Health-related quality of life in patients with congenitally corrected transposition of the great arteries.

OBJECTIVE: The study's objectives were to survey the quality of life in patients with congenitally corrected transposition of the great arteries and to compare the responses of those who have undergone anatomic repair with those who have a systemic right ventricle.
METHODS: Thirty-eight patients who underwent anatomic repair and 13 patients after either conventional repair or no surgical procedure were enrolled. Subjects completed the PedsQL 4.0 Generic and 3.0 Cardiac Modules. Scores were also compared with those of patients from the literature with at least moderately severe cardiovascular disease. Mean differences between groups were compared, and the association between clinical variables and score in the anatomic repair subgroup was measured.
RESULTS: Caregivers of patients in the anatomic repair group reported similar scores compared with the non-anatomic repair group in all functional domains The anatomic repair group self-reported lower school function (63 vs 81, P = .02). On the Cardiac Module, patients in the anatomic repair group self-reported fewer problems related to residual heart disease (75 vs 63), appearance (81 vs 68), and treatment anxiety (74 vs 59), although the differences were not significant. Compared with patients with other heart disease, the anatomic repair group scored lower, with the largest differences in cognition and communication. Prolonged hospital stay and need for a pacemaker were associated with lower quality of life after anatomic repair.
CONCLUSIONS: Patients in the anatomic repair group had similar quality of life compared with patients in the non-anatomic group, except in the domain of school functioning. Prolonged hospital stay and need for a pacemaker after anatomic repair may be risk factors for lower quality of life.