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RECURRENT HEMOPTYSIS IN UNILATERAL PULMONARY ARTERY AGENESIS

      TOPIC: Signs and Symptoms of Chest Diseases
      TYPE: Fellow Case Reports
      INTRODUCTION: Unilateral pulmonary artery agenesis (UPAA) is a rare condition that is typically from a defect during birth. It may remain asymptomatic and only be diagnosed in adulthood. Symptoms may include dyspnea on exertion, recurrent pulmonary infections, and pleural effusion. Hemoptysis has been described in 10% of patients. Imaging modalities include pulmonary angiography and aortography to localize and embolize collaterals for a therapeutic approach. This case presents a patient with known UPAA with recurrent hemoptysis with difficulty in localization of bleed.
      CASE PRESENTATION: A 27-year-old female with history of factor 5 Leiden deficiency, von Willebrand's disease, polycystic ovarian syndrome, morbid obesity, absent right pulmonary artery, atrial septal defect, and patent ductus arteriosus presented to the emergency department after experiencing an episode of hemoptysis. She denied any chest pain, fever, or chills, however, since her recent diagnosis with COVID-19, she has had ongoing shortness of breath. In the emergency room, she had a temperature of 98.6°F, heart rate of 123bpm, respiratory rate of 18 breaths/min, blood pressure of 186/113mmHg and Spo2 of 100% on ambient air. Physical exam was unremarkable. Her labs consisted of a normal PT and INR, unremarkable CMP, Hemoglobin of 13.8 g/dL, platelets of 250,000/uL. A CT angiogram was done showing no pulmonary embolism but confirming absence of the right pulmonary artery and showing a hypoplastic right lung (Figure1). She was subsequently admitted under observation. A 2D echo did not show any evidence of pulmonary hypertension. She had additional small episodes of hemoptysis and therefore underwent a bronchoscopy, however, was completely obscured by blood upon entry and was withdrawn. She underwent a repeat bronchoscopy in the operating room which showed a slow ooze from the posterior segment of the right upper lobe. She did not have any further episodes of hemoptysis and was therefore discharged. She presented again with significant hemoptysis requiring intubation followed by a bronchoscopy where a large thrombus and blood was removed. Due to her complex pathology, she was flown to a tertiary care center where her hemoptysis eventually subsided.
      DISCUSSION: Unilateral pulmonary artery agenesis has a prevalence of 1 in 200,000 patients caused by failure in connection of the sixth aortic arch and pulmonary trunk. It may be accompanied with other congenital heart abnormalities. Therapeutic options include surgical reconstruction of the pulmonary artery early in childhood. In adulthood, one may consider surgical occlusion of collateral vessels or pneumonectomy to avoid hemoptysis.
      CONCLUSIONS: Outcomes are not well-described given the rare condition, however, routine surveillance in the development of pulmonary hypertension is important. Given our patient's known diagnosis, she was referred to her congenital cardiologist for close follow up.
      REFERENCE #1: Steiropoulos P, Archontogeorgis K, Tzouvelekis A, Ntolios P, Chatzistefanou A, Bouros D. Unilateral pulmonary artery agenesis: a case series. Hippokratia. 2013;17(1):73-76.
      REFERENCE #2: Johnson, T.R.C, Thieme, S.F, Deutsch, M.A, et al. Circulation. 2021. Unilateral Pulmonary Artery Agenesis. [online] Available at: https://www.ahajournals.org/doi/10.1161/CIRCULATIONAHA.108.777698 [Accessed 13 April 2021
      DISCLOSURES: No relevant relationships by Tokunbo Matthews, source=Web Response
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