Venovenous Extracorporeal Membrane Oxygenation During Bilateral Lung Transplantation for a Patient With Eisenmenger Syndrome Complicated With Giant Pulmonary Artery Aneurysm, Azygos Continuation, and Central Airway Obstruction

Abstract

AN INTRACARDIAC left-to-right shunt increases pulmonary blood flow and pulmonary artery (PA) pressure (PAP), a condition that can cause pulmonary vascular injury and result in the development of Eisenmenger syndrome (ES) in the terminal stage. Moreover, the extreme increase in pulmonary blood flow and/or pulmonary vascular resistance (PVR) is associated with giant PA aneurysms (PAAs); however, these cases are rare. 1 Butto F Lucas Jr, RV Edwards JE Pulmonary arterial aneurysm. A pathologic study of five cases. Chest. 1987; 91: 237-241 Abstract Full Text Full Text PDF PubMed Scopus (78) Google Scholar For patients with ES and giant PAA, heart-lung transplantation (HLTx) or, alternatively, bilateral lung transplantation (BLTx), combined with intracardiac repair and PA replacement, are the only 2 available life-saving interventions. 2 Noda M Okada Y Saiki Y et al. Reconstruction of pulmonary artery with donor aorta and autopericardium in lung transplantation. Ann Thorac Surg. 2013; 96: e17-e19 Abstract Full Text Full Text PDF PubMed Scopus (12) Google Scholar ,3 Toyama H Saito K Takei Y et al. Two cases of bilateral lung transplantation combined with intracardiac repair and pulmonary artery replacement: perioperative managements based on the left ventricular function. J Anesth. 2015; 29: 957-961 Crossref PubMed Scopus (3) Google Scholar In patients with severe pulmonary arterial hypertension (PAH), PVR elevation, low systemic pressure, and high airway pressure should be avoided during anesthesia, as all these factors can induce right ventricular (RV) failure and create a lethal situation. Additionally, a giant PAA sometimes can compress the coronary arteries and airways, which can cause coronary ischemia and airway obstruction after anesthesia induction. In such cases, venoarterial (VA) extracorporeal membrane oxygenation (ECMO) often is used. 4 Ius F Tudorache I Warnecke G. Extracorporeal support, during and after lung transplantation: The history of an idea. J Thorac Dis. 2018; 10: 5131-5148 Crossref PubMed Scopus (22) Google Scholar However, when peripheral VA ECMO is used in patients with narrowed peripheral arteries, the return flow can decrease, and the mixing point of the patient's arterial blood with the ECMO's return flow can move to the distal portion of the aortic arch, 5 Alwardt CM Patel BM Lowell A et al. Regional perfusion during venoarterial extracorporeal membrane oxygenation: A case report and educational modules on the concept of dual circulations. J Extra Corpor Technol. 2013; 45: 187-194 PubMed Google Scholar a situation that can cause watershed phenomena and/or hypoxic encephalopathy (Harlequin syndrome). 6 Avgerinos DV DeBois W Voevidko L et al. Regional variation in arterial saturation and oxygen delivery during venoarterial extracorporeal membrane oxygenation. J Extra Corpor Technol. 2013; 45: 183-186 PubMed Google Scholar ,7 Napp LC Kühn C Hoeper MM et al. Cannulation strategies for percutaneous extracorporeal membrane oxygenation in adults. Clin Res Cardiol. 2016; 105: 283-296 Crossref PubMed Scopus (130) Google Scholar Herein, the authors report the management of BLTx in a patient with ES, severe PAH, giant PAA, a constricted trachea and right bronchus, and an interruption of the inferior vena cava (IVC) with azygos and hemiazygos continuation. As the authors believed that the constricted trachea and right bronchus could become obstructed and cause ventilation difficulties after general anesthesia induction, they initially had deemed it safer to use VA ECMO for the patient. However, it would have been impossible to ensure sufficient VA ECMO because of the patient's narrowed axillary and femoral arteries. To solve these problems, a drainage cannula was placed in the azygos vein, a return cannula was placed in the right atrium (RA), and venovenous (VV) ECMO was established before the induction of general anesthesia.