Quantitative Lung Function Assessment in 2-year old children after Congenital Diaphragmatic Hernia using Fourier Decomposition Magnetic Resonance Imaging

傅里叶分解磁共振成像对2岁儿童先天性膈疝术后肺功能的定量评估

• 批准号: 397806429
• 负责人: Privatdozentin Dr. Meike Weis
• 金额: --
• 依托单位: Institut für Klinische Radiologie und Nuklearmedizin
• 依托单位国家: 德国
• 项目类别: Research Grants
• 财政年份: 2018
• 资助国家: 德国
• 起止时间: 2017-12-31 至 2022-12-31
• 项目状态: 已结题
• 来源: https://gepris.dfg.de/gepris/projekt/397806429?language=en
• 关键词: Quantitative; Lung; Function; Assessment; year

Congenital diaphragmatic hernia (CDH) leads to herniation of abdominal organs into the thoracic cavity and consequently to impaired lung development, followed by lung hypoplasia. Lung hypoplasia and pulmonary hypertension are known to be the main determinants of survival outcome in cases of CDH if no severe additional malformations or syndromes are present. Lung hypoplasia is not only attributed to malformed airway structures but also to a dysfunctional micro vascular system. In healthy children the amount of alveoli increases until the age of about three and afterwards lung growth is based on the enlargement of preexistent alveoli. Therefore, postnatal development of lung tissue during childhood may be possible for children after CDH repair. Up to now, little is known to which extent the affected lung catches up.Current methods for perfusion weighted lung magnetic resonance imaging (MRI) are either based on dynamic contrast enhanced (DCE) MRI or arterial spin labeling (ASL) techniques. Although DCE-MRI offers much higher signal to noise ratio (SNR) than ASL techniques, the additional efforts in administering the contrast agent and its potential side-effects are disadvantages, especially in subjects with kidney disease (i.e. NSF). Recently, also a major debate has arisen which long-term side-effects may be associated to accumulation of gadolinium based contrast-agents in the brain. Therefore, especially in children the repetitive usage of contrast agent (e.g. for children with CDH enrolled into a follow-up program) should be evaluated critically and individually.With MRI lung perfusion and lung ventilation can be measured via the FD technique without the need for ionizing radiation. Our study group has demonstrated previously that DCE-measurements in 2-year old children after CDH are possible and detect side differences in lung perfusion values. Besides, in adult patients radiation free and contrast agent free quantification of perfusion and ventilation and thereby, V/Q imaging has been proven to be feasible by FD-techniques. Therefore, MRI is a suitable tool for follow-up of children after CDH, as being capable to visualize both lung morphology and lung function. The objectives of this research proposal is to implement the Fourier Decomposition technique for lung imaging in 2-year old children and to investigate the clinical value of the ventilation and perfusion maps for children with congenital diaphragmatic hernia. This comprises implementing 2D and 3D imaging techniques and post processing at 1.5T and its translation to 3T as well as correlation of the perfusion maps to DCE-MRI. Clinically, diagnostic value of the developed techniques, its power to detect changes in perfusion/ ventilation between contra- and ipsilateral lung and eventually describe implication of combined quantitative perfusion and ventilation imaging in CDH children on patient treatment and respectively follow-up procedure.

先天性腹股沟疝（CDH）导致腹腔器官疝入胸腔，从而导致肺发育受损，随后是肺发育不全。肺发育不良和肺动脉高压是CDH患者生存结局的主要决定因素，如果没有严重的其他畸形或综合征。肺发育不全不仅归因于畸形的气道结构，而且归因于功能障碍的微血管系统。在健康儿童中，肺泡的数量增加，直到大约三岁，之后肺的生长是基于先前存在的肺泡的扩大。因此，CDH修复后的儿童可能在儿童期出生后发育肺组织。目前肺磁共振灌注成像（perfusion weighted lung magnetic resonance imaging，MRI）的方法主要是基于动态增强（dynamic contrast enhanced，DCE）或动脉自旋标记（arterial spin labeling，ASL）技术。尽管DCE-MRI提供了比ASL技术高得多的信噪比（SNR），但在给予造影剂方面的额外努力及其潜在副作用是缺点，尤其是在患有肾脏疾病（即NSF）的受试者中。最近，还出现了一个主要的争论，即长期副作用可能与脑中钆基造影剂的积累有关。因此，特别是在儿童中，应严格和个别地评估造影剂的重复使用（例如，对于纳入随访计划的CDH儿童）。使用MRI，可以通过FD技术测量肺灌注和肺通气，而无需电离辐射。我们的研究小组先前已经证明，在CDH后的2岁儿童中进行DCE测量是可能的，并检测肺灌注值的侧差异。此外，在成人患者中，无辐射和无造影剂的灌注和通气定量，因此，已证明通过FD技术进行V/Q成像是可行的。因此，MRI是CDH后儿童随访的合适工具，因为它能够显示肺形态和肺功能。本研究的目的是在2岁儿童中实施肺成像的傅立叶分解技术，并研究通气和灌注图对先天性膈疝儿童的临床价值。这包括实施2D和3D成像技术和在1.5T下的后处理及其到3 T的转换以及灌注图与DCE-MRI的相关性。在临床上，所开发的技术的诊断价值，它的权力，以检测对侧和同侧肺之间的灌注/通气的变化，并最终描述了在CDH儿童的患者治疗和后续程序的联合定量灌注和通气成像的含义。