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CC BY 4.0 · Thorac Cardiovasc Surg 2025; 73(S 03): e31-e38
DOI: 10.1055/a-2620-3643
Pediatric and Congenital Cardiology

Ebstein Repair in a High-Altitude Setting ≥2,500 m: First Experience from Bolivia

Sven C. Weber
1   Department of Congenital Heart Disease-Pediatric Cardiology, Deutsches Herzzentrum der Charité (DHZC), Berlin, Germany
,
Inge von Alvensleben
2   Kardiozentrum, La Paz, Bolivia
,
Valentin Vadiunec
3   Department of Anesthesiology, Deutsches Herzzentrum der Charité (DHZC), Berlin, Germany
,
Andre Iben
4   Department of Congenital Heart Surgery-Pediatric Heart Surgery, Deutsches Herzzentrum der Charité (DHZC), Berlin, Germany
,
Felix Berger
1   Department of Congenital Heart Disease-Pediatric Cardiology, Deutsches Herzzentrum der Charité (DHZC), Berlin, Germany
,
Hannes Sallmon
5   Division of Pediatric Cardiology, Medical University of Graz, Graz, Austria
,
Joachim Photiadis
4   Department of Congenital Heart Surgery-Pediatric Heart Surgery, Deutsches Herzzentrum der Charité (DHZC), Berlin, Germany
› Author Affiliations
Funding This project was financed by KidsHeart International (Berlin, Germany), a non-profit organization that aims to foster sustainable pediatric and congenital cardiac surgery programs in middle- and low-income regions worldwide (www.kidsheartsinternational.org).
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Abstract

Background

Contemporary surgical approaches for Ebstein anomaly are based on a paradigm shift towards earlier surgery in order to avoid the deleterious effects of chronic right ventricular (RV) volume overload. In addition, RV dysfunction may worsen in the setting of high altitude, and to date, no results on Ebstein anomaly surgery have been reported from a high-altitude setting.

Methods

We herein present first postoperative results from Ebstein anomaly patients who underwent cone reconstruction (with or without bidirectional Glenn anastomosis) in Cochabamba, Bolivia (>2,500 m above sea level) using a specific high-altitude protocol for prophylactic medical treatment of presumed pulmonary hypertension (PH), including sildenafil, iloprost, and higher FiO2.

Results

Four patients underwent surgical correction of Ebstein anomaly (median age 9 years, range 4–12 years, all female). Ebstein anomaly was classified as Carpentier type C in three and as Carpentier Type B in one patient. All patients showed some degree of atrial shunting while one patient exhibited an additional perimembranous ventricular septal defect. All underwent cone reconstruction of the tricuspid valve. Due to massive intraoperative bleeding, which required rethoracotomy, subsequently causing impaired RV function, one patient underwent concomitant “one and a half ventricle” repair. All other patients showed an uncomplicated postoperative course and all were alive with a good and/or improved RV function and only minimal-to-mild tricuspid regurgitation after 1 year.

Discussion

Cone reconstruction in children with Ebstein anomaly is feasible in a high-altitude setting when using a dedicated protocol to prophylactically manage PH.

Keywords

Ebstein anomaly - cone reconstruction - bidirectional Glenn anastomosis - high altitude - middle- and low-income regions


Publication History

Received: 12 July 2024

Accepted: 21 May 2025

Accepted Manuscript online:
26 May 2025

Article published online:
24 June 2025

© 2025. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)

Georg Thieme Verlag KG
Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany

 

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