Congenital Heart Disease Patients Face Early Dementia Risk

The association may reflect late neurologic consequences

Children with congenital heart disease surviving to adulthood are more likely to develop dementia and to do so at a younger age than the general population, a large population-based study found.

Overall, the Danish congenital heart disease population was at 61% elevated risk of dementia compared with peers without congenital defects (95% CI 1.29-2.02), researchers led by Carina Bagge, BSc, of Aarhus University Hospital in Denmark, reported online in Circulation.

They were particularly likely to get early-onset dementia (HR 2.59 before age 65, 95% CI 1.76-3.81) but more likely to get dementia later on in life as well (HR 1.32, 95% CI 1.00-1.75).

Dementia rates were markedly higher for subgroups as well:

  • Congenital heart disease (CHD) without extra-cardiac defects: HR 1.38, 95% CI 1.08-1.76
  • Adults with mild-to-moderate defects: HR 1.50, 95% CI 1.14-1.97
  • Adults with severe defects: HR 1.96, 95% CI 1.15-3.34

“Our findings extend the knowledge of long-term neurologic impairment and mental health functional morbidities in the CHD population,” they wrote. “While previous studies have reported elevated risks of adverse neurodevelopmental outcomes among CHD individuals, including increased occurrence of depression, autism, and epilepsy compared to the general population, our study examined an older adult population to determine the later-life consequences of this neurologic outcome.”

“While the underlying pathophysiologic mechanisms are not completely understood, the potential etiologic factors appear multifactorial and consistent with previous finding within the neurodevelopmental scientific literature,” the investigators said.

Their Danish registry-based study included all adults with congenital heart disease diagnosed from 1963 through 2012 and alive at 30 years of age (n=10,632). The most common conditions were a atrial septal defect (26%) and ventricular septal defect (22%).

Those survivors were matched 1:10 to non-congenital heart disease peers from the Danish Civil Registration System.

The results of the study didn’t change when excluding all individuals with diagnosed mild cognitive impairments and amnestic syndromes, nor did the reclassification of unspecified dementias as Alzheimer’s disease alter the findings, according to Bagge and colleagues.

However, there was the possibility of congenital heart disease misclassification and survival bias in the records they relied upon.

“The CHD population in our study represented adults who survived at least to age 30. These survivors, with an increased risk of dementia, reflect a healthier CHD cohort, and our analyses may underestimate the true association between CHD and dementia,” they also acknowledged.

Cancer Risk Rises With Cumulative CV-Procedure Radiation in Adults With Congenital Heart Disease

Repeated exposure to radiation from imaging for cardiovascular procedures in adults with congenital heart disease is related, in dose-response fashion, to increased lifetime risk of cancer, a longitudinal cohort-based study suggests[1].

The cumulative incidence of cancer in >24,000 such patients, who were at least aged 25 in 1995, was 15.3%; cancer was diagnosed at a median age of 55. The risk over 15 years was 8.5% for those with a history of at least six cardiovascular procedures, during which they were exposed to low-dose ionizing radiation (LDIR), and only 3.3% for those who had undergone up to one such procedure (P<0.0001).

The findings in a population that undergoes repeated cardiovascular imaging, often starting at a young age, and which is aging in growing numbers, support radiation levels “as low as reasonably achievable” for any imaging procedure, authors of the study, led by Dr Sarah Cohen (McGill University, Montreal, QC), conclude in their report published December 21, 2017 in Circulation.

They also argue for “the selection of non–LDIR-related imaging whenever medically appropriate” for patients with congenital heart disease.

The analysis covered 250,791 person-years of follow-up in 24,833 adult patients with congenital heart disease who were aged 18 to 64 from 1995 to 2009 without a prior cancer diagnosis. Lifetime LDIR exposure from procedures was imputed based on expected dosages by procedure according to the literature, caution the authors.

A case-control analysis matched each patient who developed cancer with four random patients from the cohort without cancer based on sex, congenital heart disease severity, birth year, and age at cancer diagnosis. In that analysis, 5.5% of cases and 2.8% of controls were among those with a history of at least six cardiovascular procedures. And 74.3% of cases and 83.7% of controls had a history of no more than one procedure (P<0.0001), the authors write.

