Pulmonary Vascular Disease: Thoracic Vascular Disorders |

A Case of Unilateral Pulmonary Artery Agenesis FREE TO VIEW

Janushe Patel, MD; Kala Davis-McDonald, MD
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St. Agnes Hospital, Baltimore, MD

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):1233A. doi:10.1016/j.chest.2016.08.1342
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SESSION TITLE: Thoracic Vascular Disorders

SESSION TYPE: Student/Resident Case Report Slide

PRESENTED ON: Monday, October 24, 2016 at 11:00 AM - 12:00 PM

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 throughout both lungs, right-sided aortic arch, 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. Echocardiogram demonstrated 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 rehabilitation 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.

DISCUSSION: UPAA is an uncommon anomaly that is typically associated with congenital cardiac defects. Isolated UPAA may be asymptomatic or present with recurrent respiratory tract infections, decreased exercise tolerance and shortness of breath with exertion3. Diagnosis of unilateral pulmonary agenesis is typically made upon CT imaging of the chest. Patients with unilateral pulmonary agenesis are at higher risk for ventilation-perfusion defects.

CONCLUSIONS: In retrospect patient’s history of exercise-induced asthma may have been a symptom of UPAA. Its presence was a contributing factor to her severe hypoxemic respiratory failure. Congenital pulmonary artery agenesis is a rare cause of acute respiratory distress in adults with no prior hospitalizations and can often go undiagnosed.

Reference #1: Frantzel O. Angeborener Defect der rechten Lungenarterie.Virchows Arch Path Anat1868; 43:420

Reference #2: Brassord J, Johnson J. Unilateral absence of pulmonary artery: data from cardiopulmonary exercise testing. Chest 1993; 103: 293-95

Reference #3: Ten Harkel AD, Blom NA, Ottenkamp J: Isolated unilateral absence of a pulmonary artery: a case report and review of the literature. Chest. 2002, 122: 1471-1477. 10.1378/chest.122.4.1471.

DISCLOSURE: The following authors have nothing to disclose: Janushe Patel, Kala Davis-McDonald

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