Myocardial edema in paroxysmal permeability disorders: The paradigm of Clarkson's disease.

摘要: Abstract Purpose Paroxysmal Permeability Disorders (PPDs) comprise a variety of diseases characterized by recurrent and transitory increase endothelial permeability. Idiopathic Systemic Capillary Leak Syndrome (ISCLS) is rare PPD that leads to an abrupt massive shift fluids proteins from the intravascular interstitial compartment. In some cases, tissue edema may involve myocardium, but its role in development shock has not been elucidated so far. Materials methods Assessment cardiac involvement during ten life-threatening ISCLS episodes admitted ICU. Results Transthoracic echocardiographic examination was performed eight episodes, whereas poor acoustic window prevented ultrasound assessment two episodes. Myocardial detected echocardiography marked pericardial effusion one-episode. Cardiac magnetic resonance showed diffuse myocardial another episode. one case, caused fulminant left ventricular dysfunction, which required extracorporeal life support. The mean septum thickness higher phase compared recovery [15.5 mm (13.1–21 mm) vs. 9.9 mm (9–11.3 mm), p = .0003]. resolved within 72 h. Conclusions During early phases ISCLS, commonly occurs can induce transient potentially contributing pathogenesis shock.