Objective：This study aims 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 esophageal dilatation and without esophageal dilatation were compared. Kaplan-Meier and Cox methods were used for survival analysis. Esophageal dilatation is defined by coronal diameter of the esophagus lumen ＞9 mm 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 without esophageal dilatation were 100.0%，95.2%，84.8%，while the overall 1-，3- and 5-year survival rates of patients with esophageal dilatation were 93.6%，73.0%，62.3% . After adjustment for age，sex，PAH duration，and N - terminal pro - B type natriuretic peptide(NT-proBNP)，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.