The oxygen partial pressure in high-altitude areas is low. Long-term exposure to high-altitude areas leads to a state of low-pressure hypoxia. The combination of low pressure and oxygen levels triggers a variety of mechanisms that disrupt normal bodily functions. One key response to hypoxia is hypoxic pulmonary vasoconstriction, which, when aggravated, can induce the development of high altitude heart disease (HAHD). HAHD is a clinical type of chronic mountain sickness mainly characterized by vasoconstriction and hyperproliferative remodeling of the pulmonary artery. As the pressure in the pulmonary artery continues to rise, it increases the posterior load on the right heart, causing right ventricular hypertrophy. Over time, this can lead to right heart failure or even complete heart failure. Despite extensive on HAHD in recent years, its prevalence remains high. While researchers are committed to finding an ideal treatment, this remains a huge challenge, particularly as awareness of the HAHD subtype is still limited. Here, we review the recent research on the pathogenesis and treatment of HAHD, with the aim of providing new clues for its prevention and treatment.