Both X-linked and autosomal dominant EDMD patients risk not only bradyarrhythmia (requiring pacemaker implant) but also atrial fibrillation/flutter, which often anticipates atrial standstill and can cause disabling embolic stroke at a relatively young age. Antithromboembolic prophylaxis has to be recommended in EDMD patients with atrial fibrillation/flutter or atrial standstill. With careful monitoring, survival after pacemaker implant may be long. Heart failure, which seems to occur only in a minority of patients, may be severe.
To date, aspirin desensitization is employed with patients with nonsteroidal anti-inflammatory drugs (NSAIDs) exacerbated respiratory diseases (NERD) or with aspirin or NSAIDs hypersensitive patients needing a stent procedure for coronary artery disease. On the other hand, few data exist regarding aspirin desensitization in other cardiological features and particularly we haven’t data on different NSAIDs desensitization. Only for NERD patients we have data on ketorolac use. We report an efficacious desensitization procedure for ibuprofen in urticaria/angioedema patient with pericarditis and myocarditis associated.
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