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Objective:  To extend our knowledge on tolerance of acute high-altitude exposure and hemodynamic response to exercise in adolescents with congenital heart disease (AscCHD) without meaningful clinical or functional restriction.  Methods:  A symptom limited cardiopulmonary exercise stress test and a non-invasive cardiac output measurement during steady state exercise were performed at 540 m and at 3454 m a.s.l. Symptoms of acute mountain sickness were noted.  Results:  We recruited 21 healthy controls and 16 AscCHD (59% male, mean age 14.7 ± 1.1 years). Three subjects (2 controls, 1 AscCHD) presented light symptoms of acute mountain sickness (dizziness and headache). During the symptom limited exercise test at lowland, control subjects showed a significantly higher power to weight index (3.5 ± 0.6 W/kg  vs . 3.0 ± 0.7 W/kg,  p  &lt; 0.001), heart rate (188.8 ± 10.4 1/min  vs . 179.4 ± 13.1 1/min,  p  &lt; 0.050) and ventilation (92.8 ± 22.9 l/min  vs . 75.4 ± 18.6 l/min, &lt;0.050). At altitude, power to weight index only remained significantly higher in the control group (2.8 ± 0.6 W/kg  vs . 2.6 ± 0.6 W/kg,  p  &lt; 0.001). Pulmonary blood flow (PBF) at lowland showed no difference between the control and the AscCHD group, neither at rest (5.4 ± 0.8 l/min  vs . 5.1 ± 0.9 l/min,  p  = 0.308), nor during the steady state test (10.6 ± 2.4 l/min  vs . 10.5 ± 2.0 l/min,  p  = 0.825). At high altitude, PBF increased by 110% and 112%, respectively (12.8 ± 2.32 l/min  vs . 12.5 ± 3.0 l/min; intergroup difference:  p  = 0.986).  Conclusions:  High altitude exposure was well tolerated in an unselected group of AscCHD. No significant difference in the cardio-pulmonary adaptation to a control group was noted during a steady state exercise. Symptoms of minor acute mountain sickness did occur, which should however not be misinterpreted as signs of hemodynamic maladaptation.

Cardiopulmonary Response to Exercise at High Altitude in Adolescents with Congenital Heart Disease