High-Altitude Pulmonary Edema with Absent Right Pulmonary Artery

Abstract

High-altitude pulmonary edema potentially is fatal. Adults with unilateral absence of a right pulmonary artery are particularly susceptible to high-altitude pulmonary edema. The occurrence of high-altitude pulmonary edema was documented in a child with congenital absence of the right pulmonary artery. Improvement occurred only upon descent to low altitude. Physicians should be aware of this life-threatening condition in children ascending to high altitude, particularly in individuals with unilateral absence of a pulmonary artery.

High altitude pulmonary edema in children: A systematic review

“…Finally, 35 articles were selected and included in this systemic review (Table 1). 1,4–12,15,22–45 These articles contain data describing 210 cases of HAPE, of which 56.6% occurred in the US and 23.8% in Colombia. Table 1 details the demographic characteristics by age, sex, country, altitude, and year of publication.…”

“…Finally, 35 articles were selected and included in this systemic review (Table 1). 1,[4][5][6][7][8][9][10][11][12]15, These articles contain data describing 210 cases of HAPE, of which 56.6% occurred in the US and 23.8% in Colombia. [ [16][17][18]) was based on 66 cases where the individual age (not the means) was available (Figure 2).…”

“…The most common mentioned cut-off altitude point for the risk of developing HAPE is 2500 meters above sea level (masl), 2,3 nevertheless cases in the range from 1700 to 2500 masl has been reported in several publications. 1,[4][5][6][7][8][9][10][11] Three subtypes of HAPE are currently recognized: classic HAPE (C-HAPE) which is seen in individuals who habitually live at low altitude and travel to high altitude 7 ; re-entry HAPE (R-HAPE) which occurs in individuals who live at high altitude, travel to low altitude and come back to their home altitude 7 ; and high-altitude residents pulmonary edema (HARPE) which develops without a change in altitude in individuals who live at high altitude. 7 A fourth subtype of HAPE has been described in people who live at high altitude without handling difficulties and present the disease when traveling to even higher altitudes.…”

Absent Pulmonary Artery Presenting as High-Altitude Pulmonary Edema

“…Interestingly, HAPE is seen in approximately 10% of patients with an absent pulmonary artery. [3][4][5][6] This is thought to be caused by development of pulmonary hypertension secondary to altitude and exercise at higher elevation. 5 supplementary oxygen.…”

Update on High-Altitude Pulmonary Edema: Pathogenesis, Prevention, and Treatment

“…27 The role of increased pulmonary blood flow as an etiologic factor is supported by case reports in which the absence of the right pulmonary artery leads to the development of HAPE limited to the left lung after ascent to moderate altitudes. 19,57 In a HAPE-susceptible individual who experiences an abnormal increase in PA pressure upon rapid ascent to high altitude, this results in portions of the pulmonary capillary bed being subjected to increased hydrostatic pressures that may cause stress failure. First proposed by Hultgren, 58 the concept of ''uneven HPV'' has been demonstrated in microsphere studies by nonuniform spatial distribution of HPV in pigs and cephalization of pulmonary perfusion in HAPE-susceptible individuals.…”