Long-Term Outcomes of Selexipag in Schistosomiasis-Associated Pulmonary Arterial Hypertension

NCT07453030 | Not Yet Recruiting

• 1 other identifier: SGP 27907
• Study Type: observational
• Target: 30
• Locations: 1 country
• Sites: 1

Brief Summary

Schistosomiasis-associated pulmonary arterial hypertension is a serious condition that can lead to shortness of breath, heart failure, frequent hospitalizations, and early death. Although treatments for pulmonary arterial hypertension have improved over time, patients with this specific cause of the disease are often not included in long-term studies. Selexipag is an oral medication used to treat pulmonary arterial hypertension and is part of routine clinical care in Brazil. Its long-term effects in patients with schistosomiasis-associated pulmonary arterial hypertension are not well understood. The PROPULSE-Sch study aims to evaluate long-term clinical outcomes in patients with schistosomiasis-associated pulmonary arterial hypertension who received selexipag, compared with similar patients who did not receive this medication before it became available at the study center. This is an observational study using data from routine medical care. All treatments are prescribed by the treating physicians, and participation in the study does not change patient care. The results may help improve understanding of long-term outcomes and support treatment decisions in this population.

Conditions

Pulmonary Arterial Hypertension (PAH)

Keywords

Pulmonary Arterial Hypertension; Schistosomiasis; Prostacyclin Pathway; Real-World Evidence; Propensity Score Matching; Long-Term Outcomes; Observational Study

Outcome Measures

Primary Outcomes (1)

• Time to Clinical Worsening

  Time from the index date (T0) to the first occurrence of clinical worsening, defined as any of the following events: all-cause mortality; lung transplantation; non-elective hospitalization due to pulmonary arterial hypertension or right heart failure; therapeutic escalation defined as initiation of parenteral prostacyclin or addition of a new class of pulmonary arterial hypertension-specific therapy; or sustained worsening of World Health Organization functional class confirmed in two consecutive assessments at least 12 weeks apart and accompanied by objective evidence of disease progression, including a ≥15% decrease in six-minute walk distance and/or a ≥30% increase in BNP or NT-proBNP compared with baseline.

  From index date (T0) up to 36 months of follow-up

Secondary Outcomes (10)

• Clinical Improvement Composite Outcome

  6, 12, 24, and 36 months after index date

• Change in WHO Functional Class

  Up to 36 months of follow-up

• Change in 6-Minute Walk Distance (6MWD)

  Up to 36 months

• Change in BNP or NT-proBNP Levels

  Up to 36 months

• Hemodynamic Parameters

  Up to 36 months of follow-up

Study Arms (2)

Selexipag-Treated PAH-Sch Cohort

Adult patients with schistosomiasis-associated pulmonary arterial hypertension who initiated oral selexipag as part of routine clinical care. The index date (T0) is defined as the date of selexipag initiation recorded in the medical record. Patients are followed longitudinally to assess long-term clinical outcomes.

Mirror PAH-Sch Cohort Without Selexipag

Adult patients with schistosomiasis-associated pulmonary arterial hypertension who did not receive selexipag and were followed at the same center prior to its availability. The index date (T0 mirror) is defined as the first visit at which patients met clinical eligibility criteria comparable to those of the treated cohort.

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

The study population includes adult patients with schistosomiasis-associated pulmonary arterial hypertension (PAH-Sch) followed at a specialized pulmonary hypertension referral center. The population comprises two observational cohorts: patients who initiated oral selexipag as part of routine clinical care and a mirror cohort of clinically similar patients who did not receive selexipag prior to its availability at the center. All patients have confirmed pre-capillary pulmonary arterial hypertension and stable background therapy at the index date. Participants are followed longitudinally using data obtained from routine clinical practice to assess long-term clinical outcomes. No study-mandated interventions are performed.

