A patient with Eisenmenger Syndrome (congenital heart disease with secondary pulmonary hypertension and right-to-left shunt) may be severely affected by travel to a high altitude.
Possible consequences of travel to high altitude:
(1) worsening pulmonary hypertension due to hypoxic pulmonary vasoconstriction, with greater right-to-left shunting
(2) acute right heart failure
(3) severe exercise restriction
(4) cerebrovascular accident
(5) hemoptysis
(6) worsening of polycythemia
(7) ventricular arrhythmia with sudden death
(8) thromboembolism
Pre-travel evaluation:
(1) Cancel the trip to altitude if possible.
(2) Correct any iron deficiency prior to departure (microcytic erythrocytes are less deformable and are associated with a higher blood viscosity).
(3) Make sure sufficient oxygen supplies are available.
(4) Arrange for emergency evacuation if necessary.
(5) Make sure that no exertion is planned.
(6) Consider anticoagulation.
Management:
(1) Provide supplemental oxygen.
(2) Monitor oxygen saturation with a pulse oximeter if possible.
(3) Prevent dehydration.
(4) Monitor for signs of clinical deterioration, hypotension and/or heart failure.
(5) Consider isovolumic phlebotomy. especially if there are signs of hyperviscosity.
(6) Avoid drugs that could cause peripheral vasodilatation.
(7) Keep any exertion to an absolute minimum.
NOTE: A patient with Eisenmenger syndrome who travels in a commercial airplane with pressurization usually does quite well provided supplemental oxygen is available and no sudden depressurization occurs.
Specialty: Emergency Medicine, Critical Care, Pulmonology, Neurology