title: Ventricular function and cardiac reserve in contemporary Fontan patients.
abstract: OBJECTIVE
  Total cavopulmonary connection (TCPC) has been the preferred treatment for patients with univentricular hearts. Current TCPC-techniques are the intra-atrial lateral tunnel (ILT) and the extracardiac conduit (ECC). We aimed to determine ventricular function during rest and stress, and to compare results for both techniques and for left (LV) versus right ventricular (RV) dominance.
  
  
  METHODS
  99 patients, aged 12.5 ± 4.0 years underwent echocardiography and magnetic resonance imaging (MRI), and 69 patients underwent stress MRI.
  
  
  RESULTS
  Echocardiography showed impaired systolic and diastolic function. MRI parameters were comparable between ILT and ECC at rest. During dobutamine there was a decrease in end-diastolic volume (EDVi) (91 ± 21 vs. 80 ± 20 ml/m(2) p<0.001). Ejection fraction (EF) and cardiac index (CI) during dobutamine were lower for ILT patients (59 ± 11 (ILT) vs. 64 ± 7% (ECC), p=0.027 and 4.2 ± 1.0 (ILT) vs. 4.9 ± 1.0L/min/m(2) (ECC), p=0.006), whereas other parameters were comparable. TEI-index was higher in ILT-patients (0.72 ± 0.27 (ILT) vs. 0.56 ± 0.22 (ECC), p=0.002). Diastolic function was frequently impaired in patients with a dominant RV (67% (RV) vs. 39% (LV), p=0.011). Patients with dominant LV's had smaller end-systolic volume (ESVi) (40 ± 13 (LV) vs. 47 ± 16 (RV) ml/m(2), p=0.030) and higher EF (55 ± 8 (LV) vs. 49 ± 9 ml/m(2) (RV), p=0.001) and contractility (2.3 ± 0.8 (LV) vs. 1.9 ± 0.7 mmHg/ml/m(2) (RV), p=0.050) during rest and higher EF during dobutamine (63 ± 8 (LV) vs. 58 ± 10 ml/m(2) (RV), p=0.043).
  
  
  CONCLUSIONS
  Ventricular function is relatively well preserved in modern-day Fontan patients. With dobutamine stress there is a decrease in EDVi. ECC patients have higher CI and EF during stress. Patients with a dominant RV have lower systolic, including impaired contractility, and diastolic function.
authors.affiliation: Department of Pediatrics, Division of Pediatric Cardiology, Erasmus Medical Center - Sophia Children's Hospital, Rotterdam, The Netherlands; Department of Radiology, Erasmus Medical Center, Rotterdam, The Netherlands.
authors.forename: S S M
authors.initials: SS
authors.lastname: Bossers
authors.affiliation: Department of Pediatric Cardiology, Radboud University Medical Centre - Amalia Children's Hospital, Nijmegen, The Netherlands,; Department of Pediatrics, Pediatric Cardiology Unit, Tel-Aviv Sourasky Medical Center, Tel Aviv, Israel.
authors.forename: L
authors.initials: L
authors.lastname: Kapusta
authors.affiliation: Department of Pediatric Cardiology, Academic Medical Center, Amsterdam, The Netherlands.
authors.forename: I M
authors.initials: IM
authors.lastname: Kuipers
authors.affiliation: Department of Pediatric Cardiology, University Medical Center Utrecht - Wilhelmina Children's Hospital, Utrecht, The Netherlands.
authors.forename: G
authors.initials: G
authors.lastname: van Iperen
authors.affiliation: Department of Radiology, Erasmus Medical Center, Rotterdam, The Netherlands.
authors.forename: A
authors.initials: A
authors.lastname: Moelker
authors.affiliation: Department of Radiology, Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: L J M
authors.initials: LJ
authors.lastname: Kroft
authors.affiliation: Department of Radiology, Academic Medical Center, Amsterdam, The Netherlands.
authors.forename: S
authors.initials: S
authors.lastname: Romeih
authors.affiliation: Department of Clinical Chemistry, Erasmus Medical Center, Rotterdam, The Netherlands.
authors.forename: Y
authors.initials: Y
authors.lastname: de Rijke
authors.affiliation: Department of Paediatric Cardiology, Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: A D J
authors.initials: AD
authors.lastname: Ten Harkel
authors.affiliation: Department of Pediatrics, Division of Pediatric Cardiology, Erasmus Medical Center - Sophia Children's Hospital, Rotterdam, The Netherlands; Department of Radiology, Erasmus Medical Center, Rotterdam, The Netherlands. Electronic address: w.a.helbing@erasmusmc.nl.
authors.forename: W A
authors.initials: WA
authors.lastname: Helbing
mesh_terms: D000293:Adolescent; D002648:Child; D004280:Dobutamine / Q000378:metabolism; D005260:Female; D018729:Fontan Procedure; D053805:Fractional Flow Reserve, Myocardial; D006330:Heart Defects, Congenital / Q000601:surgery*; D006352:Heart Ventricles / Q000000981:diagnostic imaging / Q000503:physiopathology*; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D013312:Stress, Physiological / Q000502:physiology; D014463:Ultrasonography; D016276:Ventricular Function / Q000502:physiology*
chemical_list: D004280:Dobutamine
keywords: Cardiac function; Congenital heart disease; Echocardiography; Fontan; MRI; Total cavopulmonary connection