Heterotaxy syndrome is a rare congenital disorder characterized by the abnormal arrangement of thoracoabdominal organs and complex cardiovascular malformations. Left isomerism, a subtype, typically features bilateral left atrial appendages, polysplenia, and interrupted inferior vena cava (IVC) with azygos continuation. This case is an example of a brain abscess in Heterotaxy syndrome, which is a rare complication seen in patients with chronically uncorrected cardiac anomaly, which causes Eisenmengerization of the heart and allows the bacteria to bypass the physiological lung defences and cause brain abscess.

Case Presentation: A 26-year-old female presented with fever, right hemiparesis, and giddiness for 3–4 days. Clinical examination revealed central cyanosis, oxygen saturation of 75% on room air, grade 3 digital clubbing, and a pansystolic murmur at the left sternal border. Brain MRI revealed a 25 × 30 × 55 mm left frontal lobe lesion with perilesional edema, midline shift, and subfalcine herniation, which points towards a brain abscess.

Cardiac workup demonstrated multiple uncorrected complex anomalies: Atrioventricular discordance, mesocardiac with L ventricular loop, double outlet right ventricle, large ventricular septal defect with bidirectional shunting, severe pulmonary stenosis, right superior and inferior pulmonary veins draining into common atrium with absent interatrial septum. CT imaging confirmed situs ambiguous with left isomerism, midline liver, right-sided stomach bubble, and polysplenia. The IVC was interrupted with azygos continuation into the superior vena cava. Additional findings included hypoplastic pulmonary arteries and major aortopulmonary collateral arteries, which cause reduced pulmonary flow and chronic cyanosis. The patient was started on high-flow oxygen, broad-spectrum intravenous antibiotics, and antiepileptics. Neurosurgery was consulted, and the abscess was drained. After a month, a staged cardiac surgical approach was planned, including right ventricular outflow tract resection, intraventricular tunnel repair, Senning procedure, and pacemaker implantation.

Conclusion: This case highlights the severe neurological risks associated with uncorrected cyanotic congenital heart disease in heterotaxy syndrome.

Jonsi Tavethia, who is currently doing her MBBS internship at BJ Medical College in Ahmedabad, India. She is a research enthusiast and has multiple publications. Her primary field of interest is cardiology, and she loves to explore interesting cases.