Hostname: page-component-7b9c58cd5d-v2ckm Total loading time: 0 Render date: 2025-03-15T12:19:50.902Z Has data issue: false hasContentIssue false
  • English
  • Français

Lower limb exercise generates pulsatile flow into the pulmonary vascular bed in the setting of the Fontan circulation

Published online by Cambridge University Press:  28 February 2018

Rachael Cordina ,
David S. Celermajer  and
Yves d’Udekem
Rachael Cordina*
Affiliation:
Sydney Medical School, University of Sydney, Sydney, NSW, Australia Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia
David S. Celermajer
Affiliation:
Sydney Medical School, University of Sydney, Sydney, NSW, Australia Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia
Yves d’Udekem
Affiliation:
Heart Research Group, Murdoch Children’s Research Institute, Melbourne, VIC, Australia Department of Paediatrics, Faculty of Medicine, The University of Melbourne, Melbourne, VIC, Australia Department of Cardiac Surgery, Royal Children’s Hospital, Melbourne, VIC, Australia
*
Author for correspondence: Rachael Cordina, MBBS, PhD, FRACP, Department of Cardiology, Royal Prince Alfred Hospital, Missenden Rd, Camperdown, Sydney, NSW, Australia. Tel: +61 2 9515 6111; Fax: +61 2 9550 6262; E-mail: rachael.cordina@sydney.edu.au
Rights & Permissions [Opens in a new window]

Abstract

The absence of a subpulmonary ventricle in the Fontan circulation results in non-pulsatile pulmonary blood flow. Lower limb exercise in this setting can generate pulsatile pulmonary blood flow.

Keywords

Fontanexerciseperipheral muscle pumpskeletal muscleexercise physiology
Type
Images in Congenital Cardiac Disease
Information
Cardiology in the Young , Volume 28 , Issue 5 , May 2018 , pp. 732 - 733
Check for updates
Copyright
© Cambridge University Press 2018 

The absence of a subpulmonary ventricle in the univentricular Fontan circulation results in non-pulsatile low contributing to attenuated pulmonary artery growth, pulmonary endothelial dysfunction, increased pulmonary vascular resistance, and circulatory demise. This unique circulation, which lacks a subpulmonary cardiac power source, relies heavily on the peripheral muscle pump for venous return during exercise. We previously demonstrated that an important deficiency of skeletal muscle mass exists in these patients and that resistance training to augment the peripheral muscle pump improves peak exercise capacity, and stroke volume at rest and during exercise.Reference Cordina, O’Meagher and Karmali 1

A 30-year-old woman with a Fontan circulation – total cavopulmonary connection – for pulmonary atresia with ventricular septal defect and hypoplastic right ventricle was assessed with echocardiography (CX50, S51 probe; Philips, Amsterdam, The Netherlands) using pulsed-wave Doppler within the intracardiac cavopulmonary connection on a semi-erect ergometer, at a 45° tilt. The electrocardiography leads were placed on the legs to detect leg movement instead of cardiac activity. At rest, non-pulsatile, low-velocity flow was demonstrated (Fig 1, panel A). During lower limb exercise, pulsatile flow with increased velocity was observed in the cavopulmonary pathway.

Figure 1 Pulsed-wave Doppler spectral trace measured within an intracardiac total cavopulmonary connection at the onset of semi-erect lower limb exercise demonstrating a marked change in flow velocity and pulsatility.

The peripheral muscle pump may not only aid venous return by simply pushing the volume back to the heart but also creates periodic pulsatile increases in flow. Regular exercise to maintain peripheral muscle bulk and improve pulmonary blood flow characteristics is probably crucial for a healthy Fontan circulation.

Acknowledgements

None.

Financial Support

This testing was supported by a grant from the National Health and Medical Research Council (NHMRC Project Grant 1047923). The authors acknowledge support provided to the Murdoch Children’s Research Institute by the Victorian Government’s Operational Infrastructure Support Program. Y.d’U. is a NHMRC Clinician Practitioner Fellow (1082186).

Conflicts of Interest

Y.d’U. is consultant for MSD and Actelion. The remaining authors have nothing to disclose.

Ethical Standards

The authors assert that all procedures contributing to this work comply with the ethical standards of the National Statement on Ethical Conduct in Human Research (Australia) and with the Helsinki Declaration of 1975, as revised in 2008, and has been approved by the Sydney Local Health District Ethics Committee.

References

1. Cordina, RL, O’Meagher, S, Karmali, A, et al. Resistance training improves cardiac output, exercise capacity and tolerance to positive airway pressure in Fontan physiology. Int J Cardiol 2013; 168: 780–788.Google Scholar
Figure 0

Figure 1 Pulsed-wave Doppler spectral trace measured within an intracardiac total cavopulmonary connection at the onset of semi-erect lower limb exercise demonstrating a marked change in flow velocity and pulsatility.