Management of Liver Transplantation in a Pediatric Patient With Heterotaxy Syndrome and Bradyarrhythmia: A Case...

Heterotaxy syndrome is a rare congenital disorder characterized by abnormal arrangement of thoracoabdominal organs. Associated cardiovascular and visceral malformations pose significant anesthetic and surgical challenges during major procedures such as liver transplantation.

An eight-month-old female (6.2 kg) with extrahepatic biliary atresia and heterotaxy syndrome was scheduled for living donor liver transplantation. She had situs ambiguous, dextrocardia, right aortic arch, bilateral superior vena cava, and polysplenia. Persistent bradycardia (heart rate 50 bpm) was unresponsive to atropine, with long pauses on Holter monitoring. Isoprenaline infusion maintained a heart rate of 90 bpm preoperatively. On the day of surgery, induction was achieved with fentanyl, propofol, and atracurium. Transvenous pacing via internal jugular veins was unsuccessful due to anatomical variations; hence, left femoral access was used for pacing and central line placement. Intraoperative anesthesia with sevoflurane, fentanyl, and atracurium ensured stability during the 12-hour procedure. Postoperatively, the patient required re-intubation on day 2 for sepsis, which was managed with antibiotics. The pacemaker was removed on day 6 after stable rhythm with isoprenaline and oral orciprenaline. She recovered well and was discharged from ICU on day 20.

Successful anesthetic management in heterotaxy syndrome requires detailed preoperative imaging, individualized pacing strategy, and vigilant intraoperative monitoring. Multidisciplinary coordination is key to achieving favorable outcomes in pediatric liver transplantation with complex congenital anomalies.