Higher Mortality Trend Among CF Patients With CVD

BOSTON — With the remarkable advances made in therapy over the past decade, many patients with cystic fibrosis (CF) can expect to survive into their 50s and even well beyond. But as patients with CF live longer, they are increasingly likely to develop complications such as cardiovascular diseases (CVDs) that beset many older adults. And as evidence from a new study suggests, there is an increasing need for cardiovascular screening and specialized cardiac care for these patients.

Among more than 83,000 patients with CF hospitalized for any reason from 2016 through 2021, less than 1% of patients had a cardiac cause listed, but in unadjusted analyses, these patients had a more than twofold risk for inhospital death than those with CF hospitalized for other causes, reported Adnan Bhat, MD, assistant professor of hospital medicine at the University of Florida, Gainesville, Florida.

Although the excess mortality was no longer statistically significant in analyses adjusted for potential confounding factors, the data highlight a trend that requires further exploration, he said during an oral abstract session at the American College of Chest Physicians (CHEST) 2024 Annual Meeting.

“There’s a trend for people with cystic fibrosis admitted for cardiac causes to have a higher inhospital mortality and increased rate of discharge to nursing facilities, especially for patients admitted for heart failure. The clinical implication is that there is an increased need to start screening for cardiovascular risk factors,” he said.

National Database Sample

Bhat and colleagues conducted a retrospective study using the National Inpatient Sample database to identify all hospitalizations among patients with CF in the United States from 2016 through 2021.

They included all hospitalizations with a principal diagnosis code for atrial fibrillation, heart failure, or myocardial infarction.

Of 83,250 total hospitalizations during the study period, 415 (0.5%) were for primary cardiac causes. These included 170 hospitalizations for atrial fibrillation, 95 for heart failure, and 150 for myocardial infarction.

Patients hospitalized for cardiac causes had a higher mean age (59.5 vs 34.5 years) and more comorbidities than patients hospitalized for other causes. These comorbidities included hyperlipidemia, chronic kidney disease, obesity, and a family history of coronary artery disease.

In all, 5% of patients hospitalized for cardiac cause died in hospital, compared with 2% of patients hospitalized for other reasons (P = .044).

However, in logistic regression analyses adjusting for age, sex, and race, this difference was no longer significant.

Similarly, an unadjusted analysis showed that patients with cardiac disease were twice as likely to be discharged to a nursing facility (8% vs 4%, respectively), but this difference too disappeared in adjusted analyses.

The risk for inhospital mortality appeared to be highest among those patients with a primary diagnosis of heart failure, who had an 11% rate of inhospital death, compared with 2% among patients with CF hospitalized for other causes.

The total number of deaths was too small, however, to allow for regression analysis, Bhat said.

Nonetheless, taken together, the data indicate a trend toward increased mortality from cardiovascular causes among older patients with CF, as well as the need for further research into the cardiovascular health of these patients, Bhat concluded.

Better Nutrition, Higher Risk

In an interview with Medscape Medical News, Yuqing A. Gao, MD, from the Santa Monica Pulmonary Sleep Clinic, Santa Monica, California, who was not involved in the study, commented that with the advent of elexacaftor/tezacaftor/ivacaftor modulator therapy, patients with CF tend to have increases in body mass index and improved nutritional intake and absorption, which in turn could increase hyperlipidemia and other factors that might in turn contribute to increased CVD risk.

“It’s really an interesting area of research, and there’s hope that this will bring more focus on how to better screen [for CVD risk] because that’s something that’s very much not known at this time,” she said.

Gao was co-moderator for the session where Bhat presented the data.

Bhat did not report a study funding source. Bhat and Gao reported no relevant financial relationships.

Neil Osterweil, an award-winning medical journalist, is a long-standing and frequent contributor to Medscape Medical News.