Abstract

Background: Idiopathic pulmonary arterial hypertension (IPAH), a subtype of World Health Organization (WHO) Group I pulmonary hypertension (PH), is characterized by elevated pulmonary vascular resistance (PVR) and right ventricular (RV) dysfunction in the absence of an identifiable cause1. Patients with IPAH are considered at high perioperative risk, but outcome data in this population remain limited2.

Methods: We conducted a retrospective case series including 36 adult IPAH patients under follow-up at a Belgian national reference center. These patients underwent 61 elective non-cardiac procedures between 1992 and 2023. Diagnosis was confirmed by right heart catheterization (RHC)3. Preoperative cardiac index (CI) was categorized into high, intermediate, or low risk using European Society of Cardiology (ESC) and European Respiratory Society (ERS) 2022 criteria4. The primary outcome was 30-day all-cause mortality. Secondary outcomes included 90-day mortality, unplanned intensive care unit (ICU) admission, and prolonged hospitalization. Associations with baseline hemodynamic parameters were explored descriptively.

Results: Thirty-day mortality was 1.6% (1/61); 90-day mortality was 4.9% (3/61). Descriptive analysis showed that all deaths occurred in patients with low CI and elevated PVR5. Unplanned ICU admission occurred in 6.6% (4/61), and prolonged hospitalization in 18.0% (11/61). No statistically significant associations were found between baseline hemodynamics and adverse outcomes.

Conclusion: Despite modern disease-targeted therapies, patients with IPAH remain vulnerable to perioperative complications and delayed mortality6. Adverse events occurred predominantly in patients with high-risk hemodynamic profiles or procedures performed in non-expert centers7. Structured preoperative risk stratification and referral to experienced centers may improve perioperative outcomes in this population.

Ethical approval: Ethics Committee Research UZ/KU Leuven (S64278; Chair: Prof. Dr. L. Helsen; 18/12/2023).