Pediatrics. 2021 Jun;147(6). doi: 10.1542/peds.2020-021808.

The Heart of the Matter: Secondary Hypogammaglobulinemia and Constrictive Pericarditis.

Pediatrics

Erica G Schmitt, Aarti S Dalal, Alok Kothari, Maleewan Kitcharoensakkul

PMID: 34049957 DOI: 10.1542/peds.2020-021808

Abstract

Constrictive pericarditis is the final common result of a number of processes that affect the pericardium. Establishing the diagnosis and determining the underlying etiology of constrictive pericarditis are often a diagnostic rendezvous. Here, we describe a patient who presented to the general practitioner with edema, ascites, and weight gain and was found to have constrictive pericarditis secondary to an inflammatory myofibroblastic tumor of the mediastinum. Interestingly, she had a relative lack of cardiorespiratory complaints, and, aside from the edema and mildly elevated jugular venous pressure, she had an unremarkable cardiac and pulmonary examination. During the diagnostic evaluation for constrictive pericarditis, she was found to have hypogammaglobulinemia and profound lymphocytopenia. A stool α-1-antitrypsin level was sent and was elevated, which confirmed the diagnosis of protein-losing enteropathy, a rare but important complication of constrictive pericarditis. This case highlights important diagnostic considerations and management of these complications for the general practitioner.

Copyright © 2021 by the American Academy of Pediatrics.

Conflict of interest statement

Publication Types

• Journal Article