Aims Novel therapies are needed for recurrent pericarditis, particularly when corticosteroid dependent and colchicine resistant. Based on limited data, interleukin-1 blockade with anakinra may be beneficial. The aim of this multicentre registry was to evaluate the broader effectiveness and safety of anakinra in a ‘real world’ population. Methods and results This registry enrolled consecutive patients with recurrent pericarditis who were corticosteroid dependent and colchicine resistant and treated with anakinra. The primary outcome was the pericarditis recurrence rate after treatment. Secondary outcomes included emergency department visits, hospitalisations, corticosteroid use and adverse events. Among 224 patients (46 ± 14 years old, 63% women, 75% idiopathic), the median duration of disease was 17 months (interquartile range 9–33). Most patients had elevated C-reactive protein (91%) and pericardial effusion (88%). After a median treatment of 6 months (3–12), pericarditis recurrences were reduced six-fold (2.33–0.39 per patient per year), emergency department admissions were reduced 11-fold (1.08–0.10 per patient per year), hospitalisations were reduced seven-fold (0.99–0.13 per patient per year). Corticosteroid use was decreased by anakinra (respectively from 80% to 27%; P < 0.001). No serious adverse events occurred; adverse events consisted mostly of transient skin reactions (38%) at the injection site. Adverse events led to discontinuation in 3%. A full-dose treatment duration of over 3 months followed by a tapering period of over 3 months were the therapeutic schemes associated with a lower risk of recurrence. Conclusion In patients with recurrent pericarditis, anakinra appears efficacious and safe in reducing recurrences, emergency department admissions and hospitalisations.
Imaging in blunt thoracic trauma: The importance of clinical correlation
We present the case of a 26‐year‐old female restrained front‐seat passenger who presents following a motor vehicle accident, with CT angiogram features suggestive of possible acute aortic injury. However, clinical features including relative hemodynamic stability and absence of typical symptoms were discordant with these imaging findings. This case illustrates that even with ECG‐gating, CT angiogram artifact mimicking acute aortic injury may still occur. Careful evaluation and clinical correlation is of vital importance, both to ensure acute aortic injury is not missed and that patients are not erroneously sent for aortic surgery when there is no aortic injury. Careful clinical evaluation must be combined with imaging in all cases of suspected aortic trauma, and at times multimodality imaging is indicated to direct the decision making strategy.
Contained right atrial rupture: an unusual presentation of a rare primary cardiac tumour
Advanced imaging techniques, including contrast echocardiography and CMR, provided valuable characterization and evaluation of a boy with an unusual cardiac mass. Imaging features assisted with differentiation of benign from malignant etiology and excluding thrombus. Accurate imaging techniques saved our patient risks associated with unnecessary surgery or anticoagulation. The ability to accurately define size serially assisted in guiding expectant management. He died from a noncardiac cause at age 28, and autopsy demonstrated a hamartoma of mature cardiac myocytes.
Previously Unreported in Women
Galactosidase Alpha
Pro409Ser Variant Is Associated With Fabry Disease
Objective To determine the prevalence and outcomes of intracranial aneurysm (IA) in patients with bicuspid aortic valve (BAV). Methods Retrospective review of patients with BAV who underwent brain MR angiography at the Mayo Clinic from 1994 to 2013. Results There were 678 patients included in this study—mean age 57±13 years, men 480 (71%), mean follow-up 10±3 years (5913 patient-years). Coarctation of aorta (COA) was present in 154 (23%) patients. There were 59 IAs identified in 52 of 678 patients (7.7%). IA was present in 20/154 patients (12.9%) with COA and 32/524 patients (5.7%) without COA (p<0.001). For the patients without COA, female gender and right–left cusp fusion were risks factors for IA in women after adjustment for all potential variables (HR 1.76, CI 1.31 to 2.68, p=0.03). There was no significant trend in the risk for IA across age tertiles: age ≤40 years versus 41–60 years (HR 1.19, p=0.34), and age 41–60 years versus 61–80 years (HR 1.06, p=0.56). Among the 52 patients with IA, enlargement occurred in three patients (6%), rupture in two patients (4%) and four patients (8%) underwent coil embolisation. For the 626 patients without IA at baseline, no patient developed IA over 7±2 years of imaging follow-up. Conclusions BAV is associated with a higher prevalence of IA compared to the general population, and this risk is higher in patients with COA, right–left cusp fusion and female gender.
Commentary on: Savall F, Dedouit F, Telmon N, Rouge D. Takotsubo cardiomyopathy in a jeweller following a hold‐up: forensic considerations. J Forensic Sci 2013;58:1648–50.