• Journal of critical care · Jun 2020

    Observational Study

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

    • Maddalena A Wu, Emanuele Catena, Chiara Cogliati, Davide Ottolina, Antonio Castelli, Roberto Rech, Tommaso Fossali, Sonia Ippolito, Antonio L Brucato, and Riccardo Colombo.
    • Department of Internal Medicine, ASST Fatebenefratelli Sacco, "Luigi Sacco" Hospital - Polo Universitario - University of Milan, Via G.B. Grassi 74, 20157 Milan, Italy.
    • J Crit Care. 2020 Jun 1; 57: 13-18.

    PurposeParoxysmal Permeability Disorders (PPDs) comprise a variety of diseases characterized by recurrent and transitory increase of endothelial permeability. Idiopathic Systemic Capillary Leak Syndrome (ISCLS) is a rare PPD that leads to an abrupt massive shift of fluids and proteins from the intravascular to the interstitial compartment. In some cases, tissue edema may involve the myocardium, but its role in the development of shock has not been elucidated so far.Materials And MethodsAssessment of cardiac involvement during ten life-threatening ISCLS episodes admitted to ICU.ResultsTransthoracic echocardiographic examination was performed in eight episodes, whereas a poor acoustic window prevented cardiac ultrasound assessment in two episodes. Myocardial edema was detected by echocardiography in eight episodes and marked pericardial effusion in one-episode. Cardiac magnetic resonance showed diffuse myocardial edema in another episode. In one case, myocardial edema caused fulminant left ventricular dysfunction, which required extracorporeal life support. The mean septum thickness was higher during the shock phase compared to the recovery phase [15.5 mm (13.1-21 mm) vs. 9.9 mm (9-11.3 mm), p = .0003]. Myocardial edema resolved within 72 h.ConclusionsDuring early phases of ISCLS, myocardial edema commonly occurs and can induce transient myocardial dysfunction, potentially contributing to the pathogenesis of shock.Copyright © 2020 Elsevier Inc. All rights reserved.

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