Early Detection of Pulmonary Arterial Hypertension in Connective Tissue Disease: A Single-Center Retrospective Study

This study aimed to identify indicators for the early diagnosis and management of pulmonary arterial hypertension (PAH) in patients with connective tissue disease (CTD).

This retrospective study included patients with CTD who met the criteria for right heart catheterization (RHC) according to contemporary guidelines for pulmonary hypertension (PH) with an intermediate to high echocardiographic probability of PH at a medical center in Taiwan. The data collected was analyzed.

This study enrolled 92 patients, comprising 21 with systemic sclerosis (SSc) and 71 without SSc (non-SSc). PH and PAH were diagnosed in 67 (72.8%) and 55 (59.8%) patients, respectively, and 41 (44.6%) patients required PAH-specific medications. A high echocardiographic probability of PH significantly predicted both PH and PAH in all patients (adjusted odds ratio [OR] 7.30, 95% confidence interval [CI] 2.34-22.76 and OR 6.02, 95% CI 1.65-21.93), as well as in the non-SSc subgroup (adjusted OR 20.51 [4.41-95.45] and 15.95 [2.62-96.92]). N-terminal pro-B-type natriuretic peptide (NT-proBNP) ≥ 300 pg/mL predicted PH in the overall cohort and non-SSc subgroup (adjusted OR 3.55 [1.15-10.96] and 5.22 [1.15-23.66]). Pericardial effusion was associated with PH and PAH in the overall cohort (adjusted OR 11.99 [1.35-106.62] and 11.13 [1.23-101.07]) but not in the non-SSc subgroup. None of the aforementioned parameters significantly associated with the need for PAH-specific medications.

A high echocardiographic probability of PH and NT-proBNP ≥ 300 pg/mL predict PH in CTD patients. Pericardial effusion may further aid in PH/PAH detection, though not in the non-SSc subgroup. RHC remains essential to confirm treatment eligibility.