Self-reported functional health status following interrupted aortic arch repair: A Congenital Heart Surgeons’ Society Study

Anusha Jegatheeswaran, Marshall L. Jacobs, Christopher A. Caldarone, Paul M. Kirshbom, William G. Williams, Eugene H. Blackstone, William M. DeCampli, Kim F. Duncan, Linda M. Lambert, Henry L. Walters, Christo I. Tchervenkov, Brian W. McCrindle

Research output: Contribution to journalArticle

5 Citations (Scopus)

Abstract

Objectives: Improved survival after congenital heart surgery has led to interest in functional health status. We sought to identify factors associated with self-reported functional health status in adolescents and young adults with repaired interrupted aortic arch. Methods: Follow-up of survivors (aged 13-24 years) from a 1987 to 1997 inception cohort of neonates included completion of functional health status questionnaires (Child Health Questionnaire-CF87 [age <18 years, n = 51] or the Short Form [SF]-36 [age ≥18 years, n = 66]) and another about 22q11 deletion syndrome (22q11DS) features (n = 141). Factors associated with functional health status domains were determined using multivariable linear regression analysis. Results: Domain scores of respondents were significantly greater than norms in 2 of 9 Child Health Questionnaire-CF87 and 4 of 10 SF-36 domains and only lower in the physical functioning domain of the SF-36. Factors most commonly associated with lower scores included those suggestive of 22q11DS (low calcium levels, recurrent childhood infections, genetic testing/diagnosis, abnormal facial features, hearing deficits), the presence of self-reported behavioral and mental health problems, and a greater number of procedures. Factors explained between 10% and 70% of domain score variability (R 2 = 0.10-0.70, adj-R 2 = 0.09-0.66). Of note, morphology and repair type had a minor contribution. Conclusions: Morbidities associated with 22q11DS, psychosocial issues, and recurrent medical issues affect functional health status more than initial morphology and repair in this population. Nonetheless, these patients largely perceive themselves as better than their peers. This demonstrates the chronic nature of interrupted aortic arch and suggests the need for strategies to decrease reinterventions and for evaluation of mental health and genetic issues to manage associated deteriorations.

Original languageEnglish (US)
Pages (from-to)1577-1587.e10
JournalJournal of Thoracic and Cardiovascular Surgery
Volume157
Issue number4
DOIs
StatePublished - Apr 2019

Fingerprint

22q11 Deletion Syndrome
Thoracic Aorta
Health Status
Mental Health
Genetic Testing
Hearing
Thoracic Surgery
Survivors
Young Adult
Linear Models
Regression Analysis
Surgeons
Newborn Infant
Calcium
Morbidity
Survival
Surveys and Questionnaires
Infection
Population
Child Health

Keywords

  • CHD
  • CHD interrupted arch
  • congenital heart disease
  • database
  • outcomes
  • quality of life
  • statistics
  • statistics regression analysis

ASJC Scopus subject areas

  • Surgery
  • Pulmonary and Respiratory Medicine
  • Cardiology and Cardiovascular Medicine

Cite this

Jegatheeswaran, A., Jacobs, M. L., Caldarone, C. A., Kirshbom, P. M., Williams, W. G., Blackstone, E. H., ... McCrindle, B. W. (2019). Self-reported functional health status following interrupted aortic arch repair: A Congenital Heart Surgeons’ Society Study. Journal of Thoracic and Cardiovascular Surgery, 157(4), 1577-1587.e10. https://doi.org/10.1016/j.jtcvs.2018.11.152

Self-reported functional health status following interrupted aortic arch repair : A Congenital Heart Surgeons’ Society Study. / Jegatheeswaran, Anusha; Jacobs, Marshall L.; Caldarone, Christopher A.; Kirshbom, Paul M.; Williams, William G.; Blackstone, Eugene H.; DeCampli, William M.; Duncan, Kim F.; Lambert, Linda M.; Walters, Henry L.; Tchervenkov, Christo I.; McCrindle, Brian W.

In: Journal of Thoracic and Cardiovascular Surgery, Vol. 157, No. 4, 04.2019, p. 1577-1587.e10.

