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

Area of Science:

• Congenital heart surgery
• Pediatric cardiology
• High-altitude medicine

Background:

• Ebstein anomaly necessitates early surgical intervention to prevent right ventricular (RV) volume overload.
• Right ventricular dysfunction can be exacerbated at high altitudes.
• No prior surgical results for Ebstein anomaly from high-altitude settings have been reported.

Purpose of the Study:

• To report the initial postoperative outcomes of Ebstein anomaly patients undergoing cone reconstruction at high altitude (>2,500m).
• To evaluate a specific high-altitude protocol for prophylactic management of pulmonary hypertension (PH).

Main Methods:

• Cone reconstruction of the tricuspid valve was performed in four pediatric patients with Ebstein anomaly.
• A high-altitude protocol including sildenafil, iloprost, and higher FiO2 was used to manage presumed pulmonary hypertension.
• One patient required a "one and a half ventricle" repair due to intraoperative complications.

Main Results:

• All four patients survived the surgery and were alive at 1-year follow-up.
• Patients demonstrated good or improved RV function with minimal-to-mild tricuspid regurgitation.
• The high-altitude protocol was effective in managing pulmonary hypertension during the perioperative period.

Conclusions:

• Tricuspid valve cone reconstruction is a viable surgical option for pediatric Ebstein anomaly in high-altitude environments.
• A dedicated high-altitude protocol is crucial for managing pulmonary hypertension and ensuring favorable surgical outcomes.
• This study presents the first reported surgical results for Ebstein anomaly from a high-altitude setting.