Patients with transthyretin amyloid cardiomyopathy (ATTR-CM) and aortic stenosis (AS) experienced worse clinical outcomes compared with patients who had only AS, according to a study recently published in the Journal of the American Heart Association.
ATTR-CM and AS — the narrowing of a major artery of the heart — are distinct cardiac diseases that can sometimes overlap. Physicians increasingly recognize that the two disorders often coexist, with significant clinical implications for long-term outcomes.
What is ATTR-CM?
Transthyretin amyloidosis cardiomyopathy (ATTR-CM) is a rare progressive disease of the heart muscle that leads to congestive heart failure. It occurs when the transthyretin protein produced by the liver is unstable. Symptoms include fatigue; shortness of breath; irregular heart rate or palpitations; swelling of the legs, ankles and stomach; brain fog; wheezing; and dizziness. It often goes underdiagnosed because of a lack of awareness and knowledge of the disease. There is currently no cure for ATTR-CM.
“These findings further highlight the need to identify [ATTR-CM] earlier in patients with AS to shorten their pathway to disease‐modifying treatment and potentially improve clinical outcomes,” study authors said.
Read more about ATTR-CM testing and diagnosis
The study sought to assess the clinical characteristics of patients who have AS, ATTR-CM or both. Researchers accessed U.S. administrative health claims data from the Optum Clinformatics Data Mart database between October 2015 and September 2021. The database is derived from health plan claims that encompass approximately 17 million to 19 million people.
A total of 355,430 individuals met the eligibility criteria. They were divided into three categories: AS only (345,738 patients; 97.3%), ATTR-CM only (8,453 patients; 2.4%) and combined AS and ATTR-CM (1,239 patients; 0.3%).
The research team compared outcomes among the three categories of patients. Patients with a combination of AS and ATTR-CM had a significantly higher risk of death and heart failure hospitalization compared with patients with AS only.
Two-year mortality rates and two-year heart failure hospitalization rates were best in patients with only AS and the worst in patients with combined ATTR-CM and AS.
Among the patients who received aortic valve replacement, patients with combined AS and ATTR-CM were at a significantly higher risk of death compared with patients with AS alone. In addition, the two-year risk of death and stroke or transient ischemic attack was higher among patients with combined AS and ATTR-CM compared with those with AS alone.
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