Impact of pulsatile pulmonary blood flow on cardiopulmonary exercise performance after the Fontan procedureCentral MessagePerspective
Lukas Klemm, MS,
Frank Klawonn, MSc, PhD,
Christoph Röhlig, MD,
Thibault Schaeffer, MD,
Helena Staehler, MS,
Paul Philipp Heinisch, MD, PhD,
Nicole Piber, MD,
Alfred Hager, MD, PhD,
Peter Ewert, MD, PhD,
Jürgen Hörer, MD, PhD,
Masamichi Ono, MD, PhD
Affiliations
Lukas Klemm, MS
Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, Technische Universität München, Munich, Germany; Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universität, Munich, Germany
Frank Klawonn, MSc, PhD
Biostatistics, Helmholtz Center for Infection Research, Braunschweig, Germany; Department of Computer Science, Ostfalia University, Wolfenbüttel, Germany
Christoph Röhlig, MD
Department of Congenital Heart Disease and Pediatric Cardiology, German Heart Center Munich, Technische Universität München, Munich, Germany
Thibault Schaeffer, MD
Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, Technische Universität München, Munich, Germany; Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universität, Munich, Germany
Helena Staehler, MS
Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, Technische Universität München, Munich, Germany; Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universität, Munich, Germany
Paul Philipp Heinisch, MD, PhD
Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, Technische Universität München, Munich, Germany; Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universität, Munich, Germany
Nicole Piber, MD
Department of Cardiovascular Surgery, German Heart Center Munich, Technische Universität München, Munich, Germany
Alfred Hager, MD, PhD
Department of Congenital Heart Disease and Pediatric Cardiology, German Heart Center Munich, Technische Universität München, Munich, Germany
Peter Ewert, MD, PhD
Department of Congenital Heart Disease and Pediatric Cardiology, German Heart Center Munich, Technische Universität München, Munich, Germany
Jürgen Hörer, MD, PhD
Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, Technische Universität München, Munich, Germany; Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universität, Munich, Germany
Masamichi Ono, MD, PhD
Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, Technische Universität München, Munich, Germany; Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universität, Munich, Germany; Address for reprints: Masamichi Ono, MD, PhD, Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, Lazarettstraße 36, 80636 Munich, Germany.
Objective: To evaluate the exercise capacity in patients following Fontan–Kreutzer, Fontan–Björk, and total cavopulmonary connection (TCPC). Methods: Patients who performed exercise capacity tests at least once after the Fontan procedure between 1979 and 2007 were included. Patients after Fontan–Björk procedure were divided into 2 groups according to the pulmonary blood flow (PBF) pattern: patients with pulsatile PBF and those without. Peak oxygen uptake (VO2) was measured and percent-predicted VO2 was calculated. Results: A total of 227 patients were nominated. The types of Fontan procedure included Fontan–Kreutzer in 48 (21.1%) patients, Fontan–Björk in 38 (16.7%); 11 (4.8%) with pulsatile PBF and 27 (11.9%) without pulsatile PBF; and TCPC in 141 (62.1%). Median age at the Fontan procedure was 4.5 years (interquartile range, 2.1-8.2 years). A total of 978 cardiopulmonary exercise tests were performed at median follow-up of 17.7 years (interquartile range, 11.3-23.4 years) postoperatively. Analysis using linear mixed-effects models demonstrated that percent-predicted VO2 was greater in patients with pulsatile PBF after Fontan–Björk compared with patients after other types of Fontan procedure (P < .001). The same results were obtained when the longitudinal percent predicted VO2 was performed using only patients with tricuspid atresia and double inlet left ventricle (P < .001). Conclusions: Among long-term survivors after various types of Fontan procedures, patients with pulsatile PBF after the Fontan–Björk procedure demonstrated better exercise performance compared to those after TCPC, those after the Fontan–Kreutzer procedure, and those after the Fontan-Björk procedure with non-pulsatile PBF. The results implicate the importance of pulsatile PBF to maintain the Fontan circulation.