In multivariate analysis of the entire cohort, cumulative LDIR exposure independently predicted a cancer diagnosis at a per-procedure odds ratio (OR) of 1.08 (95% CI 1.04–1.13). The OR was 1.10 (95% CI 1.05–1.16) after researchers excluded anyone diagnosed with a smoking-related cancer.

Covariates in the analysis included age, sex, year of birth, congenital heart disease severity, hypertension, diabetes, hypercholesterolemia, obesity, heart failure, atrial arrhythmia, coronary artery disease, kidney disease, peripheral atherosclerosis, pulmonary hypertension, history of stroke, history of infective endocarditis, and surgical history.

Adjusted ORs by degree of LDIR exposure, compared to the lowest-exposure group with no more than one cardiovascular procedure, were

  • 1.39 (95% CI 1.06–1.82) for two or three procedures.
  • 1.38 (95% CI 0.90–2.12) for four or five procedures.
  • 2.37 (95% CI 1.47–3.84) for six and more procedures.

In women, the most frequently diagnosed malignancies were breast cancer in 34.5%, respiratory cancers in 14.2%, genitourinary cancers in 13.3%, and gastrointestinal cancers in 11.5%.

The most frequent in men were genitourinary cancers in 30.8%, gastrointestinal cancers in 23.2%, hematologic cancers in 19.0%, and respiratory cancers in 14.8%.

Post-traumatic Stress Disorder Seen in Many Adults Living with Congenital Heart Disease

CHOP Study May Reveal Unmet Medical Needs in a Growing Patient Group


Adults living with congenital heart disease (CHD) may have a significantly higher risk of post-traumatic stress disorder (PTSD) than people in the general population.

A single-center study from The Children’s Hospital of Philadelphia (CHOP) found that as many as 1 in 5 adult patients had PTSD symptoms, with about 1 in 10 patients having symptoms directly related to their heart condition. The researchers suggest that clinicians and caregivers need to be aware of possible PTSD symptoms, such as anxiety and depression, in their patients.

“Although the life expectancy of adults living with CHD has improved, ongoing care may include multiple surgeries and procedures,” said the study’s senior author, Yuli Kim, MD, a cardiologist at CHOP. “These patients remain at risk for both cardiac and non-cardiac effects of their chronic condition, and face unique life stressors that may place them at elevated risk for psychological stress.”

Kim is the director of the Philadelphia Adult Congenital Heart Center, a joint project of CHOP and the Hospital of the University of Pennsylvania. Her research team’s study appeared in the March issue of the American Journal of Cardiology. It was the first analysis of PTSD in an adult CHD population.

Due to surgical and medical advances, there are now more American adults living with congenital heart defects than the annual number of children being born with them, even though heart defects are the most common birth defect in the U.S.

The researchers enrolled 134 patients with congenital heart defects and used two validated mental health scales with questions related to anxiety, depression and PTSD. Of 134 patients who completed one scale, 27 (21 percent) met criteria for global PTSD symptoms. Of the 127 patients who completed another scale, 14 patients (11 percent) had PTSD symptoms specifically related to their CHD or treatment.

The high prevalence of PTSD in this patient cohort — 11 to 21 percent — is several times higher than the 3.5 percent rate observed in the general population. The authors noted that the prevalence is comparable to that found in children with CHD and in adults with acquired heart disease.

The researchers also found two factors most strongly linked to PTSD in their patients: elevated depressive symptoms and the patient’s most recent cardiac surgery. Patients who had undergone cardiac surgery at an earlier year were more likely to have PTSD. This finding may reflect recent medical and surgical advances that lessen traumatic impacts, or alternatively, a “residual stress” explanation — that traumatic stress produces chronic, lasting effects.

The study team also noted that non-medical traumatic events may have contributed to PTSD in some patients. In addition, said the authors, the self-report measurements used in the study may not be as accurate as a clinical interview.

Overall, the new study may reveal important unmet needs in a growing population of patients. “The high prevalence of PTSD detected in these adult CHD patients has important clinical implications,” said corresponding author Lisa X. Deng, of CHOP’s Division of Cardiology. She noted that less than half of the study patients who showed PTSD symptoms were being treated for PTSD, and added that, “We need to conduct more research to identify measures along the lifespan to support our patients and ensure that they have a good quality of life.”