Dr. Philippe Charron,
This session was perfectly in line with the spotlight of the 2013 ESC congress “Heart interacting with systemic organs”.
Prof. Denis Duboc (Paris, France) discussed the issues related to the management of inherited neuromuscular disorders, often seen first by neurologists. The speaker reviewed the three most frequent inherited diseases: dystrophinopathies (especially the better prevention of myocardial dysfunction thanks to ACE inhibitors in children with Duchenne muscular dystrophy), Emery-Dreifuss muscular dystrophy (with early indication for pacemaker and ICD implantation) and Steinert myotonic dystrophy (particularly the impact of electrophysiology testing/early pacemaker).
Prof. Alida Caforio (Padua, Italy) presented an excellent review of the various immune-related diseases that are often first seen by internal medicine colleagues. She detailed the diagnostic clues and main therapeutics of diseases such as Churg-Strauss disease, Wegener’s granulomatosis, systemic lupus erythematosus, polymyositis/dermatomyositis, and sarcoidoisis.
Prof. Petar Seferovic (Belgrade, Serbia) described the various endocrine diseases that may present myocardial complications (thyroid diseases, acromegaly, pheochromocytoma, carcinoid syndrome) with a special emphasis on diabetic cardiomyopathy, a still mysterious clinical entity, despite numerous investigations (prevalence of diastolic dysfunction: about 10 percent in his large experience, role of cardiotoxicity of free fatty acid).
Finally, Prof. Michael Arad (Ramat Gan, Israel) gave a talk on metabolic diseases that are most often encountered by paediatricians. He underlined the importance of performing metabolic investigations in children with a cardiomyopathy in order to appropriately manage the therapeutics (often specific) and genetic counselling.
General diagnostic clues for these diseases are: cardiomyopathy + particular ECG (abnormal conduction defect, pre-excitation, large QRS voltage) + multisystemic involvement. The major diseases to search for are Pompe disease, PRKAG2 disease, Danon disease, mitochondrial disease.
Session Title: Dilated cardiomyopathies in systemic disorders
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