Objective: To explore the prognostic value of esophageal dilatation in connective tissue disease with pulmonary arterial hypertension (CTD-PAH). Methods: A total of 170 patients with CTD?PAH diagnosed by echocardiography were collected from the Departments of Rheumatology, the First Affiliated Hospital of Nanjing Medical University from 2010 to 2022. Clinical characteristics, echocardiographic parameters, Chest CT parameters and survival of patients with non-esophageal dilatation and esophageal dilatation were compared. Kaplan?Meier and Cox methods were used for survival analysis. Esophageal dilatation is defined by coronal diameter of >9mm of the esophagus lumen at the infra-aortic portion of the esophagus. Results: The mortality of patients with esophageal dilatation was higher than that of patients without esophageal dilatation. The overall 1?, 3? and 5?year survival rates of patients with non-esophageal dilatation and esophageal dilatation were 100%, 95.2%, 84.8% and 93.6%, 73.0%, 62.3%. After adjustment for age, sex and disease duration, resting esophageal dilatation remained an independent risk factor for prognosis of patients with CTD ?PAH. Conclusion：Esophageal dilatation may be one of the indexes to predict the long?term poor prognosis of patients with CTD?PAH.