A Noninvasive Prognostic Model for Pulmonary Arterial Hypertension Associated With Connective Tissue Diseases Based...

Patients with connective tissue diseases (CTDs) and pulmonary arterial hypertension (PAH) have a poor prognosis, and there is a lack of effective noninvasive prognostic tools. This study aimed to retrospectively analyze clinical data and multislice computed tomography (MSCT) chest CT parameters in CTD-PAH patients, and to develop a noninvasive prognostic model incorporating indicators.

A total of 170 patients with CTD-PAH admitted to the First Affiliated Hospital of Nanjing Medical University between May 2010 and April 2022 were enrolled in this study. Data on chest computed tomography-derived pulmonary artery diameters, esophageal dilatation, and interstitial lung disease (ILD) scores were collected. Patients were followed for 5 years to assess all-cause mortality. A nomogram incorporating MSCT parameters was developed and validated to predict the long-term prognosis.

Independent risk factors for 5-year all-cause mortality in CTD-PAH patients included main pulmonary artery diameter (MPAd) (HR: 1.109, 95% CI: 1.010-1.218, P =0.030*), esophageal dilatation (HR: 2.757, 95% CI: 1.220-6.230, P =0.015*), and ILD score (HR: 1.066, 95% CI: 1.019-1.114, P =0.005*). A threshold MPAd of >35.70 mm was associated with a worse prognosis. The nomogram model, with a score >125, predicted a significantly lower 5-year survival rate in CTD-PAH patients.

MPAd, esophageal dilatation, and ILD score are independent risk factors for 5-year all-cause mortality in CTD-PAH patients. The nomogram, which integrates these MSCT parameters, provides a reliable noninvasive tool for predicting reduced 5-year survival, offering valuable prognostic insight for personalized management of CTD-PAH.