SPC365

Real-World Early Risk Response to Upfront Parenteral Prostacyclins Pulmonary Hypertension

Session: Sessão de Posters 58 - Otimizar os cuidados cardiovasculares: perspetivas de enfermagem e de técnicos de cardiopneumologia
Speaker: Débora Repolho

Congress: CPC 2026
Topic: F. Valvular, Myocardial, Pericardial, Pulmonary, Congenital Heart Disease
Theme: 21. Pulmonary Circulation, Pulmonary Embolism, Right Heart Failure
Subtheme: 21.4 Pulmonary Circulation, Pulmonary Embolism, Right Heart Failure - Treatment
Session Type: Posters Eletrónicos
FP Number: ---
Authors: Débora Repolho; Filipa Ferreira; Sofia Alegria; Otilia Simões; Barbara Ferreira; Catarina Abreu; Silvia Vitorino; Paloma Ferreira; Helder Pereira

<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Aptos,sans-serif"><strong>Introduction</strong></span></span></p>

<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Aptos,sans-serif">Pulmonary hypertension (PH) is a progressive and life-threatening disease in which risk stratification is essential to guide therapy and assess prognosis. Validated multiparametric models such as REVEAL and ESC/ERS 2022 are now central to evaluating clinical evolution and treatment response. Guidelines recommend reassessment 3&ndash;4 months after treatment escalation or every 3&ndash;6 months in stable patients</span></span></p>

<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Aptos,sans-serif"><strong>Objectives</strong><br />
To evaluate changes in global risk profile from baseline to 6 months in patients starting upfront combination therapy including parenteral prostacyclin analogues, and to determine the proportion achieving low-risk status. A secondary aim was to assess agreement between validated risk scores</span></span></p>

<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Aptos,sans-serif"><strong>Methods</strong></span></span></p>

<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Aptos,sans-serif">Retrospective observational study including consecutive patients with confirmed pre-capillary PH who initiated upfront combination therapy incorporating parenteral prostacyclin (intravenous or subcutaneous). Clinical, laboratory, functional, echocardiographic and hemodynamic data were collected at baseline and 6 months. Baseline risk was assessed using REVEAL 2.0 (R2) and ESC/ERS 2022 (E/E); 6-month reassessment used REVEAL Lite 2 (RL) and COMPERA (C). Wilcoxon signed-rank and Spearman correlation were applied</span></span></p>

<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Aptos,sans-serif"><strong>Results</strong></span></span></p>

<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Aptos,sans-serif">Twelve patients were included (mean age 46 &plusmn; 14 years, 75% female). 66.7% had chronic thromboembolic PH and 33.3%&nbsp;had pulmonary arterial hypertension. Only one patient received upfront triple therapy. The remaining patients started dual therapy targeting the nitric oxide pathway. Parenteral prostanoid therapy consisted mainly of epoprostenol (91.6%), with one patient treated with treprostinil (8,4%).&nbsp; At baseline, 75% were in WHO Functional Class IV. Detailed clinical, laboratory, functional, echocardiographic and hemodynamic evolution is presented in supplementary table. Baseline risk assessment showed a mean R2 score of 12.0 &plusmn; 1.7 and a mean E/E score of 2.5 &plusmn; 0.1. Three patients died in the first 6 months (mortality of 25%): one from right ventricular failure. Among the remaining nine survivors, 6-month risk reassessment showed substantial improvement, with a mean RL score of 3.3 &plusmn; 2.0, and a mean C score of 1.2 &plusmn; 0.1. All survivors showed a reduction in risk category with 77.7% achieving a low-risk status (RL score) </span></span></p>

<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Aptos,sans-serif">Correlation between R2 and E/E scores was strong at baseline (&rho; = 0.746, p = 0.005) and remained strong at 6months when comparing RL with C (&rho; = 0.797, p = 0.010). Changes in individual patient risk status are illustrated in the Sankey diagram (Figure 1)</span></span></p>

<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Aptos,sans-serif"><strong>Conclusion</strong></span></span></p>

<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Aptos,sans-serif">Our study demonstrates that upfront combination therapy including parenteral prostanoids resulted in marked early clinical improvement, with 77.7% of survivors achieving low-risk status at 6 months. Strong agreement between validated risk scores was observed at baseline and maintained during follow-up, supporting their complementary use in risk-guided management</span></span></p>