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.