Longitudinal functional health status in young adults with repaired dextro-transposition of the great arteries: A Congenital Heart Surgeons' Society study

J Thorac Cardiovasc Surg. 2020 Feb;159(2):604-614.e3. doi: 10.1016/j.jtcvs.2019.07.072. Epub 2019 Aug 22.


Objectives: Improved survival has led to interest in functional health status (FHS) as patients with dextro-transposition of the great arteries (d-TGA) transition to adulthood. Our primary objectives were (1) evaluation of The Medical Outcomes Study Short Form-36 Health Survey (SF-36) results; (2) comparison with results of patients who completed the Child Health Questionnaire-Child Form 87 (CHQ-CF87) previously, or the PedsQL Generic Core Scales (PedsQL) survey subsequently; and (3) determination of factors associated with SF-36 domains.

Methods: Survivors from the d-TGA Congenital Heart Surgeons' Society cohort (1985-1989) completed the SF-36 (2010) as a measure of FHS (n = 210; age 21-26 years). Patient characteristics, medical history, psychosocial factors, and previous adolescent CHQ-CF87 FHS assessment (2000) were explored for association with SF-36 domains, along with comparison with recent PedsQL data (2017).

Results: Patients scored themselves the same/higher than published normative data in 10 of 10 SF-36 summary scores/domains and similar in 5 of 6 PedsQL summary scores/domains. Factors commonly associated with lower summary scores/domains of the SF-36 were presence of cardiac symptoms, heart condition impacting physical activity/overall health/quality of life, unemployment, and lack of postsecondary education. Less commonly associated factors were lower birth weight, greater total medication number, female sex, shorter procedure-free interval, poor health knowledge, lower family income, younger age at SF-36, living with parents, and being married. These factors accounted for 17% to 47% of the variation in FHS summary scores/domains. FHS was minimally related to d-TGA morphology and repair type.

Conclusions: Patients with d-TGA surviving into adulthood, regardless of morphology or repair type, can primarily expect normal FHS. Addressing the challenges of patients with d-TGA entering adulthood requires consideration of psychosocial factors and clinical management.

Keywords: CHD; database; epidemiology; great vessel anomalies; quality of life; regression analysis; statistics; transposition.

MeSH terms

  • Adult
  • Female
  • Health Status*
  • Humans
  • Longitudinal Studies
  • Male
  • Quality of Life
  • Surveys and Questionnaires
  • Transposition of Great Vessels* / epidemiology
  • Transposition of Great Vessels* / mortality
  • Transposition of Great Vessels* / surgery
  • Young Adult