Ventricular tachycardia as the first manifestation of Churg-Strauss syndrome.
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ABSTRACT:
SUBMITTER: Budanova M
PROVIDER: S-EPMC6283746 | BioStudies | 2017-01-01
REPOSITORIES: biostudies
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Project description:BACKGROUND:Myocardial infarction is most commonly caused by thrombosis occurring on a background of coronary atherosclerosis, resulting in reduced coronary flow. Less often, myocardial infarction can occur in the absence of coronary disease. The pathomechanism of myocardial infarction in such patients is heterogeneous and more challenging to diagnose and treat. European Society of Cardiology published a position paper on myocardial infarction in patients with non-obstructive coronary disease, with definitions and recommendations for investigations, in what has hitherto been an under-recognized and under-investigated Cinderella-like condition. However, the importance of obtaining a diagnosis is all the more important, since one treatment approach with revascularization and antithrombotic treatment does not 'fit all'. CASE SUMMARY:A 70-year-old male patient presented with chest pain at rest, associated with rise in troponin and without ECG changes. A diagnosis of non-ST elevation myocardial infarction was made. Coronary angiography showed a smooth stenosis which resolved with administration of intracoronary nitrate. A diagnosis of coronary artery spasm was made, and treatment initiated. After 18 months, the patient had recurrent chest pains at rest, unresponsive to glyceryl trinitrate (GTN). Cardiac magnetic resonance revealed extension of subendocardial infarction, without inducible ischaemia. CT coronary angiogram (CTCA) showed non-obstructive coronaries. Blood tests showed significant eosinophilia, raised troponin, and C-reactive protein (CRP) that fluctuated without correlation with symptoms or any ECG changes. A diagnosis of Churg-Strauss syndrome was made, and immunosuppression commenced. DISCUSSION:Churg-Strauss syndrome is an autoimmune vasculitis in patients with history of atopy or late-onset asthma which when involving coronary arteries can lead to myocardial injury mimicking acute coronary syndrome (ACS). Identification is important to allow initiation of immunosuppression which can prevent development or progression.
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Project description:Previous studies suggest a role for eotaxin-3, TARC/CCL17 and IgG4 in newly-diagnosed patients with eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss) with highly active disease. The role of these biomarkers in relapsing disease is unclear.Serum levels of TARC/CCL17, eotaxin-3, IgG4, and IgG4/IgG ratio were determined in serum samples from a longitudinal cohort of patients with EGPA (105 visits of 25 patients). Epidemiological, clinical and laboratory data were available for all visits.At the first visit, 80% of patients were using glucocorticoids and 68% additional immunosuppressive drugs. Disease flares were seen at 18 visits. The median BVAS and BVAS/WG scores at time of relapse were 4 and 2, respectively. None of the biomarkers tested were useful to discriminate between active disease and remission. Patients treated with prednisone had lower eotaxin-3 and eosinophil levels compared to patients not taking glucocorticoids irrespective of disease activity. Use of immunosuppressive agents was not associated with biomarker levels.Serum levels of TARC/CCL17, eotaxin-3, IgG4, and IgG4/IgG ratio do not clearly differentiate active and inactive disease in established EGPA. Defining biomarkers in EGPA remains a challenge especially during times of glucocorticoid use.
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Project description:Background:Primary cardiac fibroma is exceedingly rare. This condition involves a significant risk of life-threatening arrhythmias during follow-up and its prognosis is not as favourable as other benign tumours. We report a case of cardiac fibroma that was preoperatively diagnosed with echocardiography and magnetic resonance imaging. This fibroma was excised early as a preventative measure to avoid sudden death. Case summary:A 46-year-old woman presented to our hospital with a 1-year history of chest tightness at rest. Echocardiography showed a large, isoechoic, well-circumscribed mass within the left ventricular myocardium with calcified tissue. Magnetic resonance imaging showed an intramural ventricular mass with iso signal intensity on T1-weighted imaging and low-signal intensity on T2-weighted imaging. There was no enhancement on first-pass perfusion imaging and homogeneous hyperenhancement on late gadolinium enhancement imaging. These features suggested a diagnosis of cardiac fibroma. Complete resection was performed to avoid sudden death and pathological analysis confirmed the tumour as cardiac fibroma. The patient was discharged 9?days after surgery and remains disease-free 5?months after surgery. Discussion:Cardiac fibroma is a pathologically benign tumour with an unfavourable prognosis because of lethal arrhythmias, which can be controlled by its resection. Thus, it is important to preoperatively distinguish cardiac fibroma from other benign tumours, in order to prioritize surgical intervention for those with cardiac fibromas. Preoperative diagnosis with echocardiography and magnetic resonance imaging and early preventative surgery are the keys to improve prognosis of patients with cardiac fibromas.
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