BROADCASTS.com

CardioNerds (Amit and Dan) join Dr. Omid Amidi (CardioNerds Academy Graduate) and Dr. Marwah Shahid from the UCLA Cardiology Fellowship program along with Dr. Evelyn Song (CardioNerds Academy House Faculty and Heart Failure Hospitalist at UCSF) to discuss a complex case focused on management of severe coronary artery disease in a patient with Glanzmann thrombasthenia. Dr. Rushi Parikh (Interventional cardiologist, UCLA) provides the ECPR for this episode. Audio editing by\xa0CardioNerds Academy Intern, student doctor\xa0Akiva Rosenzveig.\n\n\n\nGlanzmann Thrombasthenia is a bleeding disorder due to impairment of platelet aggregation secondary to a mutation in the GPIIB/IIIA receptor. This case is focused on work up of stable coronary artery disease followed by a discussion on duration of dual antiplatelet therapy post percutaneous coronary intervention in a patient with Glanzmann thrombasthenia. \xa0\n\n\n\nCheck out this published case in JACC: Case Reports\n\n\n\n\n\nJump to: Case media - Case teaching - References \n\n\n\n\n\n\n\n\n\n\n\nCardioNerds Case Reports PageCardioNerds Episode PageCardioNerds AcademyCardionerds Healy Honor Roll\n\n\n\n\n\nCardioNerds Journal ClubSubscribe to The Heartbeat Newsletter!Check out CardioNerds SWAG!Become a CardioNerds Patron!\n\n\n\n\n\n\n\n\n\nCase Media \n\n\n\n\nSee the published case in JACC: Case Reports\n\n\n\n\n\n\n\n\nEpisode Schematics & Teaching\n\n\n\n\n\n\n\n\n\nPearls\n\n\n\n\n1. Patients with Glanzmann thrombocytopenia (GT) may have a higher risk of bleeding, depending on their disease phenotype.\n\n\n\n2. It is unclear whether the mechanism of GT protects patient against stent thrombosis in the setting of PCI. Additionally, there is little data on the use of antiplatelet agents in patients with GT.\n\n\n\n3. Short-term DAPT may be a reasonably safe option for patients with GT undergoing PCI.\n\n\n\n4. We report a successful case of percutaneous coronary intervention in a patient with GT with no complications at a 1 year follow up.\n\n\n\n\nNotes\n\n\n\n1. What is Glanzmann thrombasthenia?\n\n\n\nGT is an inherited platelet disorder that is characterized by spontaneous bleeding with phenotypic variability ranging from minimal bruising to potentially fatal hemorrhaging.\xa0 GT is caused by autosomal recessive inheritance of quantitative or qualitative deficiencies of functional \u03b1IIb\u03b23 integrin coded by ITGA2B or ITGB3 genes for \u03b1IIb and \u03b23, respectively. As a result, platelets may be stimulated, but the platelet glycoprotein IIb/IIIa receptor is unable to bind fibrinogen to cross-link platelets, rending them potentially ineffective. In platelet aggregation studies, there is lack of response to collagen, epinephrine, arachidonic acid, and ADP stimulation. Thus, platelet aggregation is impaired.\xa0\n\n\n\n2. What is known about PCI and antiplatelet therapy in the setting of Glanzmann thrombasthenia?\n\n\n\nTo the best of our knowledge, this is the first case report of percutaneous coronary intervention in the setting of GT. It is unclear if the mechanism of GT alone provides sufficient antiplatelet activity and whether antiplatelet therapy leads to significantly increased bleeding risk. The use of antiplatelet therapy is not well studied in the GT population. What we do know is that the mechanism of GT prevents platelet aggregation\u2014the final step in platelet-related thrombosis\u2014while oral antiplatelet therapy affects platelet activation, thus, in our patient we felt that short term DAPT was reasonable. It is important to note that in the event of an active bleed requiring platelet transfusion, donor platelets possess functional glycoprotein IIb/IIIa receptors and thus exponentially increase the risk of stent thrombosis. Therefore, unlike our case, if a patient is not maintained on chronic oral antiplatelet therapy, initiation of oral or intravenous antiplatelet therapy should be considered to prevent stent thrombosis at the time of platelet transfusion.\n\n\n\nLike any other patient with a high bleeding risk, it is important to have clear indications to conduct a coronary angiogram in patient...