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Updated: Jun 10, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Isolated unilateral absence of the pulmonary artery (IUAPA) presenting with progressive interstitial shadows: A case
Tomohito Takeshige1,2, Ryo Koyama1, Makiko Kohmaru1
1Department of Respiratory Medicine Juntendo University Nerima Hospital Tokyo Japan.
Abstract:
Unilateral absence of the pulmonary artery (UAPA) is a rare congenital condition. When UAPA occurs without associated congenital heart disease, it is referred to as isolated unilateral absence of the pulmonary artery (IUAPA). IUAPA is frequently not diagnosed until adulthood. A 61-year-old female patient presented with right chest pain and a worsening interstitial shadow over 9 years. Contrast-enhanced computed tomography revealed the absence of blood flow from the pulmonary artery in the right lung, leading to the diagnosis of IUAPA. In this case, the diagnosis of UAPA was established after approximately 40 years. This case underscores the importance of identifying vascular abnormalities to differentiate this condition in patients presenting with nonspecific symptoms and interstitial shadows.
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