A 32weeks old male neonate was delivered vaginally with a birth weight
of 1800grams due to premature labour. His right upper and lower limb
oxygen saturation was 50%. In view of poor respiratory efforts, he was
mechanically ventilated immediately after birth. Chest X-ray revealed
bilateral diffuse interstitial reticular pattern of the lungs (Nutmeg
lung) suggestive of pulmonary lymphangiectasia. Echocardiography showed
intact thick interatrial septum(Figure 2A) , atresia of the
mitral valve, left ventricle(Figure 2B) and aortic valve. The
left atrium appeared tense with prominent appendage and the pulmonary
veins were dilated(Figure 2C&D) . The pulmonary artery arose
from the morphological right ventricle(RV) with adequate antegrade flows
but the ascending aorta had no connection with the RV, the aortic valve
was atretic and the blood flow was retrograde from Circle of Willies(Figure 2E-F) since the aortic arch was interrupted after the
first branch and the patent ductus arteriosus was supplying the left
common carotid artery, left subclavian artery and descending
aorta[Type C interrupted aortic arch (IAA)]. There was no
aortopulmonary window or visible collaterals from descending aorta.
Treatment options like urgent balloon atrial septectomy for survival
followed by palliative surgery like Norwood operation or primary heart
transplantation were discussed. Considering the guarded long-term
outcome, the parents opted for comfort care. A schematic cartoon
demonstrating the anatomy and hemodynamics of the described lesion is
shown in Figure 3 .