2024 FSA Podium and Poster Abstracts
P064: ANESTHETIC MANAGEMENT FOR TETHERED CORD REPAIR IN A PATIENT WITH VACTERL AND DOUBLE OUTLET RIGHT VENTRICLE
Armish Singh1; Tam Le1; Ryan Lorenzo1; Karla Castro2; 1HCA Westside Hospital; 2Nemours Children's Hospital
Introduction: VACTERL association is a rare congenital disorder occurring in approximately 1 in 40,000 live-born infants [1], characterized by the presence of at least three congenital malformations, including vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula (TEF), renal anomalies, and limb deformities. Double outlet right ventricle (DORV) is another rare cono-truncal anomaly where both the aorta and pulmonary artery arise from the right ventricle. DORV may manifest as a standalone condition or in conjunction with other cardiac or noncardiac anomalies. It accounts for only 1%-1.5% of all congenital heart diseases [2]. Management of VACTERL typically involves immediate postnatal surgical correction of specific congenital anomalies (e.g., anal atresia, specific cardiac malformations, and/or TEF), followed by long-term medical management [1].
We present a case of an 8-year-old male with VACTERL associated imperforated anus, esophageal atresia with tracheobronchial fistula, and double outlet right ventricle with ventricular septal defect (VSD). The patient previously underwent repair of the imperforated anus, TEF, and the double outlet right ventricle, along with VSD repair and pulmonary valvotomy in 2016. Despite successful cardiac repairs, the patient developed severe pulmonary insufficiency and right heart enlargement. This case report discusses the anesthetic management for this patient who is now undergoing laminectomy with release of tethered cord, addressing the challenges posed by this rare and complex pathology.
Methods: Anesthetic management aimed to prevent hemodynamic instability and worsening pulmonary regurgitation during the tethered cord repair. Hemodynamic goals focused on minimizing factors known to increase pulmonary vascular resistance (PVR) and optimizing right ventricular function. This included minimize pain, sympathetic stimulation, hypoxia, hypercarbia, hypothermia, acidosis, and optimizing airway pressures. Milrinone was chosen as the primary inotrope, with dobutamine and vasopressin considered as alternatives.
Anesthesia induction was performed with sevoflurane, avoiding nitrous oxide due to pulmonary insufficiency. Two Peripheral IV’s and arterial line placement were established. Ketamine, propofol, and morphine were administered for intubation, followed by total intravenous anesthesia (TIVA) with propofol and remifentanil for maintenance. Care was taken to avoid excessive positive end-expiratory pressure (PEEP) during mechanical ventilation. The procedure was completed without complication, and the patient was extubated and transferred to the ICU for postoperative care.
Results: The patient tolerated the procedure well without hemodynamic instability or adverse events. Postoperative recovery was uneventful, and the patient was discharged home after a brief ICU stay without the need for vasopressor support.
Conclusion: This case highlights successful tethered cord repair in a patient with complex congenital anomalies, including VACTERL association and DORV. Multidisciplinary collaboration among anesthesiologists, surgeons, and cardiologists was essential for optimizing perioperative care and ensuring favorable outcomes. Careful anesthesia management focused on maintaining hemodynamic stability and minimizing factors contributing to pulmonary vascular resistance, contributing to the successful outcome of the procedure.
References:
Sujakhu E, Shilpakar R, Shrestha D. Successful Repair of TEF and DORV in a Child in a Resource-Limited Setting. Case Rep Cardiol. 2023;2023:1095670. Published 2023 Feb 14. doi:10.1155/2023/1095670
Children's Hospital Boston. Double Outlet Right Ventricle (DORV) - Boston Children's Hospital. Accessed February 18, 2024. https://www.childrenshospital.org/conditions/dorv