Abstract

We report a peculiar case of a 77 years woman affected by chronic thromboembolic pulmonary hypertension (CTEPH) with surgical indication to pulmonary endarterectomy (PEA) but with the concomitant presence of anti-PF4-heparin autoantibodies. Coagulation disorders are frequently associated in patients with CTEPH and therefore full thrombophilic pattern evaluation is completed as standard preoperative protocol in all our patients. In this case, history of chronic thrombocytopenia induced us to extend preoperative evaluation to the presence of anti-PF4-heparin autoantibodies. Once heparin-induced thrombocytopenia was documented, an alternative approach rather than standard cardiopulmonary bypass with systemic heparinization was mandatory. Due to the characteristics of surgical procedure (i.e., prolonged CPB time, possible risk of intraoperative and postoperative bleeding), we decided to adopt plasmapheresis and immunoglobulin infusion before surgery, thus allowing the standard use of heparin in CPB. The patient underwent a successful PEA without complications. Hemodynamic and clinical results are satisfactory after 3 years of follow-up.

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