title: Arterial elastance and its impact on intrinsic right ventricular function in palliated hypoplastic left heart syndrome.
abstract: BACKGROUND
  Systemic right ventricular (RV) function is a major determinant of long-term outcome in patients with palliated hypoplastic left heart syndrome (HLHS). Abnormal elastic properties of the reconstructed aorta may negatively impact on ventricular function. We therefore aimed to assess arterial elastance and its relationship to systemic RV function in these patients.
  
  
  METHODS
  Fifty-six HLHS patients (median age 5.4 years; range 2.9-14.2 years) were studied at a median of 2.6 years (range 0.8-12.7 years) after completion of the Fontan circulation with the pressure-volume conductance system.
  
  
  RESULTS
  Arterial elastance (Ea) was abnormally high and correlated inversely with RV ejection fraction (r=-0.42, P=0.001). However, end systolic elastance (Ees) - a load independent measure of intrinsic systolic ventricular function - and more so end diastolic stiffness (Eed) were positively correlated with Ea (Ees vs. Ea: r=0.44, P=0.001: Eed vs. Ea: r=0.62, P<0.0001). Patients who were treated for significant aortic arch obstruction after surgical palliation showed higher Ea and Eed even four years after successful treatment compared to the remainder of the group (Ea: 3.4 ± 1.2 vs. 2.8 ± 1.0 mmHg/ml, P=0.04 and Eed: 0.67 ± 0.44 vs. 0.45 ± 0.3 mmHg/ml, P=0.04).
  
  
  CONCLUSIONS
  Arterial elastance is abnormally high in palliated HLHS patients and negatively impacts on ejection fraction but not on intrinsic systolic RV function early after completion of the Fontan circulation. Increased arterial elastance, however, is associated with increased RV diastolic stiffness with potential adverse effects on long-term outcome. Furthermore, arterial elastance and diastolic stiffness are particularly high in patients who needed treatment for aortic arch obstruction.
authors.affiliation: Department of Congenital Heart Disease and Paediatric Cardiology, University Hospital of Schleswig-Holstein, Campus Kiel, Arnold-Heller-Str.3, Haus 9, 24105 Kiel, Germany. Electronic address: jana.schlangen@pedcard.uni-kiel.de.
authors.forename: Jana
authors.initials: J
authors.lastname: Schlangen
authors.forename: Gunther
authors.initials: G
authors.lastname: Fischer
authors.forename: Colin
authors.initials: C
authors.lastname: Petko
authors.forename: Jan Hinnerk
authors.initials: JH
authors.lastname: Hansen
authors.forename: Inga
authors.initials: I
authors.lastname: Voges
authors.forename: Carsten
authors.initials: C
authors.lastname: Rickers
authors.forename: Hans-Heiner
authors.initials: HH
authors.lastname: Kramer
authors.forename: Anselm
authors.initials: A
authors.lastname: Uebing
mesh_terms: D000293:Adolescent; D001011:Aorta / Q000502:physiology / Q000601:surgery; D001017:Aortic Coarctation / Q000503:physiopathology* / Q000601:surgery; D001794:Blood Pressure / Q000502:physiology; D002648:Child; D002675:Child, Preschool; D004548:Elasticity; D054459:Elasticity Imaging Techniques; D005260:Female; D018729:Fontan Procedure; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000503:physiopathology* / Q000601:surgery; D008297:Male; D013318:Stroke Volume / Q000502:physiology; D013599:Systole / Q000502:physiology; D014655:Vascular Resistance / Q000502:physiology; D059289:Vascular Stiffness / Q000502:physiology*; D016278:Ventricular Function, Right / Q000502:physiology*
keywords: Arterial elastance; Coarctation; Hypoplastic left heart syndrome; Ventricular function