The European Rare Disease Network for HHT Frameworks for management of hereditary haemorrhagic telangiectasia in general and speciality care.

Hereditary haemorrhagic telangiectasia (HHT) is a complex, multisystemic vascular dysplasia affecting approximately 85,000 European Citizens. In 2016, eight founding centres operating within 6 countries, set up a working group dedicated to HHT within what became the European Reference Network on Rare Multisystemic Vascular Diseases. By launch, combined experience exceeded 10,000 HHT patients, and Chairs representing 7 separate specialties provided a median of 24 years' experience in HHT. Integrated were expert patients who focused discussions on the patient experience. Following a 2016-2017 survey to capture priorities, and underpinned by more than 40 monthly meetings, and new data acquisitions, VASCERN HHT generated position statements that distinguish expert HHT care from non-expert HHT practice. Leadership was by specialists in the relevant sub-discipline(s), and 100% consensus was required amongst all clinicians before statements were published or disseminated. One major set of outputs targeted all healthcare professionals and their HHT patients, and include the new Orphanet definition; Do's and Don'ts for common situations; Outcome Measures suitable for all consultations; COVID-19; and anticoagulation. The second output set span aspects of vascular pathophysiology where greater understanding will assist organ-specific specialist clinicians to provide more informed care to HHT patients. These cover cerebral vascular malformations and screening; mucocutaneous telangiectasia and differential diagnosis; anti-angiogenic therapies; circulatory interplays between anaemia and arteriovenous malformations; and microbiological strategies to counteract loss of normal pulmonary capillary function. Overall, the integrated outputs, and documented current practices, provide frameworks for approaches that augment the health and safety of HHT patients in diverse health-care settings.

Global impact of the COVID-19 pandemic on subarachnoid hemorrhage

Introduction: During the COVID-19 pandemic, decreased volumes of stroke admissions and mechanical thrombectomy were reported. The objective was to examine whether subarachnoid hemorrhage(SAH) hospitalizations and ruptured aneurysm coiling interventions demonstrated similar declines. Methods: We conducted a global, retrospective, observational study across 6 continents, 37 countries, and 140 comprehensive stroke centers. Patients with diagnosis of SAH, ruptured aneurysm coiling interventions, COVID-19 were identified using ICD-10 codes or by prospectively maintained stroke databases. The 3-month cumulative volume, monthly volumes for SAH hospitalizations and ruptured aneurysm coiling procedures were compared for the period before (1- year and immediately before) and during the COVID-19 pandemic (March 1 to May 31, 2020). Results: There was a significant decline in SAH hospitalizations with 2,044 admissions in the 3 months immediately before and 1,585 admissions during the pandemic, representing a decline of 22.5% (95%CI, -24.3 to -20.7, p<0.0001). Embolization of ruptured aneurysms declined with 1,170 to 1,035 procedures, respectively, representing an 11.5%(95%CI, -13.5 to -9.8, p=0.002) drop. Hospitals with higher COVID-19 hospitalization burden demonstrated greater declines in SAH and ruptured aneurysm coiling compared to lower COVID-19 burden. A relative increase in coiling of ruptured aneurysms was noted in low coiling volume hospitals of 41.1% (95%CI, 32.3-50.6, p=0.008) despite a decrease in SAH admissions in this tertile. Conclusions: There was a global decrease in subarachnoid hemorrhage admissions and ruptured aneurysm embolizations during the COVID-19 pandemic. Among low-volume coiling SAH hospitals, there was an increase in the ruptured aneurysm coiling intervention. These findings in SAH are consistent with a global decrease in other emergencies such as stroke and myocardial infarction.