Differential predictors of mPAP progression in Systemic Sclerosis and Non-SSc connective tissue disease patients
Y Orihara, K D Min, S Hayashi, K Shimizu, J Ohno, I Sunayama, M Sugahara, A Eguchi, M Asakura, M IshiharaAbstract
Background
Acceleration time/ejection time (AcT/ET) has been reported as a predictor of pulmonary artery pressure (PAP) progression beyond 20 mmHg in patients with connective tissue disease (CTD) and normal mean pulmonary artery pressure (mPAP). However, whether predictive factors differ between systemic sclerosis (SSc) and non-SSc CTD remains unclear.
Objective
To identify predictive factors for PAP progression exceeding 20 mmHg in patients with SSc and non-SSc CTD who initially had normal PAP.
Methods
We retrospectively analyzed CTD patients who underwent at least two transthoracic echocardiography (TTE) examinations at our institution. A total of 173 patients were included: 72 with SSc and 101 with non-SSc CTD. Mean PAP was estimated by echocardiography in all patients, and clinical and echocardiographic parameters were evaluated for their association with PAP progression.
Results
In the SSc group, mPAP increased from 17.1 mmHg to 18.9 mmHg, (p=0.001) and 29% of patients showed an elevation of mPAP >20 mmHg during median follow up period of 485 days. SSc patients with elevated mPAP had significantly lower baseline acceleration time to ejection time (AcT/ET) ratios of 0.39 compared to those without elevation (0.44) (p < 0.001). Meanwhile, in the non-SSc group, increased from 16.5 mmHg to 18.4 mmHg (p < 0.001) and 31% showed such elevation during median follow up period of 661 days. No significant difference in AcT/ET was observed. However, tricuspid annular plane systolic excursion (TAPSE) was significantly lower in patients with elevated mPAP (p = 0.035).
Conclusion
Even in CTD patients with mPAP <20 mmHg, different echocardiographic markers may be useful for predicting future mPAP elevation depending on the underlying disease. AcT/ET may be predictive in SSc, while TAPSE may be more relevant in non-SSc CTD.