Congenital Aortic Arch Abnormality in Tetralogy of Fallot: A Rare Case Report

Abstract A 4-year-old male child was referred to the paediatrics department of our hospital from an Anganwadi with complaints of easy fatiguability compared to peers, dyspnea on normal exertion and refusal to feed for 10 days. There was no history of palpitation, bluish discolouration, pedal edema, abdominal distention, cough, or orthopnoea. On auscultation, a grade 2 end systolic murmur was heard. Full blood count revealed a Hb 10 g/dl, MCV 67.2 fL, MCH 20.9, and HCT 32.1%. Liver and kidney function tests were within normal limits. However, the Total T3 (2.85 nmol/L) and TSH were elevated (9.30 mmol/L). Chest X-ray indicated cardiomegaly with right ventricular enlargement with clear lung fields and pleural spaces. ECG further showed right axis deviation and enlargement of the right ventricle. He was diagnosed with Tetralogy of Fallot on 2D Echo, having confluent good-sized pulmonary arteries (Macgoon’s ratio: 1:4), large malaligned VSD with BD shunt, severe valvular and sub-pulmonary annular stenosis. These findings were further confirmed on MDCT Chest with Pulmonary Angiography. Furthermore, Bilateral anomalous origin of both right and left vertebral arteries from the arch of aorta was also observed. Later the patient was scheduled for elective surgical repair. Sauvage patch closure of VSD was done with 5-O continuous prolene sutures followed by reconstruction of the RVOT by augmenting it with a pericardial patch. The postoperative course was uneventful with minimal drainage. The patient was discharged on Injection Ceftumand Injection Lefoflox with infective endocarditis prophylaxis. In this case, we aimed to discuss the diagnostic implications of variations in the aortic arch with a special focus on the imaging and approach to the management.