Contents lists available at ScienceDirect Respiratory Medicine Case Reports journal homepage: www.elsevier.com/locate/rmcr Case report Unilateral pulmonary artery agenesis: An unusual cause of unilateral ARDS Lakshmi Saladi, MD a,* , Swati Roy, MD b , Gilda Diaz-Fuentes, MD, FCCP a a Division of Pulmonary and Critical Care Medicine, Bronx Lebanon Hospital Center, Icahn School of Medicine at Mount Sinai, Bronx, NY 10457, USA b Department of Medicine, Bronx Lebanon Hospital Center, Icahn School of Medicine at Mount Sinai, Bronx, NY 10457, USA ARTICLE INFO Poster presentation at New York State Thoracic Society Conference and American Thoracic Society Conference. Keywords: Unilateral pulmonary artery agenesis Acute respiratory distress syndrome Pulmonary hypertension Pulmonary edema Hemoptysis ABSTRACT Unilateral pulmonary artery agenesis (UPAA) is a rare malformation that can present as an isolated anomaly or may be associated with certain congenital cardiac anomalies, such as tetralogy of Fallot, atrial septal defect, coarctation of aorta, right aortic arch, truncus arteriosus and pulmonary atresia. Clinical presentation is non- specific which makes the diagnosis elusive; chronic dyspnea, hemoptysis or recurrent infections are the most common manifestations. Patients may remain asymptomatic until adulthood. There is no definitive treatment for patients with UPAA. Acute respiratory distress syndrome (ARDS) is usually a bilateral disease, unilateral ARDS has been described after lung resection or trauma. We present a case of a 39 year-old woman who developed unilateral ARDS and was later diagnosed with isolated UPAA. 1. Case presentation A 39-year-old woman presented for elective right robotic ne- phrectomy prior to renal transplant. Her medical history was significant for end stage renal disease on hemodialysis, atrophic left kidney and deformed right kidney. Her deceased brother had unknown congenital malformations. She had no toxic habits. Immediately after induction of general anesthesia, she developed severe laryngospasm and attempts for intubation were unsuccessful. She was placed on non-invasive po- sitive pressure ventilation with some improvement, however 4 to 6 hours later she developed progressive hypoxic respiratory failure as- sociated with massive hemoptysis and shock. She required intubation and transient use of vasopressors. Pertinent findings on examination were decreased breath sounds in left hemithorax and a grade IV/VI systolic murmur along left sternal border. No jugular venous distention or leg edema was present. She was dialyzed the day prior to planned surgery. Chest-roentgenogram (CXR) showed extensive right sided infiltrates and clear left lung (Fig. 1). Laboratory studies were significant for anemia, leukocytosis, and chronic renal failure. Fiberoptic bronchoscopy revealed normal mu- cosa, no endobronchial lesion or source of bleeding and no evidence of diffuse alveolar hemorrhage. Respiratory and blood cultures were ne- gative. Patient was started on antibiotics for possible aspiration pneumonia. Chest computed tomogram (CT) revealed absent left pulmonary artery and extensive right lung consolidation (Fig. 2). Echocardiogram revealed severe pulmonary hypertension (pul- monary artery systolic pressure: 62 mmHg) with right ventricular dys- function and normal left ventricular function. Ventilation/perfusion scan confirmed absence of perfusion to the left lung (Fig. 3). She was managed with lung protective strategy and later liberated from mechanical ventilation and discharged home. 2. Discussion The first reported case of UPAA was published in 1868 by Frantzel O. Angeborener and was demonstrated angiographically in 1952 by Madoff and colleagues [1,2] UPAA is rare, with a prevalence of 1:200,000 in young adults and usually occurs in conjunction with cardiovascular anomalies. This condition commonly affects the right lung and occurs on the side opposite the aortic arch [3]. Pulmonary artery branches are formed from the sixth aortic arches in embryos during the fourth week of gestation. During normal devel- opment, the proximal portions of the sixth arch persist, forming the right and left main branches of the pulmonary trunk. The pulmonary vascular bed may form normally even when a main branch of the pulmonary artery is absent because it develops from ventral branches of the dorsal aorta [4]. This condition can be under diagnosed as 30% of patients are https://doi.org/10.1016/j.rmcr.2018.02.004 Received 16 July 2017; Accepted 18 February 2018 * Corresponding author. E-mail addresses: lsaladi@bronxleb.org (L. Saladi), SRoy@bronxleb.org (S. Roy), gfuentes@bronxleb.org (G. Diaz-Fuentes). Abbreviations: ARDS, Acute respiratory distress syndrome; CT, Computed tomogram; CXR, Chest-roentgenogram; ET CO2, End tidal carbon dioxide; UPAA, Unilateral pulmonary artery agenesis Respiratory Medicine Case Reports 23 (2018) 148–151 2213-0071/ © 2018 The Authors. Published by Elsevier Ltd. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/BY-NC-ND/4.0/). T