and treating a heretofore sneaky heart condition. Dr. Brian Kolski

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