NASHVILLE, Tenn. -- Roughly one in 10 adults with cystic fibrosis also had a diagnosis of heart failure, according to a real-world study of patient medical records reported here.
Among the roughly 15,000 cystic fibrosis patients, acute myocardial infarction (MI) and atrial fibrillation (Afib) were the other most commonly identified cardiac disorders, each present in about one in 20 patients in the analysis presented by Andres Cordova Sanchez, MD, of SUNY Upstate Medical University in Syracuse, New York.
In an interview with MedPage Today at CHEST 2022, the annual meeting of the American College of Chest Physicians, Cordova Sanchez explained that as patients with cystic fibrosis live longer, more and more are developing chronic conditions associated with aging.
"I think it is very important to have a better understanding of these issues in this patient population, especially heart failure," he said.
Just three decades ago, the average life expectancy of a cystic fibrosis patient was around 30 years, but thanks to highly effective new therapies a baby born today with the progressive, genetic disease can expect to live to age 50.
While the average age of patients in the analysis was 33 years, Cordova Sanchez said that close to a dozen patients were in their 80s.
As neonatal screening for cystic fibrosis has become the norm, treatments have been introduced earlier. Malnutrition from pancreatic insufficiency is also less of an issue than it once was due to enzyme replacement and dietary management. And the introduction of drugs that target the cystic fibrosis transmembrane regulator (CFTR) protein have transformed cystic fibrosis treatment.
As patients have grown older, cardiac sequelae, including pulmonary hypertension, right heart dysfunction, and cardiomyopathies have increasingly been linked to cystic fibrosis.
"Cystic fibrosis might affect the cardiovascular system directly, through CFTR gene dysfunction in the heart and coronary arteries, or indirectly through hypoxia, chronic inflammation, diabetes, and pulmonary hypertension," Cordova Sanchez said.
His team analyzed health information from the electronic medical records from primarily U.S. institutions in the TriNetX database for a period of 6 years (2016 through 2021) to identify adult patients with cystic fibrosis with heart failure, acute MI, and arrhythmias.
A total of 14,958 patients were identified (mean age 33 years, 65% women).
Heart failure was present in 10.4% of the overall population, with a prevalence of around 15% in patients in their early 50s, and 20-25% in older cystic fibrosis patients. Overall, men had a slightly higher prevalence of heart failure (11.8%) than women (9.6%).
Prevalence of acute MI was 4.3%, which increased to around 9% among patients ages 55 to 64.
"It is interesting to contrast this with reports from the general population showing a heart failure prevalence of around 2% and acute myocardial infarction is usually reported to be around 3%," Cordova Sanchez said.
Afib and flutter were the most commonly identified arrhythmias, with an overall prevalence of 4.3%, followed by supraventricular tachycardia, which was reported in 3.6% of the cystic fibrosis cohort. Around 38% of the small number of patients in their mid-to-late 70s had a diagnosis of Afib.
Ventricular tachycardia and ventricular fibrillation were each reported in less than 1% of the overall patient population (0.99% and 0.23%, respectively).
Overall, men had higher incidences and prevalences of all outcomes examined, with the exception of supraventricular tachycardia. The prevalence and incidence of the arrhythmia, respectively, was 3.7% and 3.4% in women, and 3.3% and 2.8% in men.
Cordova Sanchez told MedPage Today that while heart sequelae associated with cystic fibrosis are increasingly being recognized, far more research is needed as patients live longer.
"When I was doing this research I really didn't find much in the literature about this," he said. "We know much more than we did, but there is still a lot we don't know."
Disclosures
Cordova Sanchez reported no relevant relationships.
Primary Source
CHEST
Source Reference: Cordova Sanchez AM, et al "Using real-world data to identify the prevalence of heart disease in patients with cystic fibrosis" CHEST 2022; DOI: 10.1016/j.chest.2022.08.094.