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Heparin-induced thrombocytopenia is a life-threatening immune-mediated platelet activation condition that can cause arterial and venous thromboembolism. The triggering complex, platelet factor 4/heparin antibody, has several unique immunologic characteristics that have not been well elucidated until recently. In patients undergoing cardiovascular procedures such as percutaneous coronary intervention and coronary artery bypass graft surgery, the prevalence of platelet factor 4/heparin antibody is significantly higher than that in the general population. The acuity and graveness of the thromboembolic phenomenon requires early diagnosis and empirical initiation of treatment, even before confirmatory test results are available. Also, although multiple therapeutic modalities exist, the safety and efficacy of each option depends upon the clinical setting. Therefore, this review will focus on the updated pathophysiology of heparin-induced thrombocytopenia, new diagnostic criteria, and the various treatment options for cardiovascular patients with different conditions.