title: Bilateral lung transplantation in a patient with Vascular Ehlers-Danlos syndrome.
abstract: We describe the case of a 29-year-old woman with end-stage chronic obstructive pulmonary disease secondary to vascular Ehlers-Danlos syndrome. Because of critical deterioration, respiratory arrest, and complete lung failure, she required urgent implantation of a venovenous extracorporeal membrane oxygenator as a bridge to lung transplantation. After 6 days of extracorporeal life support, a successful bilateral sequential lung transplantation was performed. This is the first case of lung transplantation in a patient with a diagnosis of chronic obstructive pulmonary disease secondary to Ehlers-Danlos syndrome.
authors.affiliation: Department of Cardiothoracic Transplantation and Mechanical Support, Royal Brompton and Harefield NHS Trust, London, United Kingdom. Electronic address: d.garciasaez@rbht.nhs.uk.
authors.forename: Diana
authors.initials: D
authors.lastname: García Sáez
authors.affiliation: Department of Cardiothoracic Transplantation and Mechanical Support, Royal Brompton and Harefield NHS Trust, London, United Kingdom.
authors.forename: Prashant N
authors.initials: PN
authors.lastname: Mohite
authors.affiliation: Department of Cardiothoracic Transplantation and Mechanical Support, Royal Brompton and Harefield NHS Trust, London, United Kingdom.
authors.forename: Bartlomiej
authors.initials: B
authors.lastname: Zych
authors.affiliation: Department of Cardiothoracic Transplantation and Mechanical Support, Royal Brompton and Harefield NHS Trust, London, United Kingdom.
authors.forename: Anton
authors.initials: A
authors.lastname: Sabashnikov
authors.affiliation: Department of Cardiothoracic Transplantation and Mechanical Support, Royal Brompton and Harefield NHS Trust, London, United Kingdom.
authors.forename: Ajay
authors.initials: A
authors.lastname: Moza
authors.affiliation: Department of Respiratory Medicine, Royal Brompton and Harefield NHS Trust, London, United Kingdom.
authors.forename: Martin
authors.initials: M
authors.lastname: Carby
authors.affiliation: Department of Cardiothoracic Transplantation and Mechanical Support, Royal Brompton and Harefield NHS Trust, London, United Kingdom.
authors.forename: André R
authors.initials: AR
authors.lastname: Simon
mesh_terms: D000328:Adult; D018450:Disease Progression; D004535:Ehlers-Danlos Syndrome / Q000150:complications* / Q000175:diagnosis; D015199:Extracorporeal Membrane Oxygenation / Q000379:methods*; D005260:Female; D005500:Follow-Up Studies; D006085:Graft Survival; D006801:Humans; D016040:Lung Transplantation / Q000379:methods*; D012131:Respiratory Insufficiency / Q000209:etiology* / Q000503:physiopathology / Q000601:surgery*; D018570:Risk Assessment; D013997:Time Factors; D016896:Treatment Outcome; D014850:Waiting Lists