The following are key points to remember from this article on the diagnosis and management of cerebral venous sinus thrombosis (CVST) with vaccine-induced thrombotic thrombocytopenia:

1. CVST is a rare manifestation of cerebrovascular disease. It usually affects young adults (mean age 35-40 years), most often women of childbearing age.
2. Risk factors for CVST in general are similar to those for venous thromboembolism and include pregnancy, exposure to certain medications (including oral contraceptives and chemotherapy), central nervous system or ear/facial infections, and head trauma.
3. Thrombocytopenia is an uncommon primary cause of CVST.
4. Coronavirus disease 2019 (COVID-19) infection is a risk factor for CVST (without thrombocytopenia). One retrospective analysis identified an incidence of 39.0 per million people (95% confidence interval [CI], 25.2-60.2) after COVID-19 infection as compared to the 2-week period before COVID-19 infection (0.41 per million people).
5. Signs and symptoms of CVST are diverse and may mimic many other neurological disorders. Presentation often includes: 1) isolated headache or increased intracranial pressure (present in ~90% of cases), 2) focal neurological presentations, 3) subacute encephalopathy, and 4) cavernous sinus syndrome/multiple cranial neuropathies.
6. The combination of CVST and thrombocytopenia had been identified in six cases following the Janssen/Johnson & Johnson (Janssen) COVID-19 vaccine at the time the article was submitted. Similar presentations of CVST and thrombocytopenia have been reported in Europe following the AstraZeneca COVID-19 vaccine. Both of these vaccines are adenoviral vectors.
7. Symptoms related to CVST and thrombocytopenia have occurred between 6-13 days after receipt of the Janssen COVID-19 vaccine. They have been reported between 5-24 days after the first dose of AstraZeneca COVID-19 vaccine in Europe.
8. No cases of CVST and thrombocytopenia have been reported following the mRNA COVID-19 vaccines from either Pfizer or Moderna.
9. The combination of CVST and thrombocytopenia following COVID-19 vaccine is named vaccine-induced thrombotic thrombocytopenia (VITT). It is also being referred to as the thrombotic and thrombocytopenia syndrome (TTS).
10. Diagnosis includes imaging (either magnetic resonance or computed tomography with venogram), complete blood count with platelet count, coagulation studies, D-dimer, fibrinogen, and platelet factor 4 (PF4) antibody enzyme-linked immunosorbent assay (ELISA).
11. Acute management should include collaboration with vascular neurology and a thrombosis expert (e.g., hematology, vascular medicine). Intravenous immunoglobulin 1 g/kg daily for 2 days has been recommended after PF4 antibody testing has been initiated. Heparin products should be avoided.
12. Anticoagulation should follow guidelines for heparin-induced thrombocytopenia (HIT), including a heparin-alternative such as argatroban, bivalirudin, fondaparinux, or a direct oral anticoagulant. Anticoagulation many be needed even in the presence of secondary intracranial hemorrhage, as it is necessary to prevent thrombus progression in order to control bleeding.
13. Future research is needed to identify the true incidence of VITT following the Janssen COVID-19 vaccine and to identify the mechanism by which this condition is occurring.

Clinical Topics: Anticoagulation Management, Congenital Heart Disease and Pediatric Cardiology, COVID-19 Hub, Noninvasive Imaging, Prevention, Pulmonary Hypertension and Venous Thromboembolism, Vascular Medicine, Anticoagulation Management and Venothromboembolism, CHD and Pediatrics and Arrhythmias, CHD and Pediatrics and Imaging, CHD and Pediatrics and Quality Improvement, Computed Tomography, Nuclear Imaging

Keywords: Anticoagulants, Cerebrovascular Disorders, Contraceptives, Oral, COVID-19, ACC COVID-19 Podcast, Craniocerebral Trauma, Enzyme-Linked Immunosorbent Assay, Fibrinogen, Headache, Hematology, Immunoglobulins, Intravenous, Intracranial Hemorrhages, Magnetic Resonance Spectroscopy, Platelet Factor 4, Pregnancy, Primary Prevention, Risk Factors, RNA, Messenger, Sinus Thrombosis, Intracranial, Stroke, Thrombocytopenia, Thrombosis, Tomography, Emission-Computed, Vascular Diseases, Venous Thromboembolism, Young Adult

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