Discussion:
Congenital, isolated agenesis of pulmonary arteries (UAPA) is a
developmental malformation that shows a unilateral absence of the
pulmonary artery without the presence of a congenital heart disease
[1]. This often leads to pulmonary hypoplasia of the ipsilateral
lung [1]. UAPA is often classified as congenital or acquired
[1]. Congenital is defined as a developmental defect or malformation
during embryogenesis [1,2]. Acquired due to chronic subclinical
infection, typically secondary to tuberculosis, that involves the hilum
of the lung [1]. UAPA has a bimodal distribution in regard to age of
presentation [1]. Those who are present in childhood are often
symptomatic as a result of congestive heart failure or pulmonary
hypertension [1]. Those who present as an adult are often
asymptomatic, typically due to collateral vasculature to the affected
lung [1,2]. While there are many modalities that can be used for
diagnosis, CT or MR pulmonary angiography is considered the gold
standard [1].