Open Access

CASE REPORT

An Unusual Cause of Irritability in a Single Ventricle Patient after Bidirectional Glenn Shunt

by Sarah Pradhan^1,*, Eileen Broomall², Russel Hirsch¹

1 The Heart Institute, Cincinnati Children’s Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA
2 Division of Neurology, Cincinnati Children’s Hospital Medical Center, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA

* Corresponding Author: Sarah Pradhan. Email:

Congenital Heart Disease 2021, 16(4), 393-396. https://doi.org/10.32604/CHD.2021.015257

Received 04 December 2020; Accepted 01 February 2021; Issue published 19 April 2021

Abstract

The differential diagnosis for irritability in children is broad. In patients with congenital heart disease, one must strongly consider cardiac etiologies such as low cardiac output or elevated central venous pressure (CVP). In patients with single-ventricle physiology, the second stage of palliation includes bidirectional Glenn, which involves anastomosis of the superior vena cava to the pulmonary artery resulting in volume offloading of the single systemic ventricle. Typically, early in the post-operative period, patients may experience a headache due to the acute increase in CVP, and symptoms improve over time. Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is a rare neurologic disorder in children, characterized by raised intracranial pressure (ICP) in the absence of brain parenchymal lesions or cerebrospinal fluid (CSF) abnormalities. While the pathogenesis of IIH is unknown, early recognition and treatment of IIH are important to prevent permanent vision loss. There are only rare reports of IIH in patients with Fontan circulation. To our knowledge, we report the first case of IIH in a 2-year-old female after bidirectional Glenn.

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