A Rare Presentation of Congenital Tetralogy of Fallot with Dysplastic Ascending Aorta

Aim: A case report of a 23 year-old female, with congenital heart lesions and the clinical features slightly at variance with Tetralogy of Fallot. Method: Combined clinical assessment of patient and evaluation with chest x-ray and gray scale cardiac ultrasound was done. Results: Clinical assessment of the patient showed the following; palpitations, easy fatigability, cough, finger clubbing, orthopnoea, and lately significant precordial hyperactivity and tachypnoea on exertion. The chest x-ray and cardiac ultrasonography showed structural defects consisting of hypoplastic ascending aortic arch, with aortic root overriding inter-ventricular septal defect, right heart chamber enlargement, and congested pulmonary outflow track. Conclusion: There was hypoplastic ascending aortic trunk (instead of hypoplasia of pulmonary trunk) a slight variation from the components of Tetralogy of Fallot. The probability is that this may have sustained the patient to adult hood. This is a rare occurrence in literature. Limitation of evaluation is also discussed.