title: Pulmonary ventilation and micro-structural findings in congenital diaphragmatic hernia.
abstract: BACKGROUND
  With increasing survival of patients with more severe forms of congenital diaphragmatic hernia (CDH) and risk of long-term respiratory morbidity, studies on lung morphology are needed. We used hyperpolarised (3) He MRI and anatomical (1) H MRI in a cohort of young adult CDH patients to image regional lung ventilation and microstructure, focusing on morphological and micro-structural (alveolar) abnormalities.
  
  
  METHODS
  Nine patients with left-sided CDH, born 1975-1993, were studied. Regional ventilation was imaged with hyperpolarised (3) He MRI, and the (3) He apparent diffusion coefficient (ADC) was computed separately for the ipsilateral and contralateral lungs. (1) H MRI was used to image lung anatomy, total lung volume and motion during free-breathing.
  
  
  RESULTS
  (3) He MRI showed ventilation abnormalities in six patients, ranging from a single ipsilateral ventilation defect (3 patients) to multiple ventilation defects in both lungs (one patient treated with extra corporeal membrane oxygenation). In eight patients, (3) He ADC values for the ipsilateral lung were significantly higher than those for the contralateral lung.
  
  
  CONCLUSIONS
  Functional and micro-structural changes persist into adulthood in most CDH patients. Ipsilateral elevated (3) He ADC values are consistent with enlargement of mean dimensions of the confining lung micro-structure at the alveolar level.
authors.affiliation: Intensive Care and Department of Paediatric Surgery, Erasmus MC-Sophia Children's Hospital Rotterdam, Rotterdam, The Netherlands.
authors.forename: Marjolein
authors.initials: M
authors.lastname: Spoel
authors.affiliation: Department of Academic Radiology, University of Sheffield, Sheffield, United Kingdom.
authors.forename: Helen
authors.initials: H
authors.lastname: Marshall
authors.affiliation: Intensive Care and Department of Paediatric Surgery, Erasmus MC-Sophia Children's Hospital Rotterdam, Rotterdam, The Netherlands.
authors.forename: Hanneke
authors.initials: H
authors.lastname: IJsselstijn
authors.affiliation: Department of Academic Radiology, University of Sheffield, Sheffield, United Kingdom.
authors.forename: Juan
authors.initials: J
authors.lastname: Parra-Robles
authors.affiliation: Department of Paediatrics-Respiratory Medicine and Allergology, Erasmus MC-Sophia Children's Hospital, Rotterdam, The Netherlands.
authors.forename: Els
authors.initials: E
authors.lastname: van der Wiel
authors.affiliation: Department of Academic Radiology, University of Sheffield, Sheffield, United Kingdom.
authors.forename: Andrew J
authors.initials: AJ
authors.lastname: Swift
authors.affiliation: Department of Academic Radiology, University of Sheffield, Sheffield, United Kingdom.
authors.forename: Smitha
authors.initials: S
authors.lastname: Rajaram
authors.affiliation: Intensive Care and Department of Paediatric Surgery, Erasmus MC-Sophia Children's Hospital Rotterdam, Rotterdam, The Netherlands.
authors.forename: Dick
authors.initials: D
authors.lastname: Tibboel
authors.affiliation: Department of Paediatrics-Respiratory Medicine and Allergology, Erasmus MC-Sophia Children's Hospital, Rotterdam, The Netherlands.|Department of Radiology, Erasmus MC, Rotterdam, The Netherlands.
authors.forename: Harm A W M
authors.initials: HA
authors.lastname: Tiddens
authors.affiliation: Department of Academic Radiology, University of Sheffield, Sheffield, United Kingdom.
authors.forename: Jim M
authors.initials: JM
authors.lastname: Wild
mesh_terms: D000328:Adult; D005260:Female; D065630:Hernias, Diaphragmatic, Congenital / Q000473:pathology / Q000503:physiopathology*; D006801:Humans; D008168:Lung / Q000002:abnormalities* / Q000473:pathology / Q000503:physiopathology; D008279:Magnetic Resonance Imaging; D008297:Male; D012123:Pulmonary Ventilation / Q000502:physiology*; D012119:Respiration; D055815:Young Adult
keywords: MRI; alveolar structure; congenital diaphragmatic hernia; lung development; pulmonary ventilation