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Long-term survival with parenteral prostacyclin therapy in pulmonary hypertension - Insights from a referral center in Portugal
Session:
SESSÃO DE POSTERS 14 - CONGÉNITOS E HTP 2
Speaker:
Débora Repolho
Congress:
CPC 2025
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:
Cartazes
FP Number:
---
Authors:
Débora Repolho; Filipa Ferreira; Ana Sofia Alegria; Ana Claudia Vieira; Barbara Ferreira; João Luz; Sofia Tavares; Helder Pereira
Abstract
<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Aptos,sans-serif"><strong>Introduction</strong><br /> <span style="background-color:white"><span style="font-family:"Arial",sans-serif"><span style="color:#2e2e2e">Therapy with parenteral prostacyclin analogues in patients with pulmonary arterial hypertension (PAH) has been established for decades and is an integral component of the current guidelines for the treatment of pulmonary hypertension.</span></span></span> However, there are few studies that report long-term experience of its use. Its administration form represents a significant burden either with subcutaneous or intravascular administration. </span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Aptos,sans-serif"><strong>Objective</strong><br /> To report the 20 years’ experience with parenteral prostacyclin therapy of a Portuguese pulmonary hypertension treatment center.</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">A retrospective longitudinal observational study that included all patients followed in single pulmonary hypertension clinic from 2002 who were treated with prostacyclin analogs, administered through subcutaneous and/or intravascular routes. Primary outcome was lung transplantation and death from any cause. The Kaplan-Meier method was employed for survival analysis.</span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Aptos,sans-serif"><strong>Results</strong><br /> 47 patients were included, with 66% diagnosed with PAH and 34% with chronic thromboembolic pulmonary hypertension (CTEPH). Baseline at diagnosis World Health Organization (WHO) Functional Class (FC) II comprised 12.8%, FC III accounted for 36.2%, and FC IV represented 51.1%. The cohort was predominantly female (78.7%), with a mean age of 45±16 years. Therapeutic options included treprostinil in 34%, epoprostenol in 38.3%, iloprost in 14.9%, while 12.8% alternated between two drugs. 76.6% of patients were adherent to the theapeutic regimen. The longest follow-up period was 21 years. Median time from diagnosis to the initiation of prostacyclin treatment was 0.4 years (IQR: 3.05 years). Seventeen patients (40%) started parenteral prostacyclins as upfront therapy. Twenty-two (46,8%) patients died: 77,2% due to heart failure, 9% sudden death, and 13,6% from other causes. Two patients (4.2%) underwent lung transplantation. </span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Aptos,sans-serif">Kaplan-Meier survival analysis estimated an overall mean transplant-free survival of 10.4 ± 1.5 years, with a median of 8.0 years (IQR: 6 years). The transplant-free survival rates at 1, 3, and 6 years were 82.1%, 72.2%, and 55.8%, respectively (Figure 1A). A sub-analysis was conducted on patients who survived the first year: Kaplan-Meier survival analysis estimated an overall mean transplant-free survival of 12.6 ± 1.6 years, with a median of 10 years (IQR: 7 years). The transplant-free survival rates at 1, 3, and 6 years were 91.2%, 80.7%, and 63.1%, respectively (Figure 1B). Epoprostenol demonstrated the best individual transplant-free survival, at 9.86 ± 2.25 years, with a median of 10 years (IQR: 10 years) (Figure 1C).</span></span></p> <p><strong><span style="font-size:11.0pt"><span style="font-family:"Aptos",sans-serif">Conclusions</span></span></strong><br /> <span style="font-size:11.0pt"><span style="font-family:"Aptos",sans-serif">Our experience with long-term parenteral prostanoid therapy demonstrate good overall survival free from transplant.</span></span></p>
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