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Case Reports
. 2024 Dec 17:2024:8283566.
doi: 10.1155/cric/8283566. eCollection 2024.

Intraoperative Hemodynamic Instability in a Patient With Ebstein's Anomaly Complicated With Eisenmenger Syndrome

Leonardo A Marquez Roa  1 , Jorge Araujo-Duran  1 , Richard Hofstra  1 , Jibran Ikram  1 , Sabry Ayad  1
Affiliations
Case Reports

Intraoperative Hemodynamic Instability in a Patient With Ebstein's Anomaly Complicated With Eisenmenger Syndrome

Leonardo A Marquez Roa et al. Case Rep Cardiol. .

Abstract

Ebstein's anomaly is a rare congenital displacement of the tricuspid valve resulting in atrialization of the right ventricle. About half of the patients with Ebstein's anomaly also have atrial septal defects, which may lead to chronic shunting and development of Eisenmenger syndrome. We describe a case of a sexagenarian male patient with a history of Ebstein's anomaly complicated with Eisenmenger syndrome undergoing robotic laparoscopic adrenalectomy who presented hemodynamic instability, hypoxemia, and likely right-to-left shunting intraoperatively, as well as the actions taken to correct it and have a successful outcome. Perioperative management of adult patients with congenital heart defects is complex and requires careful monitoring. When available, intraoperative transesophageal echocardiography is strongly recommended. To prevent right-to-left shunting, maintaining elevated systemic vascular resistance with the use of vasopressors and low positive end-expiratory pressure (PEEP) ventilation is critical.

Keywords: Ebstein's anomaly; Eisenmenger syndrome; intraoperative hemodynamic instability; intraoperative hypoxemia; positive end-expiratory pressure (PEEP); right-to-left shunting; transesophageal echocardiography (TEE).

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Conflict of interest statement

The authors declare no conflicts of interest.

Figures

Figure 1
Figure 1
Midesophageal four-chamber view of TEE evidencing an apical displacement of 2.93 cm of the septal leaflet of the tricuspid valve (double-headed arrow). RA: right atrium; RV: right ventricle; SL: septal leaflet of the tricuspid valve; AL: anterior leaflet of the tricuspid valve.
Figure 2
Figure 2
Midesophageal four-chamber view of TEE showing severe dilation of the right ventricle compared to the left ventricle. RV: right ventricle; LV: left ventricle.
Figure 3
Figure 3
Midesophageal bicaval view of TEE showing atrial septal defect of 1.59 cm (arrow). RA: right atrium; LA: left atrium; IAS: interatrial septum.
See this image and copyright information in PMC

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