Perioperative Management of Immune Thrombocytopenic Purpura in Cardiac Surgery: A Systematic Review of the Literature in Urgent and Elective Settings

Abstract

Perioperative management of immune thrombocytopenic purpura (ITP) in cardiac surgery represents a rare yet formidable clinical challenge driven by immune-mediated platelet dysfunction, obligatory systemic anticoagulation, and the profound hemostatic derangements induced by cardiopulmonary bypass. We systematically synthesized the available evidence on perioperative strategies and outcomes in adults with ITP undergoing cardiac surgery in elective and urgent or emergent settings by reviewing PubMed/MEDLINE, Embase, Scopus, Web of Science, Cochrane CENTRAL, ClinicalTrials.gov, and the WHO International Clinical Trials Registry Platform from inception through August 26, 2025. Nineteen studies comprising approximately 74 patients, predominantly with severe thrombocytopenia, were identified, including three observational cohorts and sixteen case reports or series. Reported management approaches centered on rapid immunomodulation with intravenous immunoglobulin and/or corticosteroids, selective adjunctive platelet transfusion, antifibrinolytic therapy, thrombopoietin receptor agonists in selected cases, and carefully monitored cardiopulmonary bypass anticoagulation, most commonly using unfractionated heparin with activated clotting time guidance; bivalirudin was described in isolated reports. Across studies, perioperative bleeding occurred in approximately 32% of patients, platelet transfusion in 45%, reoperation for bleeding in 8%, thrombotic complications in 6%, and perioperative mortality in 7%, with consistently inferior outcomes observed in urgent and emergent procedures. Although the evidence base remains largely case-derived and heterogeneous, these data support a coherent multimodal perioperative strategy integrating rapid platelet-directed immunotherapy, patient blood management-guided hemostasis, antifibrinolytic use, and vigilant anticoagulation, while underscoring the urgent need for prospective, multicenter registries with harmonized outcome definitions to inform standardized, evidence-based perioperative pathways for this high-risk population.