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Living at an altitude adversely affects exercise capacity in Fontan patients

Published online by Cambridge University Press:  20 September 2010

Jeffrey R. Darst*
The Heart Institute, The Children’s Hospital, Aurora, Colorado, United States of America
Marko Vezmar
Labatt Family Heart Centre, The Hospital for Sick Children, Toronto, Ontario, Canada
Brian W. McCrindle
Labatt Family Heart Centre, The Hospital for Sick Children, Toronto, Ontario, Canada
Cedric Manlhiot
Labatt Family Heart Centre, The Hospital for Sick Children, Toronto, Ontario, Canada
Amy Taylor
The Heart Institute, The Children’s Hospital, Aurora, Colorado, United States of America
Jennifer Russell
Labatt Family Heart Centre, The Hospital for Sick Children, Toronto, Ontario, Canada
Anji T. Yetman
The Heart Center, Primary Children’s Hospital, Salt Lake City, Utah, United States of America
Correspondence to: J. R. Darst, MD, The Children’s Hospital B-100, 13123 E 16th Avenue, Aurora, Colorado 80045, United States of America. Tel: +01 720 777 1949; Fax: +01 720 777 7372; E-mail:



Data assessing the effect of altitude on Fontan haemodynamics are limited to experimental models and case reports. Both suggest a detrimental impact. This study describes exercise performance in patients with Fontan circulation and matched controls at a low altitude versus at sea level. We sought to assess the impact of increasing altitude on functional capacity in patients with Fontan palliation.


A retrospective review of 22 patients at low altitude (1602 metres) and 119 patients at sea level with Fontan circulation, as well as age-, gender-, and altitude-matched controls, underwent maximal cardiopulmonary exercise testing. Linear regression models were created to determine the influence of altitude on differences in exercise variables between Fontan patients and their matched controls.


Peak oxygen consumption was 28.4 millilitres per kilogram per minute (72% predicted) for the sea-level cohort and 24.2 millilitres per kilogram per minute (63% predicted) for the moderate altitude cohort. The matched case–control differences for patients at moderate altitude were greater for peak oxygen consumption (−29% against −13%, p = 0.04), anaerobic threshold (−36% against −5%, p = 0.001), and oxygen pulse (−35% against −18%, p = 0.007) when compared with patients living at sea level. When compared to institution-matched controls, the same parameters fell by 3%, 8.9%, and 4.2%, respectively, for each increase of 1000 feet in residential altitude (p = 0.03, p = 0.001, and p = 0.05, respectively).


Patients with Fontan circulation at a higher altitude have impairment in aerobic capacity when compared with patients at sea level. Reduction in exercise capacity is associated with a reduction in stroke volume, likely related to increased pulmonary vascular resistance.

Original Articles
Copyright © Cambridge University Press 2010

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These authors contributed equally to the formation of the manuscript and are considered co−first authors.


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