Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect, typically requiring early treatment in infancy. Untreated TOF is associated with poor survival, with most uncorrected patients not surviving beyond the third decade. Here, we present a unique case of an 86-year-old female with uncorrected TOF who underwent a transcatheter aortic valve replacement (TAVR) procedure due to severe aortic stenosis (AS). The patient's TOF was identified during infancy, and she was categorized as an acyanotic "pink baby." Notably, the first palliative surgery for TOF was performed in 1944, when the patient was seven years old. The patient never underwent corrective surgery for TOF and continued to lead a symptom-free life until she developed severe AS later in life. The TAVR procedure significantly improved her symptoms, illustrating the importance of considering alternative etiologies for symptoms in elderly patients with uncorrected TOF and AS. In addition, we underscore the anesthetic management during TAVR, specifically highlighting the challenges addressed, such as the utilization of general anesthesia with transesophageal echocardiography (TEE) and the placement of a pulmonary artery (PA) catheter.
Keywords: adult congenital heart disease (achd); aortic stenosis (as); eisenmenger syndrome; tetrology of fallot; transcatheter aortic valve replacement (tavr).
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