Introduction:
Children with complex congenital heart disease (CHD) are at increased risk for neurodevelopmental impairments and reduced quality of life (QOL). Patients (pts) with congenitally corrected transposition of the great arteries (ccTGA) often undergo anatomic repair to make the left ventricle the systemic ventricle, with the goal of long term cardiac benefits. Understanding long-term outcomes and QOL may inform preoperative counseling and treatment strategies for ccTGA.
Methods:
We measured QOL using PedsQL generic and cardiac modules for pts following anatomic repair of ccTGA with Senning/Arterial Switch or Senning/Rastelli operations. Impaired QOL by the PedsQL is defined by total scores <65.4 for parent report and <69.7 for pt report (possible score 0-100). Scores were compared to healthy controls and to pts with other forms of CHD, previously described in existing literature. Risk analysis for QOL impairment was performed using univariate logistic regression.
Results:
Of 88 eligible pts, 56 (64%) returned surveys (29% female, median age 11.8 years). Respondents were more likely to have pre-operative arrhythmias or pacemaker implantation compared to non-respondents. Mean total PedsQL score was 72
+
19 for pt report and 75
+
18 for parent report; 22 of 56 (39%) pts had impaired QOL scores, compared to 20% impaired scores in pts with any type of CHD, previously described. Mean physical functioning score was 72
+
22 for pt report and 76
+
21 for parent report. Mean psychosocial summary score was 73
+
18 and 75
+
18 for pt and parent report, respectively. Communication was the lowest scored subscale of the Cardiac module by pt (63
+
26) and parent (66
+
31) report. The treatment II subscale addressing medication compliance was the highest scored subscale with scores of 90
+
8 for pts and 91
+
15 for parent, respectively. Longer cardiopulmonary bypass time (p = 0.01), surgical or catheter-based reintervention following hospital discharge (p = 0.02) and heart failure symptoms (p = 0.04) were associated with impaired QOL.
Conclusions:
After anatomic repair of ccTGA, pts are at risk of impaired QOL compared to healthy controls and to pts with a broad range of CHD. Indicators of more complex surgical and post-operative courses were associated with impaired QOL.