Impact of pulsatile pulmonary blood flow on cardiopulmonary exercise performance after the Fontan procedure

Lukas Klemm; Frank Klawonn; Christoph Röhlig; Thibault Schaeffer; Helena Staehler; Paul Philipp Heinisch; Nicole Piber; Alfred Hager; Peter Ewert; Jürgen Hörer; Masamichi Ono

doi:10.1016/j.xjon.2023.08.012

Impact of pulsatile pulmonary blood flow on cardiopulmonary exercise performance after the Fontan procedure

JTCVS Open. 2023 Sep 9:16:811-822. doi: 10.1016/j.xjon.2023.08.012. eCollection 2023 Dec.

Authors

Lukas Klemm^{1

2}, Frank Klawonn^{3

4}, Christoph Röhlig⁵, Thibault Schaeffer^{1

2}, Helena Staehler^{1

2}, Paul Philipp Heinisch^{1

2}, Nicole Piber⁶, Alfred Hager⁵, Peter Ewert⁵, Jürgen Hörer^{1

2}, Masamichi Ono^{1

2}

Affiliations

¹ Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, Technische Universität München, Munich, Germany.
² Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universität, Munich, Germany.
³ Biostatistics, Helmholtz Center for Infection Research, Braunschweig, Germany.
⁴ Department of Computer Science, Ostfalia University, Wolfenbüttel, Germany.
⁵ Department of Congenital Heart Disease and Pediatric Cardiology, German Heart Center Munich, Technische Universität München, Munich, Germany.
⁶ Department of Cardiovascular Surgery, German Heart Center Munich, Technische Universität München, Munich, Germany.

Abstract

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.

Keywords: atriopulmonary connection (Fontan–Kreutzer); atrioventricular connection (Fontan–Björk); exercise capacity; peak oxygen uptake; pulsatile pulmonary blood flow; total cavopulmonary connection.