Variation in Quality of Life Scores in Patients With Congenital Heart Disease

On a scale of 0 to 100, the median quality of life score on the linear analog scale was 80 in patients with congenital heart disease.

Variation in quality of life (QOL) in adults with congenital heart disease was related to patient characteristics as opposed to country-specific characteristics, according to findings from a large, international study published in the Journal of the American College of Cardiology.

Research on QOL in congenital heart disease has greatly increased over the last few decades, but according to the authors of the present study, most publications had “conceptual and methodological deficits,” which have yielded inconsistent results.

APPROACH-IS (Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease – International Study) was conducted in 15 countries, including Argentina, Australia, Belgium, Canada, France, India, Italy, Japan, Malta, Norway, Sweden, Switzerland, Taiwan, the Netherlands, and the United States.

Patients included had to have a diagnosis of congenital heart disease defined as “a structural abnormality of the heart or intrathoracic great vessels present at birth and of actual or potential functional significance,” be 18 years of age or older, be diagnosed before adolescence, participate in continued follow-up at a congenital heart disease center or included in a registry, and finally, have physical, cognitive, and language capabilities required to complete self-report questionnaires.

Of the 4028 adults enrolled, 53% were women (median age: 32 years), many patients worked part or full time, were married or living with a partner, and had no children. Nearly 50% had congenital heart disease of moderate complexity and 54% reported class I New York Heart Association (NYHA; asymptomatic).

Researchers reported variability in the QOL scores. On a scale of 0 to 100, the median QOL score on the linear analog scale (LAS) was 80 (interquartile range [IQR]: 70 to 90). More than 90% of patients reported a score of >50, 25.8% reported a score between 71 and 80, 27.3% between 81 and 90, and 17.4% between 91 and 100. On a scale of 5 to 35, the median QOL score on the Satisfaction With Life Scale (SWLS) was 27.

Meanwhile, in general linear mixed model (GLMM) multivariable analyses, older age; job seeking, being unemployed, or disabled; never having been married; and a higher HYHA functional class were associated with worsened QOL. The GLMM explained 21.5% of the QOL variation.

By country, Australia had the highest QOL estimate (82.1) and Japan had the lowest (71.6). Australia, Switzerland, the United States, and Malta all had an estimate of ≥80. Despite relatively good QOL scores, nearly 1 in 10 patients had ≤50 on the LAS. “Given the association between PROs [patient-reported outcomes], such as QOL, and important medical outcomes, it is of paramount importance that health care professionals identify patients with poor QOL and target interventions accordingly,” researchers wrote.

“More research should be aimed at defining features of health care delivery systems in various nations that influence QOL among patients with adult congenital heart disease,” they concluded.


Apers S, Kovacs AH, Luyckx K, et al; on behalf of the APPROACH-IS consortium and ISACHD. Quality of life of adults with congenital heart disease in 15 countries. Evaluating country-specific characteristics.  J Am Coll Cardiol. 2016;67(19):2237-2245. doi: 10.1016/j.jacc.2016.03.477.