Human immunodeficiency virus- (HIV-) related pulmonary arterial hypertension (PAH) is a rare complication of HIV infection. The pathophysiology of HIV-related PAH is complex, with viral proteins seeming to play the major role. However, other factors, such as coinfection with other microorganisms and HIV-related systemic inflammation, might also contribute. The clinical presentation of HIV-related PAH and diagnosis is similar to other forms of pulmonary hypertension. Both PAH-specific therapies and HAART are important in HIV-related PAH management. Future studies investigating the pathogenesis are needed to discover new therapeutic targets and treatments. 1. Introduction Pulmonary hypertension (PH) is a group of disorders characterized by a mean pulmonary arterial pressure (mPAP) ≥25？mmHg during right heart catheterization [1]. The most recent PH classification includes five main groups, which are shown in Table 1. The disease is called pulmonary arterial hypertension (PAH) when classified as group 1 PH and is called PH when the etiologic factor fits into other groups (groups 2–5). The association of PH with other medical disorders generally imparts a worse prognosis. For example, the presence of PH in patients with chronic obstructive pulmonary disease (COPD) is linked to greater morbidity and mortality [2]. More detailed discussion of PH is beyond the scope of this paper and can be found elsewhere [3, 4]. Table 1: Classification of PH. Human immunodeficiency virus (HIV), which belongs to the lentivirus subgroup of the retrovirus family, became known after describing cases of pneumocystis pneumonia and Kaposi sarcoma in 1981 [5, 6]. It is believed that at least 35 million people are infected with HIV worldwide [7]. HIV infection, if left untreated, can lead to a profound decline in host immunological function which in turn predisposes to a myriad of infectious diseases [8, 9] and malignant conditions [10]. Furthermore, recent evidence suggests that patients with HIV are at greater risk of cardiovascular disease and kidney disease, even after adjustment for conventional risk factors [11, 12]. Further discussion of HIV-related topics is far beyond the scope of this manuscript and can be easily found elsewhere. The goal of this paper is to summarize the current knowledge on HIV-related PAH, which belongs to group 1 PH (see Table 1). First, we will discuss the epidemiology of HIV-related PAH. Second, clinical features of PAH will be reviewed. Third, studies assessing prognostic factors and HIV-related PAH morbidity and mortality will be reviewed. Fourth,
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