Abstract Introduction Unilateral absence of the pulmonary artery (UAPA) is a rare congenital anomaly, typically diagnosed in childhood. Adult presentations are uncommon, with very few cases reported beyond the sixth decade of life. We present a case of UAPA diagnosed at age 63, which, to our knowledge, is one of the oldest reported presentations in the United States, representing a unique contribution to the current literature. Case Presentation A 63 year old woman with a history of asthma and treated latent tuberculosis presented with persistent cough, wheezing, and dyspnea following hospitalization for pneumonia. Initial sputum cultures grew fluoroquinolone sensitive Pseudomonas aeruginosa, but subsequent cultures showed resistance. Pulmonary function tests showed moderate to severe obstruction (FEV1 50%) with mild restriction (TLC 78%). High-resolution CT was done, which revealed tracheal deviation, hypoplasia of right lung, bronchiectasis, absence of right pulmonary artery and cardiac displacement into the right hemithorax. She was treated with nebulized tobramycin (300 mg twice daily for 10 days), transitioned to a new interface for nocturnal ventilatory support, and prescribed a high-frequency chest wall oscillation device. Additional work-up included echocardiography and an esophagram. She was referred to cardiology for further evaluation and remained on aspiration precautions. Discussion This case represents a rare diagnostic challenge due to the late presentation of a congenital vascular anomaly. UAPA is estimated to affect 1 in 200,000 individuals and is typically diagnosed during childhood. In adults, it is often missed or discovered incidentally. A 2017 review of 65 adult cases showed a median diagnosis age of 14 years; diagnosis at 63 years, as in this case, is among the oldest reported in the United States. Structural changes in the lung and chronic infections are among the common risk factors for poor outcomes. Our patient developed bronchiectasis and persistent Pseudomonas infection, complicated by antibiotic resistance. Timely identification and management with inhaled antimicrobials and multidisciplinary care led to clinical improvement. Conclusion This case highlights the significance of considering congenital anomalies in adults with unexplained respiratory symptoms and asymmetric imaging findings. Early diagnosis, even in advanced age, may reduce morbidity and improve outcomes. This abstract is funded by: None
Jilani et al. (Fri,) studied this question.