Rare association of TOF with absent pulmonary valve syndrome
The present case has been published in BMJ. A 2-month-old boy presented to the paediatric cardiology department with cyanosis and feeding difficulties.

Chest radiograph revealed cardiomegaly, right-sided aortic knuckle and dilated pulmonary artery segment along with mild indentation on the lower trachea and diffusely narrowed left main bronchus. A diagnosis of tetralogy of Fallot (TOF) with absent pulmonary valve syndrome was made on transthoracic echocardiogram; however, origin of right pulmonary artery (RPA) was not well visualised.

The patient further underwent CT angiography (CTA) to delineate the cardiac as well as any extracardiac abnormalities. CTA revealed features of TOF with pulmonary annular stenosis. Pulmonary valve leaflets were absent with aneurysmal dilatation of the main and left pulmonary artery (LPA).

The RPA was not seen in continuation with the main pulmonary artery; instead, it was seen to arise from the mid-ascending aorta and supplying the right lung. Review of the lung window images revealed collapse of the apicoposterior segment of the left upper lobe and superior segment of the right lower lobe.

Subsequent careful examination of the airway depicted an ‘inverted-V’-shaped trachea with diffuse mild narrowing of the left main bronchus, near-complete occlusion of the apicoposterior branch of the left upper lobe bronchus and the superior segmental branch of the left lower lobe bronchus, due to compression by the dilated left pulmonary artery. These findings were exquisitely demonstrated on virtual bronchographic images.

Read more here: http://casereports.bmj.com/content/2018/bcr-2018-227008.full
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