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Poster Walks: Poster Walk 1: Airway disease I |

P139 One lung to live: a case of unilateral pulmonary artery agenesis

J. Patel; K. Davis-McDonald
Author and Funding Information

1Internal Medicine, Kern Medical, Bakersfield

2St. Agnes Hospital, Baltimore, United States


Copyright 2017, American College of Chest Physicians and Swiss Respiratory Society SGP. All Rights Reserved.


Chest. 2017;151(5_S):A36. doi:10.1016/j.chest.2017.04.039
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Extract

Introduction: Unilateral pulmonary artery agenesis (UPAA) is a rare congenital anomaly, with only 150 cases reported since its first description in 1868. 1 We present a case encountered in clinical practice.

Case Presentation: A 24 y/o non smoking African American female with a history of morbid obesity, premature birth, exercise induced asthma, presented with complaints of progressive shortness of breath, cough, fever of 103, and chills. On arrival to the ER, she was noted to be in severe respiratory distress despite non-rebreather mask and eventually required intubation. Arterial blood gas revealed: pH 7.32, pCO2 46mmHg, pO2 41mmHg, HCO3 mEQ/L, O2 Saturation 63.6% on 100% FiO2 on mechanical ventilation. Chest x-ray was limited due to body habitus, therefore spiral CT was done to rule out pulmonary embolism given the degree of hypoxia. CT angiography was remarkable for dense right lower lobe consolidation, patchy infiltrates though out both lungs, an enlarged pulmonary trunk and right pulmonary artery, however absent left pulmonary artery. No definitive filling defects in the pulmonary arteries existed to suggest pulmonary emboli and timing of contrast was appropriate. Respiratory status worsened precipitously and patient eventually required extracorporeal membrane oxygenation (ECMO) for severe ARDS. She had a prolonged hospitalization complicated by sepsis, bacteremia, transient renal failure requiring CRRT and tracheitis. With extensive rehabiltation the patient improved and no longer requires supplemental oxygen. Four months after discharge, pulmonary function tests revealed a total lung capacity 79% predicted, residual capacity 58% predicted, FEV1- 2.88L (92%), FVC- 3.37L(86%) and a DLCO 69% predicted.

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