Objective To evaluate the prognostic value of pulmonary hypertension (PH) in newly diagnosed patients with systemic immunoglobulin light-chain amyloidosis (AL) with normal left ventricular ejection fraction. Methods The data of the first-time AL patients admitted to The Second Affiliated Hospital of Naval Medical University (Second Military Medical University) from Jun. 2014 to Dec. 2021 were analyzed retrospectively. The inclusion criteria were:all patients were confirmed with AL by biopsy and pathology and had normal left ventricular ejection fraction; and patients with congenital heart disease, moderate and severe chronic obstructive pulmonary disease and other causes of PH were excluded. According to the estimation of pulmonary artery pressure by transthoracic echocardiography, the patients were divided into PH group and non-PH group. Kaplan-Meier method was used to calculate overall survival (OS) and progress-free survival (PFS) rates, and the survival curves were drawn. Univariate and multivariate Cox proportional hazards regression models were used to analyze the effects of PH on survival and prognosis. Results Finally, 93 AL patients were enrolled, including 17 patients in the PH group and 76 patients in the non-PH group. The median follow-up time was 19.4 (1.0-87.0) months, during which 33 patients died. The median OS time of patients was 20.9 months in the PH group and not available in the non-PH group, and the median PFS time was 10.5 months and 47.0 months, respectively, showing significant differences (both P<0.05). After adjusting the baseline data, the multivariate Cox regression analysis showed that PH was an independent risk factor affecting the survival and prognosis of AL patients. The risk of death in patients with PH was 4.93 times higher than that in patients without PH (hazard ratio[HR]=4.93, 95% confidence interval[CI]1.41-17.23, P=0.012 4); the risk of disease progression in patients with PH was 3.53 times higher than that in patients without PH (HR=3.53, 95% CI 1.22-10.20, P=0.019 6). Among AL patients with cardiac infiltration, patients with PH had a higher risk of death and disease progression (HR=13.23, 95% CI 2.35-74.93, P=0.003 4; HR=3.58, 95% CI 1.09-11.71, P=0.035 3). Conclusion Echocardiographic assessment of PH has important prognostic value for AL patients with normal left ventricular ejection fraction, which should be considered in clinical practice.