Maddalena A Wu1, Emanuele Catena2, Chiara Cogliati1, Davide Ottolina2, Antonio Castelli2, Roberto Rech2, Tommaso Fossali2, Sonia Ippolito3, Antonio L Brucato4, Riccardo Colombo5. 1. Department of Internal Medicine, ASST Fatebenefratelli Sacco, "Luigi Sacco" Hospital - Polo Universitario - University of Milan, Via G.B. Grassi 74, 20157 Milan, Italy. 2. Department of Anesthesiology and Intensive Care, ASST Fatebenefratelli Sacco, "Luigi Sacco" Hospital - Polo Universitario - University of Milan, Via G.B. Grassi 74, 20157 Milan, Italy. 3. Department of Radiology, ASST Fatebenefratelli Sacco, "Luigi Sacco" Hospital - Polo Universitario - University of Milan, Via G.B. Grassi 74, 20157 Milan, Italy. 4. Department of Internal Medicine, ASST Fatebenefratelli Sacco, "Fatebenefratelli e Oftalmico" Hospital, Piazzale Principessa Clotilde 3, 20121 Milan, Italy; Department of Biomedical and Clinical Sciences, ASST Fatebenefratelli Sacco, University of Milan, Milan, Italy. 5. Department of Anesthesiology and Intensive Care, ASST Fatebenefratelli Sacco, "Luigi Sacco" Hospital - Polo Universitario - University of Milan, Via G.B. Grassi 74, 20157 Milan, Italy. Electronic address: riccardo.colombo@asst-fbf-sacco.it.
Abstract
PURPOSE: Paroxysmal 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 METHODS: Assessment of cardiac involvement during ten life-threatening ISCLS episodes admitted to ICU. RESULTS: Transthoracic 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. CONCLUSIONS: During early phases of ISCLS, myocardial edema commonly occurs and can induce transient myocardial dysfunction, potentially contributing to the pathogenesis of shock.
PURPOSE: Paroxysmal 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 METHODS: Assessment of cardiac involvement during ten life-threatening ISCLS episodes admitted to ICU. RESULTS: Transthoracic 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. CONCLUSIONS: During early phases of ISCLS, myocardial edema commonly occurs and can induce transient myocardial dysfunction, potentially contributing to the pathogenesis of shock.
Authors: Bernard Cosyns; Stijn Lochy; Maria Luiza Luchian; Alessia Gimelli; Gianluca Pontone; Sabine D Allard; Johan de Mey; Peter Rosseel; Marc Dweck; Steffen E Petersen; Thor Edvardsen Journal: Eur Heart J Cardiovasc Imaging Date: 2020-07-01 Impact factor: 6.875