Health-Related Quality of Life in Patients With Congenitally Corrected Transposition of the Great Arteries

Study Questions:

Does health-related quality of life in patients with congenitally corrected transposition of the great arteries (CCTGA) differ in patients who have undergone anatomic repair versus those who have a systemic right ventricle?


Thirty-eight patients who underwent anatomic repair and 13 patients who either underwent conventional repair (nonanatomic) or no surgical procedure were asked to complete the PedsQL 4.0 Generic and 3.0 Cardiac Modules. Scores were compared between groups, as well as compared to similar severity patients in the literature.


Enrollment was 81%, with no significant difference between groups. Caregivers reported similar scores in all functional domains for patients in both the anatomic and nonanatomic repair groups. Anatomic repair patients self-reported lower school function (p < 0.02). When the anatomic repair group was compared to other patients in the literature with “moderate cardiovascular disease,” they scored lower overall, with the largest differences in cognition (p = 0.03) and communication (p = 0.06). Prolonged hospital stay and need for a permanent pacemaker were associated with significantly lower quality of life for the anatomic repair group.


The authors concluded that health-related quality of life did not differ significantly between anatomic and nonanatomic patients except for a self-report of lower school functioning in the anatomic repair group. Lower quality of life in the anatomic repair group is associated with prolonged hospitalization and need for a permanent pacemaker.


The optimal surgical management of patients with CCTGA remains debated. There is concern regarding the long-term outcome when the right ventricle remains on the systemic side. This needs to be balanced against the morbidity and mortality associated with anatomic repairs (double switch or Senning/Rastelli), which place the left ventricle on the systemic side. This manuscript attempts to address the core issue, which is quality of life for these patients. Based on a very limited sample over a long period of time, the authors were unable to demonstrate a significant improvement in quality of life for patients who had undergone an anatomic repair, placing the left ventricle on the systemic side. These findings need to be taken into consideration when weighing the options for any individual patient. Further long-term studies to follow ventricular function, along with formal exercise testing for both anatomic and nonanatomic repair patients, will be beneficial to further elucidate the ideal management strategy.

Clinical Topics: Congenital Heart Disease and Pediatric Cardiology, Congenital Heart Disease, CHD and Pediatrics and Quality Improvement

Keywords: Heart Diseases, Morbidity, Quality of Life, Self Report, Cardiovascular Diseases, Transposition of Great Vessels, Ventricular Function, Pacemaker, Artificial, Questionnaires, Health Status, Heart Ventricles, Caregivers

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