Unilateral absence of pulmonary artery (UAPA) is a very rare condition, with an estimated prevalence of 1 in 200,000 population, which is commonly associated with various cardiovascular anomalies or can occur in an isolated manner. Isolated cases survive to adulthood and remain asymptomatic, but they may frequently experience hemoptysis, repeated infections, or symptoms like dyspnea and chest pain. Due to the rarity of the disorder and its ambiguous appearance, diagnosis can be very challenging.
Case presentation: We present a case of a 28-year-old male who visited our center with the diagnosis of ventricular septal defect with Eisenmenger syndrome elsewhere for further evaluation and was found to have right-sided UAPA with ipsilateral pulmonary hypoplasia and some associated cardiac anomalies.
Clinical discussion: Discussions are held regarding typical chest radiograph findings, diagnostic methods, and possible therapies.
Conclusion: Physicians should be aware of UAPA, which might go undiagnosed for several years despite regular medical care and can show up later in life, causing chronic respiratory symptoms along with Eisenmenger syndrome and ventricular septal defect like in our case.
Keywords: aortopulmonary window; pulmonary artery proximal interruption; unilateral absence of pulmonary artery; ventricular septal defect.
© 2023 the Author(s). Published by Wolters Kluwer Health, Inc.