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J Thorac Cardiovasc Surg. 2019 Jan 11. pii: S0022-5223(19)30001-7. doi: 10.1016/j.jtcvs.2018.11.152. [Epub ahead of print]

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

Author information

1
Division of Cardiac Surgery, Department of Surgery, University of Toronto, Hospital for Sick Children, Toronto, Ontario, Canada. Electronic address: anusha.jegatheeswaran@utoronto.ca.
2
Division of Cardiac Surgery, Department of Surgery, Johns Hopkins University, Baltimore, Md.
3
Division of Cardiac Surgery, Department of Surgery, University of Toronto, Hospital for Sick Children, Toronto, Ontario, Canada.
4
Division of Cardiothoracic Surgery, Department of Surgery, Levine Children's Hospital, Charlotte, NC.
5
Department of Thoracic and Cardiovascular Surgery, Lerner Research Institute, Cleveland Clinic Foundation, Cleveland, Ohio.
6
Division of Cardiac Surgery, Department of Surgery, University of Central Florida College of Medicine, Arnold Palmer Hospital for Children, Orlando, Fla.
7
Division of Cardiothoracic Surgery, Department of Surgery, University of Nebraska Medical Center, Omaha, Neb.
8
Division of Cardiothoracic Surgery, Department of Surgery, University of Utah, Primary Children's Hospital, Salt Lake City, Utah.
9
Division of Cardiovascular Surgery, Department of Surgery, Wayne State University, Children's Hospital of Michigan, Detroit, Mich.
10
Division of Pediatric Cardiac Surgery, Department of Surgery, McGill University, Montreal Children's Hospital, Montreal, Québec, Canada.
11
Department of Pediatrics, Division of Cardiology, University of Toronto, Hospital for Sick Children, Toronto, Ontario, Canada.

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 (R2 = 0.10-0.70, adj-R2 = 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.

KEYWORDS:

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

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