Specialist care reduces heart failure-related deaths in adults with congenital heart disease

Feb. 20, 2024

In the United States, more than 2.5 million people are living with a diagnosis of congenital heart disease (CHD). Heart failure (HF) is the leading cause of death in this rapidly growing community.

Surprisingly little is known about outcomes of adult congenital heart disease (ACHD) following heart failure hospitalization. A study published last December in the Journal of the American Heart Association compared the outcomes of HF versus non-HF hospitalizations in patients with ACHD.

"A major problem for clinicians caring for adults with CHD is identifying which patients are at highest risk of HF and its complications, including premature death," says Luke J. Burchill, M.B.B.S., Ph.D., a cardiologist at Mayo Clinic in Rochester, Minnesota. Dr. Burchill is also the immediate past president of the International Society for Adult Congenital Heart Disease and lead author of the study. "Also, traditional heart failure programs were designed for older adults with heart failure onset at a later age — commonly after age 50 or 60. A key question we sought to address with this research is, what are the healthcare needs of younger adults with HF due to CHD?"

Young adults living with CHD in the U.S. are at increased risk of death and cardiovascular complications following HF hospitalization. The mortality risk is especially important for patients under age 45. Younger patients with ACHD also have high health resource needs following HF hospitalization and require more medical or supportive care.

The retrospective cohort analysis used deidentified administrative claims data of patients ages 18 to 70 years, hospitalized between January 1, 2010, and December 31, 2020, with a primary or secondary diagnosis of ACHD. Patients of diverse ethnic, racial and geographic backgrounds in the U.S. were represented. Patients with ACHD hospitalized with and without HF were characterized to determine the predictors of 90-day and one-year mortality and quantify the risk of mortality, major adverse cardiac and cerebrovascular events (MACCE), and health resource use.

Of 26,454 unique ACHD admissions, 5,826 (22%) were ACHDHF+ and 20,628 (78%) were ACHDHF-. The ACHDHF+ hospitalizations increased from 6.6% in 2010 to 14.0% in 2020. Patients with ACHDHF+ had a higher risk of mortality, MACCE and health resource use, including rehospitalization and increased post-acute care service.

The study found that patients in cardiology care within 30 days of hospital admission had a significantly lower mortality risk at 90 days and one year. The research also shows that patients with ACHD can no longer be regarded as an emerging HF subgroup. There is an urgent need for safe and effective care pathways tailored to these patients.

Study highlights:

  • Hospitalizations for patients with ACHDHF increased 50% in the last decade.
  • Patients with ACHDHF have an 86% higher risk of dying in the years following hospitalization.
  • Cardiology clinic care within 30 days of hospitalization significantly lowers this risk.

"It's not just that the mortality risk is high — 40% of patients with ACHD will experience a major complication following HF hospitalization, with the risk of recurrent HF, atrial fibrillation, stroke and myocardial infarction being especially high following HF hospitalization," says Dr. Burchill. "Patients with ACHD need to be followed closely after HF hospitalization to prevent these complications."

The goal is for the patient with ACHD to feel better and live longer by focusing on heart function rather than heart failure, says Dr. Burchill. There are options that include new medications and replacing heart valves without opening the chest.

The research was funded by the Mayo Clinic Robert D. and Patricia E. Kern Center for the Science of Health Care Delivery and Cardiovascular Medicine at Mayo Clinic.

For more information

Agasthi P, et al. Mortality and morbidity of heart failure hospitalization in adult patients with congenital heart disease. Journal of the American Heart Association. 2023;12:e030649.

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