title: Morbidity in children and adolescents after surgical correction of truncus arteriosus communis.
abstract: BACKGROUND
  Studies of outcome after operative correction of truncus arteriosus communis (TA) have focused on mortality and rates of reintervention. We sought to investigate the clinical status of children and adolescents with surgically corrected TA.
  
  
  RESULTS
  A cross-sectional study of subjects with TA was performed. Subjects underwent concurrent genetic testing, electrocardiogram, cardiac magnetic resonance imaging, cardiopulmonary exercise testing, and completed questionnaires assessing health status and health-related quality of life. Review of their medical history provided retrospective information on cardiac reintervention and use of medical care. Twenty-five subjects with a median age of 11.8 (8.1-18.99) years were enrolled. The prevalence of 22q11.2 deletion was 32%. Incidence of hospitalization, cardiac reintervention, and noncardiac operations was highest in the first year of life. Combined catheter-based and operative reintervention rates were 52% on the conduit and 56% on the pulmonary arteries. Right ventricular ejection fraction and end-diastolic volume were normal. Moderate or greater truncal valve insufficiency was seen in 11% of subjects, and truncal valve replacement occurred in 8% of subjects. Maximal oxygen consumption (P = .0002), maximal work (P < .0001), and forced vital capacity (P < .0001) were all lower than normal for age and sex. Physical health status and health-related quality of life were both severely diminished.
  
  
  CONCLUSIONS
  Patients with TA demonstrate significant comorbid disease throughout childhood, significant burden of operative and catheter-based reintervention, and deficits in exercise performance, functional status, and health-related quality of life.
authors.affiliation: Division of Pediatric Cardiology, The Children's Hospital of Philadelphia, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA. Electronic address: obyrnem@email.chop.edu.
authors.forename: Michael L
authors.initials: ML
authors.lastname: O'Byrne
authors.forename: Laura
authors.initials: L
authors.lastname: Mercer-Rosa
authors.forename: Huaqing
authors.initials: H
authors.lastname: Zhao
authors.forename: Xuemei
authors.initials: X
authors.lastname: Zhang
authors.forename: Wei
authors.initials: W
authors.lastname: Yang
authors.forename: Amy
authors.initials: A
authors.lastname: Cassedy
authors.forename: Mark A
authors.initials: MA
authors.lastname: Fogel
authors.forename: Jack
authors.initials: J
authors.lastname: Rychik
authors.forename: Ronn E
authors.initials: RE
authors.lastname: Tanel
authors.forename: Bradley S
authors.initials: BS
authors.lastname: Marino
authors.forename: Stephen
authors.initials: S
authors.lastname: Paridon
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Goldmuntz
mesh_terms: D000293:Adolescent; D006348:Cardiac Surgical Procedures / Q000706:statistics & numerical data*; D002648:Child; D003430:Cross-Sectional Studies; D004562:Electrocardiography; D005080:Exercise Test; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications* / Q000503:physiopathology / Q000601:surgery*; D006760:Hospitalization / Q000706:statistics & numerical data*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009017:Morbidity; D010101:Oxygen Consumption / Q000502:physiology*; D011788:Quality of Life; D011795:Surveys and Questionnaires; D016896:Treatment Outcome
references.citation: Pediatr Cardiol. 2000 Jan-Feb;21(1):37-46
references.pmid: 10672613
references.citation: J Thorac Cardiovasc Surg. 1996 Apr;111(4):849-56
references.pmid: 8614146
references.citation: Ann Thorac Surg. 2011 Dec;92(6):2184-91; discussion 2191-2
references.pmid: 22115229
references.citation: Ann Thorac Surg. 2012 Jan;93(1):164-9; discussion 169
references.pmid: 22088417
references.citation: Eur J Cardiothorac Surg. 2010 Jun;37(6):1278-84
references.pmid: 20137966
references.citation: Ann Thorac Surg. 2001 Aug;72(2):391-5
references.pmid: 11515872
references.citation: J Am Coll Cardiol. 1998 Aug;32(2):492-8
references.pmid: 9708481
references.citation: Hum Mutat. 2006 Aug;27(8):814-21
references.pmid: 16791841
references.citation: Pediatr Cardiol. 2013 Oct;34(7):1687-94
references.pmid: 23604262
references.citation: Pediatr Cardiol. 2013 Apr;34(4):853-60
references.pmid: 23104594
references.citation: Expert Rev Cardiovasc Ther. 2011 Dec;9(12):1527-38
references.pmid: 22103872
references.citation: Circulation. 1976 Jul;54(1):108-11
references.pmid: 1277412
references.citation: J Thorac Cardiovasc Surg. 2013 Apr;145(4):1046-1057.e1
references.pmid: 22835225
references.citation: J Thorac Cardiovasc Surg. 2009 Oct;138(4):911-6
references.pmid: 19660342
references.citation: Pediatrics. 2010 Sep;126(3):498-508
references.pmid: 20805147
references.citation: Congenit Heart Dis. 2010 May-Jun;5(3):262-70
references.pmid: 20576045
references.citation: J Thorac Cardiovasc Surg. 2010 Aug;140(2):325-9
references.pmid: 20427059
references.citation: Eur J Cardiothorac Surg. 2001 Aug;20(2):221-7
references.pmid: 11463535
references.citation: J Cardiovasc Magn Reson. 2009 Jun 21;11:19
references.pmid: 19545393
references.citation: Ann Surg. 1984 Oct;200(4):451-6
references.pmid: 6237622
references.citation: Eur J Cardiothorac Surg. 2010 Jul;38(1):52-8
references.pmid: 20207551
references.citation: Am J Cardiol. 1965 Sep;16(3):406-25
references.pmid: 5828135
references.citation: Eur J Cardiothorac Surg. 2008 May;33(5):890-8
references.pmid: 18313324
references.citation: JAMA. 1968 Jul 8;205(2):69-73
references.pmid: 4872743
references.citation: Ann Thorac Surg. 1998 Dec;66(6 Suppl):S183-8
references.pmid: 9930445
references.citation: J Thorac Cardiovasc Surg. 2005 Mar;129(3):559-68
references.pmid: 15746739
references.citation: J Thorac Cardiovasc Surg. 1997 May;113(5):869-78; discussion 878-9
references.pmid: 9159620
references.citation: Ann Thorac Surg. 2008 Jan;85(1):172-8
references.pmid: 18154803
references.citation: Qual Life Res. 2008 May;17(4):613-26
references.pmid: 18347927