Objective：To explore the relationship between resting heart rate and long-term prognosis of patients with connective tissue disease-associated pulmonary arterial hypertension（CTD-PAH）. Methods：A total of 89 patients with CTD-PAH diagnosed by right cardiac catheterization were collected from the Departments of Rheumatology，the First Affiliated Hospital of Nanjing Medical University from 2009 to 2021. The clinical characteristics，two-dimensional echocardiographic parameters，hemodynamic parameters and survival of patients with heart rate < 100 beats/min and heart rate ≥100 beats/min were compared. Kaplan-Meier and Cox methods were used for survival analysis. Results：There was no significant difference in sex，age，WHO functional class，6-minute walking distance，N-terminal pro-brain natriuretic peptide，two-dimensional echocardiographic and hemodynamic parameters between the two groups. The mortality of patients with resting heart rate ≥100 beats/min was higher than that of patients with resting heart rate <100 beats/min（P < 0.01）. The overall 1-，3- and 5-year survival rates of patients with resting heart rate < 100 beats/min and ≥ 100 beats/min were 98.6%，90.7%，87.6% and 93.8%，66.8%，55.6%. After adjustment for age and sex， resting heart rate ≥100 beats/min remained an independent risk factor for prognosis of patients with CTD-PAH. Conclusion：The resting heart rate may be one of the indexes to predict the long-term poor prognosis of patients with CTD-PAH and may reflect the overall function of the right ventricle.