Case Presentation: A 41-year-old male with a history of dextro-transposition of the great arteries (D-TGA) presented after thirty minutes of global aphasia. Symptoms resolved prior to arrival, and the patient noted that this was the second episode in the last three months. Surgical history was notable for Mustard repair of D-TGA at the age of six months. On arrival, he underwent MRI of the brain without contrast which demonstrated a small acute infarct in the left posterior parietal lobe and punctate susceptibility artifacts in the left temporal operculum. The patient was admitted for further management, and TTE demonstrated surgically repaired D-TGA with evidence of a baffle leak. Throughout admission, the patient remained asymptomatic. Per patient preference, he was discharged to follow up with his primary cardiologist prior to further definitive management.
Discussion: D-TGA is a rare congenital abnormality that occurs when the pulmonary artery originates from the left ventricle and the aorta originates from the right ventricle. Patients with D-TGA typically require surgical correction within the first year of life due to inadequate oxygenation of the blood. While the arterial switch operation is the current standard of care, techniques developed by Mustard and Senning were previously utilized. The Mustard procedure involves creation of a baffle to redirect deoxygenated blood from the vena cava to the left heart, where it is subsequently directed to the lungs. After passing through the pulmonary vasculature, blood moves to the right heart and is circulated systemically. Utilization of the Mustard procedure predominantly occurred between the 1960’s and the 1980’s, with most patients now between the ages of thirty and sixty years old. As this group of patients continues to age, there is an increasing probability that the hospitalist will be first to encounter those presenting with complications. Frequent complications include right heart failure, conduction abnormalities, baffle stenosis, and baffle leak. Patients with these outcomes may present with nonspecific complaints including dyspnea, fatigue, and palpitations. Though baffle leak is widely recognized as a potential complication of the Mustard procedure, neurologic findings have not been well documented in the existing literature. In this patient with baffle leak and no other past medical history, cardioembolic stroke was identified as the most likely source of his neurologic deficits. TTE plays a key role in the diagnosis of baffle leak, and percutaneous intervention with a covered stent allows for definitive management.
Conclusions: Though D-TGA is usually surgically corrected shortly after birth, patients who have undergone the Mustard procedure are now likely to be encountered as adults by the hospitalist. For patients presenting with complications, symptoms may be nonspecific and can share significant overlap with other commonly encountered conditions. In contrast to existing case reports, this patient provides a rare example of neurologic complications secondary to baffle leak. TTE plays a key role in diagnosis, and interdisciplinary management is essential for successful management.