Perioperative Management of DORV

The diagnosis of double-outlet right ventricle (DORV) characterizes a complex heterogeneous group of congenital cardiac malformations for which multiple classification schemes have been used. A clear understanding of the anatomy is critical to understanding the physiologic consequences of the specific type of DORV. Perioperative considerations include the medical management of the patient during the preoperative period, anesthetic and surgical management, and postoperative care. Both anesthetic and surgical management strategies are very different depending on the type of DORV. Key principles for anesthetic management include balancing the systemic and pulmonary circulations, optimizing systemic cardiac output, and closely monitoring for impaired oxygen delivery to the tissues. Depending on the specific anatomy the patient is usually placed on a 1- or 2-ventricle pathway, and initial palliation may involve placement of a systemic arterial to pulmonary artery shunt or pulmonary artery banding. In some cases the child may undergo a complete repair during the first few months of life. Surgical outcomes, both short and long-term, are dependent on the type of DORV and surgical procedure done. These patients require long-term follow up and may present for surgical or catheter-based interventions as adults.