• Matthias Müller, Florian Lurz, Thomas Zajonz, Fabian Edinger, Uygar Yörüker, Josef Thul, Dietmar Schranz, and Hakan Akintürk.
• Pediatric Cardiac Anesthesiology Service, Pediatric Heart Centre, Department of Anesthesiology Intensive Care Medicine, Pain Therapy, University Hospital Giessen and Marburg GmbH, Giessen, Germany.
• Paediatr Anaesth. 2024 Dec 1; 34 (12): 122312301223-1230.

BackgroundPatients with hypoplastic left heart syndrome undergo the comprehensive stage 2 procedure as the second stage in the hybrid approach toward Fontan circulation. The complexity of comprehensive stage 2 procedure is considered a potential limitation, and limited information is available on its anesthetic management. This study aims to address this gap.MethodsA single-center retrospective cohort study analyzed 148 HLHS patients who underwent comprehensive stage 2 procedure, divided into Group A (stable condition, n = 116) and Group B (requiring preoperative intravenous inotropic therapy, n = 32). Demographic data, intraoperative hemodynamics, anesthetic management, and postoperative outcomes were collected.ResultsEtomidate (40%) was the most common induction agent, followed by esketamine (24%), midazolam (16%), and propofol (13%). Inhaled induction was rarely necessary (2%), occurring only in Group A patients. No statistical differences were found between groups for induction drug choice. Post-cardiopulmonary bypass management included moderate hypoventilation, inhaled nitric oxide (100%), and hemodynamic support with milrinone (97%) and norepinephrine (77%). Group B patients more frequently required additional levosimendan (20%) and epinephrine (18%). Extracorporeal membrane oxygenation was necessary in 8 patients (5%) with no between-group differences. Switching from fentanyl to remifentanil reduced postoperative ventilation time overall. However, Group B experienced significantly longer ventilation (6.3 vs. 3.5 h) and ICU stay (22 vs. 14 days). In-hospital mortality was 5% overall (Group A: 4%, Group B: 9%). Long-term survival analysis revealed a significant advantage for Group A.ConclusionThe use of short-acting opioids and adjusted ventilation modes enables optimal pulmonary blood flow and rapid transition to spontaneous breathing. Differentiated hemodynamic support with milrinone, norepinephrine, supplemented by levosimendan and epinephrine in high-risk patients, can mitigate the effects on the preoperatively volume-loaded right ventricle. However, differences in long-term survival probability were observed between groups.Trial RegistrationLocal ethics committee, Medical Faculty, Justus-Liebig-University-Giessen (Trial Code Number: 216/14).© 2024 The Author(s). Pediatric Anesthesia published by John Wiley & Sons Ltd.

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