Please review Diagnostic Algorithms for Cardiac Amyloidosis, Indiana University School of Medicine. Accessed 8-22-2025.
Today, I review, link to, and excerpt from the New York Times article on cardiac amyloidosis, Once a Death Sentence, This Heart Condition Is Finally Treatable. Aug. 4, 2025. Simar Bajaj.
All that follows is from the above resource.
Over the past 50 years, heart failure has become one of the fastest-growing cardiac killers. It was long considered a disease of aging, caused by gradual yet unavoidable changes to the heart. But doctors are now discovering that about 15 percent of cases are caused by a rogue protein called amyloid, perhaps best known for its role in Alzheimer’s. While one type tangles neurons in the brain, others infiltrate the heart, making the muscle stiff and less able to pump blood.
Until recently, cardiac amyloidosis was a death sentence, but the advent of new medications, including two approved by the Food and Drug Administration in the past year, has made this disease increasingly manageable. “We shouldn’t just be fatalistic — ‘you’re tired, you’re short of breath, you’re just old,’” said Dr. Michelle Kittleson, a cardiologist at Cedars-Sinai Medical Center. “There is a lot of hope for people with heart failure.”
Diagnoses on the Rise
For decades, cardiac amyloidosis was cast aside as a rare disease. Early signs include carpal tunnel syndrome, a narrowing of the spine and a rupture of the biceps tendon, as amyloid plaques accumulate across the body. But with little awareness of the condition, it was difficult for doctors to connect the dots.
And there was little incentive to do so, Dr. Kittleson said, because without any treatments for cardiac amyloidosis, they could only stand by and watch patients succumb to the disease.
To get diagnosed, then, was like stepping into the darkness, said Ozzie Giglio, 64, who learned he had cardiac amyloidosis in 2016. He recalled his doctor saying, “I want to get you on the heart transplant list right away. That’s the only way to save your life.”
But as researchers developed easier ways to spot cardiac amyloidosis, more patients were diagnosed, said Dr. Mathew Maurer, a cardiologist at Columbia University Irving Medical Center. Instead of needing to biopsy the heart, doctors can now use a scan that lights up amyloid plaques, and test patients’ blood and urine for the abnormal protein, he added.
However, the turning point for cardiac amyloidosis was the development of effective treatments. With doctors now able to actually help patients, there was an “explosion in recognition,” Dr. Kittleson said.
Experts believe cardiac amyloidosis is most common among men, adults 75 or older and Black people. But nobody knows for sure, since many patients aren’t tested. “I can’t give you a prevalence estimate, but clearly, we’re at the tip of the iceberg in older populations,” Dr. Kittleson said.
The New Drugs Are Life Saving
What makes cardiac amyloidosis so deadly is that the abnormal protein gets between the fibers of the heart, stiffening the muscle and impairing the heart’s rhythm, said Dr. Pablo Quintero Pinzon, a cardiologist at Beth Israel Deaconess Medical Center. Some forms are hereditary; others occur spontaneously for reasons experts don’t yet understand.
There are two types of misfolded proteins that usually cause cardiac amyloidosis, but the new drugs target only one of them, transthyretin, either silencing its production or stabilizing the molecule so that it doesn’t infiltrate the heart, Dr. Quintero said.
Trials published in The New England Journal of Medicine — and funded by pharmaceutical companies — showed that these new drugs (tafamidis, acoramidis and vutrisiran) preserved quality of life and reduced deaths by 25 to 35 percent compared to placebo. In the most recent trial, patients on vutrisiran lived nearly as long as the general population.
“We might be living in an era where you will die with cardiac amyloidosis as opposed to from it,” Dr. Kittleson said.
The Journal of American College of Cardiology dedicated its latest issue to cardiac amyloidosis on Monday. New research shows how vutrisiran improves some measures of heart relaxation and muscle damage, while staving off further declines in heart contraction and wall stress.
With the current diagnostic tools, there aren’t good ways to know if patients with cardiac amyloidosis are responding to treatment. But these new findings suggest that doctors can use echocardiogram scans and blood-based biomarkers to monitor patients’ progress and potentially predict survival, said Dr. Quintero, who was not involved in this research.
Several Questions Remain
For all their benefits, these drugs don’t cure the disease; they just “freeze” patients at whatever stage of heart failure they’re in, Dr. Kittleson said. The ability of the medications to reduce the risk of death also drops precipitously as time goes on.
That’s why early diagnosis is so important, and why researchers are eagerly awaiting results for a new drug designed to remove amyloid from the heart even after it’s built up.
There’s also still a question of how many patients will be able to afford the drugs. They are incredibly expensive, costing $250,000 to $500,000 per year. Medicare covers all three medications, so most patients won’t pay more than $2,000 out of pocket each year. But that’s still a heavy burden on the broader health care system, Dr. Maurer said.
