An 8-month-old girl (weight: 7 kg, height: 80 cm, body surface area: 0.39m2) was admitted to the hospital for mild cyanosis of the lips, and her vital signs were as follows: SpO2 of 84% (measured at fingertip), heart rate of 120 beats per minute, and blood pressure of 85/50 mm Hg. Chest auscultation revealed a grade 3/6 systolic murmur, best heard at the third intercostal space, adjacent to the left border of the sternum. Laboratory results showed that haemoglobin was 15.9 g/dL, haematocrit was 48.2%, and serum biochemistry was within the normal range. Her chest X-ray showed a right-deviated cardiac silhouette.
Examination
Abdominal ultrasound revealed situs inversus of the viscera. Doppler echocardiography confirmed the diagnosis of dextrocardia. It revealed a peri-membranous ventricular septal defect (VSD) with a diameter of 11 mm, an overriding aorta by 50%, and right ventricular outflow tract obstruction with a pressure gradient across the right ventricular outflow tract (RVOT) and pulmonary valve of 60 mm Hg. The diameter of the pulmonary valvular annulus was 9 mm. The sizes of the right and left pulmonary arteries were 6 and 5 mm, respectively.
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