Rare Cause of Secondary Pulmonary Arterial Hypertension

Unilateral absent pulmonary artery (UAPA) is a rare congenital disorder. Most of the patients will be diagnosed in the early childhood because of recurrent respiratory tract infections and hemoptysis, but adult presentation is not uncommon. We report a 47-year-old female who was earlier diagnosed as bronchiectasis with pulmonary artery hypertension but now presented with complaints of typical angina and dyspnea on exertion. During right heart catheterization we failed to enter right pulmonary artery (RPA), and conventional coronary angiogram showed a large left atrial branch of left circumflex giving collateral blood supply to the right lung. Computed tomography angiogram showed absent RPA. We report this case not only because the patient was misdiagnosed but also because of rarity of coronary collaterals in UAPA patients and unusual bilateral bronchiectasis. According to reported literature, ours is the 28th case of this nature.