Anesthesia for the Repair of Coarctation of Aorta and Ventricular Septal Defect in a Patient With Heterotaxy Polysplenia Syndrome

Coarctation of the aorta (CoA) accounts for a small percentage of all congenital heart diseases (CHD) and occurs with a rare incidence in live births. It is a frequently diagnosed cardiac defect in infancy, though some patients present later with severe complications and reduced life expectancy. Heterotaxy syndrome is marked by abnormal lateralization of abdominal and thoracic organs, including the cardiac atria. Cardiovascular anomalies are the primary cause of morbidity in children with heterotaxy syndrome. Early suspicion and accurate diagnosis enable a more focused and effective approach to treatment. This case report seeks to review the literature on this rare and remarkable subset of developmental anomalies to inform the reader about the various modes of presentation, clinical manifestations, and surgical and anesthetic management. Here, we present a unique case of a 14-year-old male with left isomerism scheduled for CoA and ventricular septal defect (VSD) repair. The following case report was previously presented at the World Conference of Anesthesia in March 2024, Singapore as a poster presentation.