ABSTRACT
Background and aims: Cerebral venous sinus thrombosis with thrombocytopenia syndrome (CVST-TTS) is a rare adverse effect of adenovirus- based SARS-CoV-2 vaccines. After the autoimmune pathogenesis of TTS was discovered, treatment recommendations were issued. The aim of this study was to evaluate if adherence to treatment recommendations was associated with lower mortality. Methods: TTS was defined according to the Brighton criteria. Cases from a prospective international CVT registry with symptom onset within 28 days of adenovirus-based SARS-CoV-2 vaccination were analysed. Treatment recommendations, following the International Society of Thrombosis and Haemostasis, included use of immunomodulation, non-heparin anticoagulants, and avoidance of platelet transfusions, unless needed for surgery. Results: Out of 178 CVT cases from 117 centres in 19 countries reported between March 29 and September 3, 2021, 95 patients fulfilled inclusion criteria. Five of 37 (14%), 13/25 (52%), and 29/33 (88%) of patients diagnosed in March, April, and from May onwards, respectively, were treated according to recommendations. Proportion of patients diagnosed in March, April, and from May onwards who received immunomodulation increased from 19/37 (51%) over 15/25 (60%) to 30/33 (90%), and the percentage of patients who were treated with heparins [26/37 (70%), 4/25 (16%), 1/33 (3%)] and platelet transfusion [15/37 (41%), 4/25 (16%), 7/33 (21%), respectively] decreased accordingly. Mortality of patients treated according to recommendations was 14/47 (30%, 95%CI 19-44%) compared to 28/48 (58%, 95%CI 44-71%) in patients not treated according to recommendations (OR 3.30, 95%CI 1.41-7.71). Conclusions: Over time, adherence to treatment recommendations improved, and mortality rate of patients with CVST-TTS decreased.
ABSTRACT
Background and aims: Cerebral venous sinus thrombosis with thrombosis with thrombocytopenia syndrome (CVST-TTS) is a serious adverse drug reaction after adenoviral SARS-CoV-2 vaccinations. CVST-TTS patients may need decompressive surgery to avoid fatal brain herniation, but despite this intervention, many CVST-TTS patients die during the initial hospital admission. Here, we describe the characteristics and outcomes of CVST-TTS patients who underwent decompressive surgery and explore predictors of mortality in CVST-TTS patients. Methods: We used data from an ongoing international registry collecting data from patients who developed CVST within 28 days of SARS-CoV-2 vaccination, reported between 29 March and 9 December 2021. TTS was defined in accordance with the Brighton Collaboration case definition. Results: Out of 97 CVST-TTS patients, 29 (30%) underwent decompressive surgery. All operated patients had an intracerebral haemorrhage before the surgery. In-hospital mortality was 19/29 (66%) in the operated and 23/68 (34%) in the non-operated group. In the operated group, the highest mortality rate was among patients who were in coma before the surgery (14/15, 93% vs 4/12, 33% in those not in coma), had bilateral absence of the pupillary response (7/7, 100% vs 8/16, 50% in patients with uni/bilateral pupillary response) and platelet count <50 x103/μL (11/14, 79% vs 6/12, 50% in cases with a platelet count ≥50 x103/μL). Conclusion: Mortality rate of CVST-TTS patients who underwent decompressive surgery is extremely high. Among the operated patients, coma before the surgery, bilateral absence of the pupillary response, and platelet count <50 x103/μL were the predictors of mortality.