title: Thoracic aortic dissection and rupture in conotruncal cardiac defects: A population-based study.
abstract: BACKGROUND
  Although the risk of thoracic aortic dissection and rupture (TAD) is well-known in bicuspid aortic valve (BAV), the risk of TAD in other congenital heart diseases (CHD), particularly conotruncal lesions like tetralogy of Fallot (TOF), truncus arteriosus, D-transposition of the great arteries (D-TGA), and double outlet right ventricle is currently unknown. The primary purpose of this study was to describe TAD in conotruncal CHD, and the secondary purpose was to explore whether an association exists between TAD and conotruncal CHD.
  
  
  RESULTS
  Using the Texas Inpatient Public Use Data File, an administrative database of all Texas hospitalizations, including >37.9 million hospitalizations from January 1999 through June 2012, 12,016 cases of TAD and 214 cases of TAD in CHD were identified. The most common lesions were BAV (42%), atrial septal defect (21%), aortic coarctation (7%), ventricular septal defect (6%), and patent ductus arteriosus (4%). Three patients with TOF, 2 with D-TGA, and 1 with truncus arteriosus were admitted with TAD. An exploratory case-control study in patients older than 1 year using multilevel logistic regression models to evaluate the association between CHD and TAD that controlled for known TAD risk factors demonstrated a significant association between TAD and BAV (OR 10, 95% CI 8.2-13) but not coarctation of the aorta or any conotruncal lesion.
  
  
  CONCLUSIONS
  TAD in conotruncal CHD is exquisitely rare. In our hospitalized population, there was no increased occurrence of TAD in conotruncal CHD above what would be expected in the rest of the hospitalized population.
authors.affiliation: Lillie Frank Abercrombie Section of Cardiology, Department of Pediatrics, Texas Children's Hospital, Baylor College of Medicine, Houston, TX, USA. Electronic address: bfrischh@bcm.edu.
authors.forename: Benjamin P
authors.initials: BP
authors.lastname: Frischhertz
authors.affiliation: The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
authors.forename: Pirouz
authors.initials: P
authors.lastname: Shamszad
authors.affiliation: Center for Clinical Research and Evidence-Based Medicine, University of Texas Health Science Center at Houston, Houston, TX, USA.
authors.forename: Claudia
authors.initials: C
authors.lastname: Pedroza
authors.affiliation: Division of Medical Genetics, Department of Internal Medicine, University of Texas Health Science Center at Houston, Houston, TX, USA.
authors.forename: Dianna M
authors.initials: DM
authors.lastname: Milewicz
authors.affiliation: Lillie Frank Abercrombie Section of Cardiology, Department of Pediatrics, Texas Children's Hospital, Baylor College of Medicine, Houston, TX, USA.
authors.forename: Shaine A
authors.initials: SA
authors.lastname: Morris
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000784:Aortic Dissection / Q000175:diagnosis / Q000401:mortality; D017545:Aortic Aneurysm, Thoracic / Q000175:diagnosis / Q000401:mortality; D001019:Aortic Rupture / Q000175:diagnosis / Q000401:mortality; D016022:Case-Control Studies; D002675:Child, Preschool; D015897:Comorbidity; D005260:Female; D006330:Heart Defects, Congenital / Q000145:classification / Q000175:diagnosis / Q000401:mortality; D006760:Hospitalization / Q000706:statistics & numerical data; D006801:Humans; D015994:Incidence; D007231:Infant, Newborn; D008297:Male; D012307:Risk Factors; D013223:Statistics as Topic; D014481:United States / Q000453:epidemiology
keywords: Aortic dissection; Congenital heart disease; Conotruncal; Tetralogy of Fallot; Transposition of the great arteries; Truncus arteriosus