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Pulmonary ventilation and micro‐structural findings in congenital diaphragmatic hernia
Author(s) -
Spoel Marjolein,
Marshall Helen,
IJsselstijn Hanneke,
ParraRobles Juan,
van der Wiel Els,
Swift Andrew J.,
Rajaram Smitha,
Tibboel Dick,
Tiddens Harm A.W.M.,
Wild Jim M.
Publication year - 2016
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.23325
Subject(s) - medicine , congenital diaphragmatic hernia , ventilation (architecture) , lung , diaphragmatic breathing , mechanical ventilation , lung volumes , radiology , anesthesia , pathology , pregnancy , mechanical engineering , fetus , genetics , alternative medicine , engineering , biology
Summary 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. Pediatr Pulmonol. 2016;51:517–524 . © 2015 Wiley Periodicals, Inc.