PT  - JOURNAL ARTICLE
AU  - X-Q Xu
AU  - Z-C. Jing
TI  - High-altitude pulmonary hypertension
AID  - 10.1183/09059180.00011104
DP  - 2009 Mar 01
TA  - European Respiratory Review
PG  - 13--17
VI  - 18
IP  - 111
4099  - http://err.ersjournals.com/content/18/111/13.short
4100  - http://err.ersjournals.com/content/18/111/13.full
SO  - EUROPEAN RESPIRATORY REVIEW2009 Mar 01; 18
AB  - High-altitude pulmonary hypertension (HAPH) is a specific disease affecting populations that live at high elevations. The prevalence of HAPH among those residing at high altitudes needs to be further defined. Whereas reduction in nitric oxide production may be one mechanism for the development of HAPH, the roles of endothelin-1 and prostaglandin I2 pathways in the pathogenesis of HAPH deserve further study. Although some studies have suggested that genetic factors contribute to the pathogenesis of HAPH, data published to date are insufficient for the identification of a significant number of gene polymorphims in HAPH. The clinical presentation of HAPH is nonspecific. Exertional dyspnoea is the most common symptom and signs related to right heart failure are common in late stages of HAPH. Echocardiography is the most useful screening tool and right heart catheterisation is the gold standard for the diagnosis of HAPH. The ideal management for HAPH is migration to lower altitudes. Phosphodiesterase 5 is an attractive drug target for the treatment of HAPH. In addition, acetazolamide is a promising therapeutic agent for high-altitude pulmonary hypertension. To date, no evidence has confirmed whether endothelin-receptor antagonists have efficacy in the treatment of high-altitude pulmonary hypertension.