title: Improved Exercise Performance in Patients With Tricuspid Atresia After the Fontan-Björk Modification With Pulsatile Systolic Pulmonary Flow.
abstract: BACKGROUND
  After the Fontan-Björk modification for tricuspid atresia, some patients show pulsatile systolic pulmonary flow. We compared the hemodynamic findings and the clinical presentation of patients with and without pulsatile systolic flow after atrioventricular connection.
  
  
  METHODS
  According to the pulmonary flow pattern by pulsed-wave Doppler assessment of transthoracic echocardiography, 41 patients after atrioventricular connection were divided into two groups: patients who showed dominant pulsatile systolic pulmonary flow (group P, n = 11), and patients who did not (group N, n = 30).
  
  
  RESULTS
  Mean follow-up time was 27.8 ± 4.7 years in group P and 25.3 ± 3.8 years in group N (p = 0.1). Patients in group P had significantly less frequently catheter ablation procedures for tachyarrhythmia (9% versus 50%, p = 0.03). No patient in group P had had cardiac decompensation, whereas 7 patients (23%) in group N had had an episode of cardiac decompensation (p = 0.08). Cardiopulmonary exercise testing revealed that patients in group P showed higher oxygen uptake compared with patients in group N (25.0 ± 7.3 versus 19.6 ± 6.0 mL · kg(-1) · min(-1), p = 0.03). Patients in group P showed higher systolic pulmonary artery pressure (21.3 ± 8.4 versus 16.8 ± 4.5 mm Hg, p = 0.05), higher right ventricular end-diastolic volume index (88.6 ± 30.2 versus 50.3 ± 28.5 mL · L(-1) · m(-2), p = 0.03), and higher right ventricle to left ventricle ratio of end-diastolic volume index (1.4 ± 0.6 to 0.7 ± 0.3, p = 0.01).
  
  
  CONCLUSIONS
  Patients with pulsatile systolic flow in the pulmonary artery had better hemodynamic and better exercise performance compared with patients without pulsatile systolic flow after atrioventricular connection. A sufficient volume and function of the right ventricle is a prerequisite to create pulsatile systolic flow.
authors.affiliation: Department of Cardiovascular Surgery, German Heart Center Munich at Technische Universität München, Munich, Germany. Electronic address: ono@dhm.mhn.de.
authors.forename: Masamichi
authors.initials: M
authors.lastname: Ono
authors.affiliation: Department of Pediatric Cardiology and Congenital Heart Disease, German Heart Center Munich at Technische Universität München, Munich, Germany.
authors.forename: Manfred
authors.initials: M
authors.lastname: Vogt
authors.affiliation: Department of Cardiovascular Surgery, German Heart Center Munich at Technische Universität München, Munich, Germany.
authors.forename: Julie
authors.initials: J
authors.lastname: Cleuziou
authors.affiliation: Department of Cardiovascular Surgery, German Heart Center Munich at Technische Universität München, Munich, Germany.
authors.forename: Jelena
authors.initials: J
authors.lastname: Kasnar-Samprec
authors.affiliation: Department of Cardiovascular Surgery, German Heart Center Munich at Technische Universität München, Munich, Germany.
authors.forename: Melchior
authors.initials: M
authors.lastname: Burri
authors.affiliation: Department of Cardiovascular Surgery, German Heart Center Munich at Technische Universität München, Munich, Germany.
authors.forename: Martina
authors.initials: M
authors.lastname: Strbad
authors.affiliation: Department of Pediatric Cardiology and Congenital Heart Disease, German Heart Center Munich at Technische Universität München, Munich, Germany.
authors.forename: Alfred
authors.initials: A
authors.lastname: Hager
authors.affiliation: Department of Cardiovascular Surgery, German Heart Center Munich at Technische Universität München, Munich, Germany.
authors.forename: Christian
authors.initials: C
authors.lastname: Schreiber
authors.affiliation: Department of Cardiovascular Surgery, German Heart Center Munich at Technische Universität München, Munich, Germany.
authors.forename: Jürgen
authors.initials: J
authors.lastname: Hörer
authors.affiliation: Department of Cardiovascular Surgery, German Heart Center Munich at Technische Universität München, Munich, Germany.
authors.forename: Rüdiger
authors.initials: R
authors.lastname: Lange
mesh_terms: D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D018619:Echocardiography, Doppler, Pulsed / Q000379:methods*; D015444:Exercise / Q000502:physiology*; D005080:Exercise Test; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure / Q000379:methods*; D005544:Forecasting; D006801:Humans; D008297:Male; D011184:Postoperative Period; D011651:Pulmonary Artery / Q000000981:diagnostic imaging / Q000503:physiopathology*; D011669:Pulmonary Wedge Pressure / Q000502:physiology; D011673:Pulsatile Flow / Q000502:physiology; D012189:Retrospective Studies; D013318:Stroke Volume / Q000502:physiology*; D013599:Systole; D018785:Tricuspid Atresia / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000601:surgery*