Altitude exposure in pediatric pulmonary hypertension-are we ready for (flight) recommendations?

Patients with congenital heart disease are surviving further into adulthood and want to participate in multiple activities. This includes exposure to high altitude by air travel or recreational activities, such as hiking and skiing. However, at an altitude of about 2,500 m, the barometric environmental pressure is reduced and the partial pressure of inspired oxygen drops from 21% to 15% (hypobaric hypoxia). In physiologic response to high-altitude-related hypoxia, pulmonary vasoconstriction is induced within minutes of exposure followed by compensatory hyperventilation and increased cardiac output. Even in healthy children and adults, desaturation can be profound and lead to a significant rise in pulmonary pressure and resistance. Individuals with already increased pulmonary pressure may be placed at risk during high-altitude exposure, as compensatory mechanisms may be limited. Little is known about the physiological response and risk of developing clinically relevant events on altitude exposure in pediatric pulmonary hypertension (PAH). Current guidelines are, in the absence of clinical studies, mainly based on expert opinion. Today, healthcare professionals are increasingly faced with the question, how best to assess and advise on the safety of individuals with PAH planning air travel or an excursion to mountain areas. To fill the gap, this article summarises the current clinical knowledge on moderate to high altitude exposure in patients with different forms of pediatric PAH.