Research output: Contribution to journalArticle

Jegatheeswaran, A, Jacobs, ML, Caldarone, CA, Kirshbom, PM, Williams, WG, Blackstone, EH, DeCampli, WM, Duncan, KF, Lambert, LM, Walters, HL, Tchervenkov, CI & McCrindle, BW 2019, 'Self-reported functional health status following interrupted aortic arch repair: A Congenital Heart Surgeons’ Society Study', Journal of Thoracic and Cardiovascular Surgery, vol. 157, no. 4, pp. 1577-1587.e10. https://doi.org/10.1016/j.jtcvs.2018.11.152
Jegatheeswaran, Anusha ; Jacobs, Marshall L. ; Caldarone, Christopher A. ; Kirshbom, Paul M. ; Williams, William G. ; Blackstone, Eugene H. ; DeCampli, William M. ; Duncan, Kim F. ; Lambert, Linda M. ; Walters, Henry L. ; Tchervenkov, Christo I. ; McCrindle, Brian W. / Self-reported functional health status following interrupted aortic arch repair : A Congenital Heart Surgeons’ Society Study. In: Journal of Thoracic and Cardiovascular Surgery. 2019 ; Vol. 157, No. 4. pp. 1577-1587.e10.
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abstract = "Objectives: Improved survival after congenital heart surgery has led to interest in functional health status. We sought to identify factors associated with self-reported functional health status in adolescents and young adults with repaired interrupted aortic arch. Methods: Follow-up of survivors (aged 13-24 years) from a 1987 to 1997 inception cohort of neonates included completion of functional health status questionnaires (Child Health Questionnaire-CF87 [age <18 years, n = 51] or the Short Form [SF]-36 [age ≥18 years, n = 66]) and another about 22q11 deletion syndrome (22q11DS) features (n = 141). Factors associated with functional health status domains were determined using multivariable linear regression analysis. Results: Domain scores of respondents were significantly greater than norms in 2 of 9 Child Health Questionnaire-CF87 and 4 of 10 SF-36 domains and only lower in the physical functioning domain of the SF-36. Factors most commonly associated with lower scores included those suggestive of 22q11DS (low calcium levels, recurrent childhood infections, genetic testing/diagnosis, abnormal facial features, hearing deficits), the presence of self-reported behavioral and mental health problems, and a greater number of procedures. Factors explained between 10{\%} and 70{\%} of domain score variability (R 2 = 0.10-0.70, adj-R 2 = 0.09-0.66). Of note, morphology and repair type had a minor contribution. Conclusions: Morbidities associated with 22q11DS, psychosocial issues, and recurrent medical issues affect functional health status more than initial morphology and repair in this population. Nonetheless, these patients largely perceive themselves as better than their peers. This demonstrates the chronic nature of interrupted aortic arch and suggests the need for strategies to decrease reinterventions and for evaluation of mental health and genetic issues to manage associated deteriorations.",
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AU - Kirshbom, Paul M.

AU - Williams, William G.

AU - Blackstone, Eugene H.

AU - DeCampli, William M.

AU - Duncan, Kim F.

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AB - Objectives: Improved survival after congenital heart surgery has led to interest in functional health status. We sought to identify factors associated with self-reported functional health status in adolescents and young adults with repaired interrupted aortic arch. Methods: Follow-up of survivors (aged 13-24 years) from a 1987 to 1997 inception cohort of neonates included completion of functional health status questionnaires (Child Health Questionnaire-CF87 [age <18 years, n = 51] or the Short Form [SF]-36 [age ≥18 years, n = 66]) and another about 22q11 deletion syndrome (22q11DS) features (n = 141). Factors associated with functional health status domains were determined using multivariable linear regression analysis. Results: Domain scores of respondents were significantly greater than norms in 2 of 9 Child Health Questionnaire-CF87 and 4 of 10 SF-36 domains and only lower in the physical functioning domain of the SF-36. Factors most commonly associated with lower scores included those suggestive of 22q11DS (low calcium levels, recurrent childhood infections, genetic testing/diagnosis, abnormal facial features, hearing deficits), the presence of self-reported behavioral and mental health problems, and a greater number of procedures. Factors explained between 10% and 70% of domain score variability (R 2 = 0.10-0.70, adj-R 2 = 0.09-0.66). Of note, morphology and repair type had a minor contribution. Conclusions: Morbidities associated with 22q11DS, psychosocial issues, and recurrent medical issues affect functional health status more than initial morphology and repair in this population. Nonetheless, these patients largely perceive themselves as better than their peers. This demonstrates the chronic nature of interrupted aortic arch and suggests the need for strategies to decrease reinterventions and for evaluation of mental health and genetic issues to manage associated deteriorations.

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