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Partial Pericardial Agenesis Mimicking Arrhythmogenic Right Ventricular Cardiomyopathy

Castelletti, Silvia, MD*; Crotti, Lia, MD, PhD*,†,‡; Dagradi, Federica, MD*; Rella, Valeria, MD; Salerno, Sabrina, MD; Parati, Gianfranco, MD†,‡; Cecchi, Franco, MD

doi: 10.1097/JSM.0000000000000733
Case Report: PDF Only

Abstract: Absence of the pericardium is a rare congenital disease in which the fibroserum membrane covering the heart is partially or totally absent. It is characterized by few echocardiography (ECG) and imaging features that can mislead the diagnosis to an inherited cardiac disease, such as arrhythmogenic right ventricular cardiomyopathy. Although it has often a benign course, this congenital defect should be identified as in some cases herniation and strangulation can be life-threatening and cause sudden cardiac death. Red flags on ECG (sinus bradycardia, variable T-wave inversion), chest x-ray (Snoopy sign, absence of tracheal deviation, and esophagus impression), and transthoracic echocardiogram (unusual windows, teardrop left ventricle, and elongated atria) should rise the suspicion of pericardium absence. The correct diagnosis, confirmed by cardiac magnetic resonance, is mandatory as the consequences on the sport activity certification, the management, and the treatment are extremely different.

*Istituto Auxologico Italiano, IRCCS, Center for Cardiac Arrhythmia of Genetic Origin, Milan, Italy;

Department of Cardiovascular, Neural and Metabolic Sciences, Istituto Auxologico Italiano, IRCCS, San Luca Hospital, Milan, Italy; and

Department of Medicine and Surgery, University of Milano-Bicocca, Milan, Italy.

Corresponding Author: Silvia Castelletti, MD, Istituto Auxologico Italiano, IRCCS, Center for Cardiac Arrhythmia of Genetic Origin, 20135 Milan, Italy (

The authors report no conflicts of interest.

Received March 15, 2018

Accepted December 21, 2018

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