High-Altitude Travel in Patients With Neuromuscular Disease and Thoracic Restrictive Disorders: A Narrative Review

Patients with neuromuscular disease and other thoracic restrictive disorders are at increased risk of hypoxia during high-altitude travel due to an ineffective hypoxic ventilatory response. Efforts to identify clinical parameters that successfully predict the need for hypoxic challenge testing, a tool used to assess the risk of hypobaric hypoxia, and its outcomes in this population have been inconsistent and limited mainly to small, retrospective studies. Consequently, recommendations regarding the evaluation and management of these patients prior to high-altitude travel have been uncertain and differ across medical societies. We provide a clinical management algorithm for this patient population that draws on the available data.

Patients should be assessed for contraindications to high-altitude travel prior to undergoing a safety screen that incorporates travel history, pulmonary comorbidities, pulmonary function test parameters, and measures of oxygenation and ventilation. Hypoxic challenge testing is recommended for those who do not pass the safety screen. Resolving any contraindications to high-altitude travel or barriers to before flight testing should be prioritized to preserve the quality of life of these patients.

Prospective studies are needed to identify and validate pulmonary function testing parameters and exercise testing outcomes that may be predictive of hypoxic challenge test failure, as well as potentially new hypoxic challenge testing protocols that incorporate noninvasive ventilation titration. Such endeavors will add more clarity to an area of medicine that has many uncertainties and important implications for the well-being of a vulnerable patient population.