Isolated Unilateral Absent Branch Pulmonary Artery with Peripheral Pulmonary Stenosis and Coronary Artery Disease

Isolated Unilateral Absent Pulmonary Artery (UAPA) is a rare congenital anomaly. It
is usually associated with congenital heart defects. A 45 year old male patient presented
with complaints of fever with cough and expectoration for 15 days and retrosternal chest
discomfort for the previous 2 days. ECG showed diffuse ST segment depression with
T wave inversion in the inferior and lateral leads. Coronary Angiogram done through
the right femoral approach revealed diffusely diseased Left Anterior Descending (LAD)
artery that was totally cut off at the mid segment. The Left Circumflex (LCx) artery was
providing blood supply to the right middle and lower lung areas. There was another
collateral arising from the Left Subclavian Artery supplying the right middle and lower
lung areas. The left pulmonary artery was normal, but branches supplying the middle
and lower lobes of the right lung were absent and the upper lobe branch had pulmonary
stenosis. UAPA is a rare clinical entity; collaterals from coronaries are extremely rare in
this condition and till now there has not been any case report of unilateral absent branch
pulmonary artery with peripheral stenosis of other branches, on the affected side and
associated coronary artery disease.