Le cardiologue et le syndrome d'Ehlers-Danlos (SED)

People with Ehlers-Danlos syndrome consult often the cardiologist for cardiovascular symptoms that are usual and sometimes disturbing but most often not serious in this connective tissue disorder that is common but poorly understood. Dysautonomia has a very important place in these symptoms. This is mainly arrhythmias with tachycardia often painful crises that may involve risk of coronary episode. They contrast with frequent bradycardia at rest. There is also orthostatic hypotension (POTS), vasomotor disorders realizing Raynaud's syndrome array without capillaroscopic test, signs of venous and lymphatic insufficiency with oedema accessible to veinous contentions, and lymphatic drainage and pressure therapy. Dyspnea is very frequent and respiratory blockages too. The origin is a respiratory dysfunction by lack of information from the sensors in a relatively unreactive connective tissue (proprioceptive disorders). Elsewhere are discrete valvular alterations detected by a heart murmur and echocardiography. They have no hemodynamic consequence and no oslérian risk. The arterial aneurysm is a complication to look systematically at least by echography of the entire arterial tree and not only of the aorta, each time the SED diagnosis is made. They are numerous in some forms tell “vascular”, also characterized by a particular fragility and internal organs detectable in 61% of cases by studying the COL3A1 gene.