CHEST Guidelines-A-26-Year-Old-Woman-With-a-Small-Right-Lung-and-a-

A-26-Year-Old-Woman-With-a-Small-Right-Lung-and-a-

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This document discusses a clinical case of a 26-year-old woman diagnosed with isolated unilateral pulmonary artery agenesis (UPAA), a rare congenital anomaly. The patient presented with an abnormal chest radiograph, showing a right-sided heart and a smaller right lung, but otherwise had no significant symptoms or past medical history. Physical examination and basic diagnostic evaluations appeared normal, but further imaging with a CT scan confirmed the diagnosis.<br /><br />UPAA involves the absence of one pulmonary artery, leading to associated anomalies such as a smaller lung and compensatory systemic circulation to the affected side. It may present with a variety of symptoms ranging from none at all to more serious ones including dyspnea, pulmonary hypertension, recurrent infections, and hemoptysis. The patient’s prior unexplained fever could be retrospectively attributed to a pulmonary infection, a common issue in UPAA due to impaired mucociliary clearance.<br /><br />The primary diagnostic method for UPAA includes imaging techniques such as CT scans, MRIs, and occasionally pulmonary angiography. Managing UPAA requires tailoring treatment to specific symptoms, such as antibiotics for infections and possible interventions like embolization for severe hemoptysis. In some patients, surgical revascularization or pharmacologic treatment for pulmonary hypertension might be necessary.<br /><br />The condition has a mortality rate of approximately 7%, predominantly due to complications such as massive pulmonary hemorrhage and pulmonary hypertension. Female patients with UPAA face increased risks, particularly regarding pregnancy, which can elevate mortality if pulmonary hypertension is present. Proper counseling and management strategies are emphasized to mitigate risks related to high altitude exposure and pregnancy.<br /><br />Overall, the article underscores the importance of early detection and appropriate management of UPAA to prevent serious complications.

Keywords

unilateral pulmonary artery agenesis

congenital anomaly

pulmonary hypertension

diagnostic imaging

pulmonary infection

hemoptysis

surgical revascularization

mortality risk

pregnancy complications

early detection