Case:
A three and half year old girl suffering with central cyanosis since birth, clubbing, exertional fatigue and recurrent respiratory infections was presented. On clinical examination, child was found with severe cyanosis and resting oxygen saturation was 74% in room air. Weight and height of the patient was recorded 13 Kg and 89 cm, respectively. Cardiovascular examination: normal heart sounds with a mild systolic heart murmur on the left sternal border, heart rate 110 beats / minute. The pulse is palpable and symmetrical in the four limbs. Respiratory examination: Clear lungs, pectus excavatum. Examination of the nervous system: normal
The child had frequent respiratory infections and hospitalized due to pneumonia a year ago. Furthermore, laboratory analysis was normal except for HB = 18, ECG: normal. Chest X-ray: Increased vascularity with an opacity most likely representing a vascular silhouette in the right lung.
Echocardiography: Secondary ASD 6.5 mm in diameter with a left to right shunt, enlargement of main pulmonary trunk and the right pulmonary artery, the rest of the echo was normal.Contrast echo confirmed the abnormal connection between the pulmonary arteries and the left atrium through the visualization of bubbles in the left atrium.
Cardiac catheterization was performed, where the presence of the fistula was confirmed between the right pulmonary artery and the left atrium (Fig. 1). Multi-slice CT shows that the pulmonary artery branch supplying the middle right lobe drains directly into the left atrium through a large fistula taking the form of a large aneurysm accompanied by an absence of the upper right pulmonary vein (Fig. 2)
The surgery was performed under general anesthesia through a median sternotomy on a cardiopulmonary bypass, the fistula was found to be 2 - 2.5 cm in size, connecting the right pulmonary artery and the left atrium at the point where the right upper pulmonary vein usually opens into the atrium, the right upper pulmonary vein was absent . By opening the right atrium and through the ASD, the fistula’s ostium was reached; closure was performed using a biological patch, followed by closure of the ASD (Fig. 3). Oxygen saturation improved to 95% immediately after surgery, on follow-up. After 6 months, the patient was asymptomatic and the clinical examination was normal.