Quality of life and exercise performance in unoperated children with anomalous aortic origin of a coronary artery from the opposite sinus of valsalva.
BACKGROUND
Anomalous aortic origin of a coronary artery is a congenital cardiac condition that can be associated with increased risk of sudden death. To date, quality of life and exercise performance have not been evaluated in patients with this condition who do not undergo surgical repair.


METHODS
We carried out a cross-sectional analysis of patients with unoperated anomalous aortic origin of a coronary artery at our institution from 1 January, 2000 to 31 January, 2016. We prospectively assessed quality of life using standardised questionnaires. Medical records were reviewed for clinical and exercise stress test data. Statistical analyses were performed using Student's t-tests and Spearman's correlation coefficients.


RESULTS
In total, 56 families completed the questionnaires. The average age at enrolment was 14.7±6 years. The majority were male (n=44, 78.6%) and had interarterial anomalous right coronary artery (n=38, 67.9%). Patients had normal quality of life on the PedsQL 4.0 Report, Child Health Questionnaire Child Form 87, and SF-36v2. Their parents had normal quality of life on the PedsQL 4.0 Parent Report, but parents of exercise-restricted patients had decreased Physical Functioning, General Health Perception, Emotional Impact on Parent, and Physical Summary scores (p<0.001-0.048) on the Child Health Questionnaire Parent Form 50.


CONCLUSIONS
Patients with unoperated anomalous aortic origin of a coronary artery appear to have normal quality of life, but parents of exercise-restricted patients have decreased general health and emotional and physical quality of life scores. Improved counselling of families may be beneficial in this group. Future studies with more patients should evaluate quality of life and exercise performance over time.
1The Children's Hospital of Philadelphia,Division of Cardiology,Philadelphia,Pennsylvania,United States of America.
Alan C
AC
Sing
2Perelman School of Medicine at the University of Pennsylvania,Philadelphia,Pennsylvania,United States of America.
Stephen
S
Tsaur
1The Children's Hospital of Philadelphia,Division of Cardiology,Philadelphia,Pennsylvania,United States of America.
Stephen M
SM
Paridon
1The Children's Hospital of Philadelphia,Division of Cardiology,Philadelphia,Pennsylvania,United States of America.
Julie A
JA
Brothers
D000293:Adolescent; D002648:Child; D003330:Coronary Vessel Anomalies / Q000503:physiopathology* / Q000601:surgery; D003430:Cross-Sectional Studies; D016757:Death, Sudden, Cardiac / Q000209:etiology; D015444:Exercise; D005080:Exercise Test; D005260:Female; D006801:Humans; D008297:Male; D015143:Philadelphia; D011788:Quality of Life; D012189:Retrospective Studies; D012720:Severity of Illness Index; D012850:Sinus of Valsalva / Q000002:abnormalities*; D011795:Surveys and Questionnaires; D055815:Young Adult
anomalous aortic origin of a coronary artery; exercise performance; quality of life