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Cardiopulmonary adaptation to short-term high altitude exposure in adult Fontan patients
  1. Roger Staempfli1,
  2. Jean-Paul Schmid1,
  3. Stephan Schenker2,
  4. Prisca Eser1,
  5. Lukas D Trachsel1,
  6. Christina Deluigi1,
  7. Kerstin Wustmann2,
  8. Corina Thomet2,
  9. Matthias Greutmann3,
  10. Daniel Tobler4,
  11. Dominik Stambach5,
  12. Matthias Wilhelm1,
  13. Markus Schwerzmann2
  1. 1Department of Cardiology, Preventive Cardiology and Sports Medicine, Inselspital, University Hospital, University of Bern, Bern, Switzerland
  2. 2Department of Cardiology, Center for Congenital Heart Disease, Inselspital, University Hospital, University of Bern, Bern, Switzerland
  3. 3Department of Cardiology, University Heart Center, Cardiology, Zurich, Switzerland
  4. 4Department of Cardiology, University Hospital, Basel, Switzerland
  5. 5Department of Cardiology, Kantonsspital, St. Gallen, Switzerland
  1. Correspondence to Professor Markus Schwerzmann, Department of Cardiology, Center for Congenital Heart Disease, Inselspital, Kinderklinik C817, University Hospital, Bern 3010, Switzerland; markus.schwerzmann{at}


Objective High altitude-related hypoxia induces pulmonary vasoconstriction. In Fontan patients without a contractile subpulmonary ventricle, an increase in pulmonary artery pressure is expected to decrease circulatory output and reduce exercise capacity. This study investigates the direct effects of short-term high altitude exposure on pulmonary blood flow (PBF) and exercise capacity in Fontan patients.

Methods 16 adult Fontan patients (mean age 28±7 years, 56% female) and 14 matched controls underwent cardiopulmonary exercise testing with measurement of PBF with a gas rebreathing system at 540 m (low altitude) and at 3454 m (high altitude) within 12 weeks.

Results PBF at rest and at exercise was higher in controls than in Fontan patients, both at low and high altitude. PBF increased twofold in Fontan patients and 2.8-fold in the control group during submaximal exercise, with no significant difference between low and high altitude (p=0.290). A reduction in peak oxygen uptake at high compared with low altitude was observed in Fontan patients (22.8±5.1 and 20.5±3.8 mL/min/kg, p<0.001) and the control group (35.0±7.4 and 29.1±6.5 mL/min/kg, p<0.001). The reduction in exercise capacity was less pronounced in Fontan patients compared with controls (9±12% vs 17±8%, p=0.005). No major adverse clinical event was observed.

Conclusions Short-term high altitude exposure has no negative impact on PBF and exercise capacity in Fontan patients when compared with controls, and was clinically well tolerated.

Trial registration number NCT02237274: Results.

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