You may qualify if:

• Adults aged 18 years or older.
• Confirmed diagnosis of pulmonary arterial hypertension associated with schistosomiasis (PAH-Sch).
• Diagnosis of pre-capillary pulmonary arterial hypertension confirmed by right heart catheterization, performed at any time prior to the index date (T0), as documented in the medical record.
• Evidence of schistosomiasis infection, including epidemiological history and ultrasonographic findings compatible with hepatosplenic schistosomiasis.
• Previous antiparasitic treatment for schistosomiasis.
• Clinical stability at the index date, defined as absence of progressive right heart failure or clinical worsening within the previous 12 weeks.
• World Health Organization (WHO) functional class I-III at the index date.
• Stable background pulmonary arterial hypertension-specific therapy with a phosphodiesterase-5 inhibitor and/or endothelin receptor antagonist for at least 12 weeks prior to the index date.
• For the treated cohort: initiation of oral selexipag as part of routine clinical care.
• For the mirror cohort: eligibility for therapeutic escalation at the index date without exposure to selexipag.

You may not qualify if:

• World Health Organization (WHO) functional class IV at the index date.
• Progressive right heart failure or clinical deterioration within the 12 weeks prior to the index date.
• Documented formal contraindication to selexipag in the medical record.

Sponsors & Collaborators

• Caio Júlio César dos Santos Fernandes: lead

Study Sites (1)

Instituto do Coração (InCor), Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo

São Paulo, São Paulo, 05403-900, Brazil

Location

Related Publications (1)

• 1. Simonneau G, Montani D, Celermajer DS, Denton CP, Gatzoulis MA, Krowka M, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. janeiro de 2019;53(1):1801913. 2. Humbert M, Sitbon O, Simonneau G. Treatment of Pulmonary Arterial Hypertension. N Engl J Med. 30 de setembro de 2004;351(14):1425-36. 3. Calderaro D, Alves Junior JL, Fernandes CJCDS, Souza R. Pulmonary Hypertension in General Cardiology Practice. Arq Bras Cardiol [Internet]. 2019 [citado 24 de agosto de 2024]; Disponível em: https://www.scielo.br/scielo.php?pid=S0066-782X2019000900419&script=sci_arttext 4. Yoo HHB. Lesões Plexiformes na Hipertensão Arterial Pulmonar: Estamos Ficando mais Próximos do Manejo com mais Paciência e Rigor? Arq Bras Cardiol. 18 de setembro de 2020;115(3):491-2. 5. D'Alonzo GE, Barst RJ, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med. 1o de setembro de 1991;115(5):343-9. 6. Barst RJ, Rubin LJ, Long WA, McGoon MD, Rich S, Badesch DB, et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. N Engl J Med. 1o de fevereiro de 1996;334(5):296-301. 7. Galiè N, Ghofrani HA, Torbicki A, Barst RJ, Rubin LJ, Badesch D, et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med. 17 de novembro de 2005;353(20):2148-57. 8. Rubin LJ, Badesch DB, Barst RJ, Galiè N, Black CM, Keogh A, et al. Bosentan Therapy for Pulmonary Arterial Hypertension. N Engl J Med. 21 de março de 2002;346(12):896-903. 9. Hoeper MM, Badesch DB, Ghofrani HA, Gibbs JSR, Gomberg-Maitland M, McLaughlin VV, et al. Phase 3 Trial of Sotatercept for Treatment of Pulmonary Arterial Hypertension. N Engl J Med. 20 de abril de 2023;388(16):1478-90. 10. Mitchell JA, Ahmetaj-Shala B, Kirkby NS, Wright WR, Mackenzie LS, Reed DM, et al. Role of pr

  RESULT

MeSH Terms

Conditions

Pulmonary Arterial Hypertension; Schistosomiasis

Condition Hierarchy (Ancestors)

Hypertension, Pulmonary; Lung Diseases; Respiratory Tract Diseases; Trematode Infections; Helminthiasis; Parasitic Diseases; Infections; Vector Borne Diseases

Study Officials

• Caio Fernandes, MD

  UNIVERSIDADE SAO PAULO

  PRINCIPAL INVESTIGATOR

Central Study Contacts

Caio Fernandes, Principal Investigator, MD

CONTACT

+551126615034; cjcfernandes@yahoo.com.br

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: OTHER
• Sponsor Type: OTHER
• Responsible Party: SPONSOR INVESTIGATOR
• PI Title: Principal investigator

Study Record Dates

• First Submitted: January 12, 2026
• First Posted: March 5, 2026
• Study Start: March 1, 2026
• Primary Completion (Estimated): March 1, 2027
• Study Completion (Estimated): March 1, 2027
• Last Updated: March 5, 2026

Record last verified: 2026-02

Data Sharing

• IPD Sharing: Will not share

Locations

BR (1)