Isolated unilateral absence of the pulmonary artery (IUAPA) presenting with progressive interstitial shadows: A case report

Tomohito Takeshige; Ryo Koyama; Makiko Kohmaru; Takashi Akimoto; Junko Watanabe; Toshifumi Yae; Kenji Kido; Kazuhisa Takahashi

doi:10.1002/rcr2.70048

Isolated unilateral absence of the pulmonary artery (IUAPA) presenting with progressive interstitial shadows: A case report

Respirol Case Rep. 2024 Oct 16;12(10):e70048. doi: 10.1002/rcr2.70048. eCollection 2024 Oct.

Authors

Tomohito Takeshige^{1

2}, Ryo Koyama¹, Makiko Kohmaru¹, Takashi Akimoto¹, Junko Watanabe¹, Toshifumi Yae¹, Kenji Kido¹, Kazuhisa Takahashi²

Affiliations

¹ Department of Respiratory Medicine Juntendo University Nerima Hospital Tokyo Japan.
² Department of Respiratory Medicine Juntendo University Faculty of Medicine and Graduate School of Medicine 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.

Keywords: interstitial change; interstitial lung disease (ILD); interstitial shadow; isolated unilateral absence of the pulmonary artery (IUAPA); unilateral absence of pulmonary artery (UAPA).