Early risk prediction in idiopathic <i>versus</i> connective tissue disease-associated pulmonary arterial hypertension: call for a refined assessment

Despite systematic screening and improved treatment strategies, the prognosis remains worse in patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) compared to idiopathic/hereditary (IPAH). We aimed investigate differences clinical characteristics, outcome, performance of ESC/ERS risk stratification tool these patient groups. This retrospective analysis included incident CTD-PAH (n=197, which 64 had interstitial lung disease, ILD) or IPAH (n=305) enrolled Swedish PAH Register 2008–2019. Patients were classified as low, intermediate, high at baseline, according “SPAHR-equation”. 1-year survival, stratified by type PAH, was investigated Cox proportional regression. At lower diffusing capacity for carbon monoxide haemoglobin, but, same time, N-terminal prohormone-brain natriuretic peptide, longer 6 min walking distance, better hemodynamics, more often a low-risk profile. No difference age, WHO-FC, renal function between groups found. survival rates 75, 82 83%, ILD, without IPAH, respectively. The mortality low-, intermediate-, high-risk whole cohort 0, 18 34% (p&lt;0.001), Corresponding percentages were: 26, 67% (p=0.008); 19, 39% (p=0.004); 16, 29% (p=0.001), assessment accurately identified but underestimated rate assessed having intermediate diagnosis.