Objective To study the clinical value of echocardiography combined with endothelin-1 (ET-1) in the early diagnosis of human immunodeficiency virus (HIV) infection-related pulmonary hypertension (PAH) to provide reference for clinic. Methods A total of 60 HIV-infected patients admitted to the Affiliated Hospital of Panzhihua University between December 2018 and December 2021 were selected for retrospective analysis, and the patients were divided into observation group (24 cases of PAH) and control group (36 cases without PAH) according to the presence of early PAH. The echocardiography parameters and serum ET-1 levels of the two groups were recorded, and the value of each parameter in the early diagnosis of HIV-related PAH was analyzed. Results The left ventricular end diastolic diameter (LVEDD), Tei index, right ventricular basal transverse diameter (RVd), LVEDD/RVd, mean pulmonary arterial pressure (mPAP) and serum ET-1 in the observation group were significantly higher than those in the control group, and the fractional area change (FAC) and pulmonary capillary wedge pressure (PCWP) were significantly lower than those of the control group (P<0.05). Pearson linear correlation analysis showed that PCWP were negatively correlated with Tei index, LVEDD/RVd and ET-1, and positively correlated with FAC (P<0.05). The mPAP were positively correlated with Tei index, LVEDD/RVd and ET-1, and negatively correlated with FAC (P<0.05). The area under the curve (AUC) of combined detection of serum ET-1 and LVEDD/RVd in the diagnosis of HIV related PAH was 0.907 (SE=0.054, P=0.002, 95%CI=0.769~1.000), the sensitivity was 0.944, and the specificity was 0.750. Conclusion Echocardiography LVEDD/RVd parameters combined with serum ET-1 are helpful in the diagnosis of HIV related PAH, and have high clinical application value.