Marjolein Spoel1, Helen Marshall2, Hanneke IJsselstijn1, Juan Parra-Robles2, Els van der Wiel3, Andrew J Swift2, Smitha Rajaram2, Dick Tibboel1, Harm A W M Tiddens3,4, Jim M Wild2. 1. Intensive Care and Department of Paediatric Surgery, Erasmus MC-Sophia Children's Hospital Rotterdam, Rotterdam, The Netherlands. 2. Department of Academic Radiology, University of Sheffield, Sheffield, United Kingdom. 3. Department of Paediatrics-Respiratory Medicine and Allergology, Erasmus MC-Sophia Children's Hospital, Rotterdam, The Netherlands. 4. Department of Radiology, Erasmus MC, Rotterdam, The Netherlands.
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.
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 CDHpatients 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 CDHpatients. Ipsilateral elevated (3) He ADC values are consistent with enlargement of mean dimensions of the confining lung micro-structure at the alveolar level.
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