title: Frequent Dilatation of the Descending Aorta in Children With Hypoplastic Left Heart Syndrome Relates to Decreased Aortic Arch Elasticity.
abstract: BACKGROUND
  Patients with hypoplastic left heart syndrome after a Norwood operation show dilatation and reduced distensibility of the reconstructed proximal aorta. Cardiac magnetic resonance imaging (CMR) and angiographic examinations indicate that the native descending aorta (DAo) is also dilated, but this has not been studied in detail.
  
  
  RESULTS
  Seventy-nine children with hypoplastic left heart syndrome in Fontan circulation (aged 6.3±3.2 years) and 18 control participants (aged 6.8±2.4 years) underwent 3.0-tesla CMR. Gradient-echo cine and phase-contrast imaging was applied to measure cross-sectional areas (CSAs), distensibility, pulse wave velocity, and the incremental elastic modulus of the thoracic aorta. CSA of the DAo in patients was also compared with published percentiles for aortic CSA. Patients had significantly larger CSA of the DAo at the level of pulmonary artery bifurcation (229.1±97.2 versus 175.7±24.3 mm/m(2), P=0.04) and the diaphragm (196.2±66.0 versus 142.6±16.7 mm/m(2), P<0.01). In 41 patients (52%), CSA of the DAo was >95th percentile level for control participants, and the incremental elastic modulus of the aortic arch and the DAo was higher than in patients with normal CSAs (arch: 90.1±64.3 versus 45.6±38.9 m/s; DAo: 86.3±53.7 versus 47.1±47.6 m/s; P<0.01). Incremental elastic modulus of the aortic arch and the DAo correlated with the CSA of the DAo (arch: r=0.5; DAo: r=0.49; P<0.01).
  
  
  CONCLUSIONS
  Children with hypoplastic left heart syndrome frequently show dilatation of their DAo associated with increased stiffness of the aortic arch. Higher aortic impedance increases the afterload of the systemic circulation and likely contributes to the burden of the systemic right ventricle.
authors.affiliation: Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital of Schleswig-Holstein, Kiel, Germany (I.V., P.W., C.H., D.G., A.A.B., G.F., A.C.A., H.M.P., I.K., H.H.K., C.R.).
authors.forename: Inga
authors.initials: I
authors.lastname: Voges
authors.affiliation: Department of Radiology, Brigham & Women's Hospital & Harvard Medical School, Boston, MA (M.J.H.).
authors.forename: Michael
authors.initials: M
authors.lastname: Jerosch-Herold
authors.affiliation: Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital of Schleswig-Holstein, Kiel, Germany (I.V., P.W., C.H., D.G., A.A.B., G.F., A.C.A., H.M.P., I.K., H.H.K., C.R.).
authors.forename: Philip
authors.initials: P
authors.lastname: Wegner
authors.affiliation: Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital of Schleswig-Holstein, Kiel, Germany (I.V., P.W., C.H., D.G., A.A.B., G.F., A.C.A., H.M.P., I.K., H.H.K., C.R.).
authors.forename: Christopher
authors.initials: C
authors.lastname: Hart
authors.affiliation: Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital of Schleswig-Holstein, Kiel, Germany (I.V., P.W., C.H., D.G., A.A.B., G.F., A.C.A., H.M.P., I.K., H.H.K., C.R.).
authors.forename: Dominik
authors.initials: D
authors.lastname: Gabbert
authors.affiliation: Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital of Schleswig-Holstein, Kiel, Germany (I.V., P.W., C.H., D.G., A.A.B., G.F., A.C.A., H.M.P., I.K., H.H.K., C.R.).
authors.forename: Abdullah
authors.initials: A
authors.lastname: Al Bulushi
authors.affiliation: Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital of Schleswig-Holstein, Kiel, Germany (I.V., P.W., C.H., D.G., A.A.B., G.F., A.C.A., H.M.P., I.K., H.H.K., C.R.).
authors.forename: Gunther
authors.initials: G
authors.lastname: Fischer
authors.affiliation: Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital of Schleswig-Holstein, Kiel, Germany (I.V., P.W., C.H., D.G., A.A.B., G.F., A.C.A., H.M.P., I.K., H.H.K., C.R.).
authors.forename: Ana Cristina
authors.initials: AC
authors.lastname: Andrade
authors.affiliation: Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital of Schleswig-Holstein, Kiel, Germany (I.V., P.W., C.H., D.G., A.A.B., G.F., A.C.A., H.M.P., I.K., H.H.K., C.R.).
authors.forename: Hoang Minh
authors.initials: HM
authors.lastname: Pham
authors.affiliation: Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital of Schleswig-Holstein, Kiel, Germany (I.V., P.W., C.H., D.G., A.A.B., G.F., A.C.A., H.M.P., I.K., H.H.K., C.R.).
authors.forename: Ines
authors.initials: I
authors.lastname: Kristo
authors.affiliation: Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital of Schleswig-Holstein, Kiel, Germany (I.V., P.W., C.H., D.G., A.A.B., G.F., A.C.A., H.M.P., I.K., H.H.K., C.R.).
authors.forename: Hans-Heiner
authors.initials: HH
authors.lastname: Kramer
authors.affiliation: Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital of Schleswig-Holstein, Kiel, Germany (I.V., P.W., C.H., D.G., A.A.B., G.F., A.C.A., H.M.P., I.K., H.H.K., C.R.).
authors.forename: Carsten
authors.initials: C
authors.lastname: Rickers
mesh_terms: D001013:Aorta, Thoracic / Q000473:pathology* / Q000503:physiopathology*; D017545:Aortic Aneurysm, Thoracic / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D004108:Dilatation, Pathologic; D055119:Elastic Modulus; D005260:Female; D018729:Fontan Procedure; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery; D019028:Magnetic Resonance Imaging, Cine; D008297:Male; D010166:Palliative Care; D011237:Predictive Value of Tests; D063177:Pulse Wave Analysis; D015203:Reproducibility of Results; D012307:Risk Factors; D014655:Vascular Resistance; D059289:Vascular Stiffness; D016278:Ventricular Function, Right
keywords: aortic elasticity; descending aortic dilatation; hypoplastic left heart syndrome; incremental elastic modulus; magnetic resonance imaging