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. 2024 Jun;34(6):3773-3785.
doi: 10.1007/s00330-023-10395-8. Epub 2023 Nov 20.

Long-term pulmonary outcome of children with congenital diaphragmatic hernia: functional lung MRI using matrix-pencil decomposition enables side-specific assessment of lung function

Carmen Streibel  1   2 , C Corin Willers  3   4 , Grzegorz Bauman  5   6 , Orso Pusterla  7   5   6 , Oliver Bieri  5   6 , Marion Curdy  7 , Matthias Horn  7 , Carmen Casaulta  7 , Steffen Berger  8 , Gabriela Marta Dekany  8 , Elisabeth Kieninger  7 , Andreas Bartenstein #  8 , Philipp Latzin #  9
Affiliations

Long-term pulmonary outcome of children with congenital diaphragmatic hernia: functional lung MRI using matrix-pencil decomposition enables side-specific assessment of lung function

Carmen Streibel et al. Eur Radiol. 2024 Jun.

Abstract

Objectives: In patients with congenital diaphragmatic hernia (CDH) the exact functional outcome of the affected lung side is still unknown, mainly due to the lack of spatially resolved diagnostic tools. Functional matrix-pencil decomposition (MP-) lung MRI fills this gap as it measures side-specific ventilation and perfusion. We aimed to assess the overall and side-specific pulmonary long-term outcomes of patients with CDH using lung function tests and MP-MRI.

Methods: Thirteen school-aged children with CDH (seven with small and six with large defect-sized CDH, defined as > 50% of the chest wall circumference being devoid of diaphragm tissue) and thirteen healthy matched controls underwent spirometry, multiple-breath washout, and MP-MRI. The main outcomes were forced expiratory volume in 1 second (FEV1), lung clearance index (LCI2.5), ventilation defect percentage (VDP), and perfusion defect percentage (QDP).

Results: Patients with a large CDH showed significantly reduced overall lung function compared to healthy controls (mean difference [95%-CIadjusted]: FEV1 (z-score) -4.26 [-5.61, -2.92], FVC (z-score) -3.97 [-5.68, -2.26], LCI2.5 (TO) 1.12 [0.47, 1.76], VDP (%) 8.59 [3.58, 13.60], QDP (%) 17.22 [13.16, 21.27]) and to patients with a small CDH. Side-specific examination by MP-MRI revealed particularly reduced ipsilateral ventilation and perfusion in patients with a large CDH (mean difference to contralateral side [95%-CIadjusted]: VDP (%) 14.80 [10.50, 19.00], QDP (%) 23.50 [1.75, 45.20]).

Conclusions: Data indicate impaired overall lung function with particular limitation of the ipsilateral side in patients with a large CDH. MP-MRI is a promising tool to provide valuable side-specific functional information in the follow-up of patients with CDH.

Clinical relevance statement: In patients with congenital diaphragmatic hernia, easily applicable MP-MRI allows specific examination of the lung side affected by the hernia and provides valuable information on ventilation and perfusion with implications for clinical practice, making it a promising tool for routine follow-up.

Key points: • Functional matrix pencil decomposition (MP) MRI data from a small sample indicate reduced ipsilateral pulmonary ventilation and perfusion in children with large congenital diaphragmatic hernia (CDH). • Easily applicable pencil decomposition MRI provides valuable side-specific diagnostic information on lung ventilation and perfusion. This is a clear advantage over conventional lung function tests, helping to comprehensively follow up patients with congenital diaphragmatic hernia and monitor therapy effects.

Keywords: Children; Congenital diaphragmatic hernias; Functional magnetic resonance imaging; Lung; Pulmonary function test.

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Conflict of interest statement

Elisabeth Kieninger reports personal fees from Sanofi-Aventis outside the submitted work.

Philipp Latzin reports personal fees from Gilead, Novartis, OM Pharma, Polyphor, Roche, Santhera, Schwabe, Vertex, Vifor, Zambon, and grants from Vertex, all outside the submitted work.

All other authors of this manuscript declare no relationships with any companies, whose products or services may be related to the subject matter of the article.

Figures

Fig. 1
Fig. 1
Lung function in healthy controls and patients with small and large CDH. Individual values in (A) FEV1 (z-score), (B) FVC (z-score), (C) FEV1/FVC (%), (D) TLC (z-score), (E) RV/ TLC (%), (F) LCI2.5 (TO). The group level is presented as grey boxplots (median, lower, and upper quartile, whiskers extending to at most 1.5*interquartile range). p values are the results of post hoc analysis of one-way ANOVA and are corrected for multiple testing. small CDH: congenital diaphragmatic hernia with primary closure; large CDH: congenital diaphragmatic hernia with patch or flap repair; FEV1: forced expiratory volume in 1 second; FVC: forced vital capacity; TLC: total lung capacity; RV: residual volume; LCI2.5: Lung clearance index, measured at classical end of nitrogen multiple-breath washout (2.5% of the normalized nitrogen starting concentration); TO: turnover (raw unit of LCI)
Fig. 2
Fig. 2
MP-MRI parameters in healthy controls and patients with small and large CDH. Individual values in (A) VDP (%), (B) QDP (%), (C) VQDmatch (%), (D) DDIV (arb. unit), and (E) DDIQ (arb. unit). The group level is presented as grey boxplots (median, lower, and upper quartile, whiskers extending to at most 1.5*interquartile range). p values are results of post-hoc analysis of one-way ANOVA and corrected for multiple testing. MP-MRI: Matrix pencil decomposition magnetic resonance imaging; small CDH: congenital diaphragmatic hernia with primary closure; large CDH: congenital diaphragmatic hernia with patch or flap repair; VDP: percentage of the lung volume with impaired fractional ventilation; QDP: percentage of the lung volume with impaired relative perfusion; VQDmatch: matched defect in perfusion and ventilation (in percent); DDIV: defect distribution index of ventilation; DDIQ: defect distribution index of perfusion; DP: defect percentage
Fig. 3
Fig. 3
MP-MRI parameters in healthy controls and patients with small and large CDH, separated by lung side. Individual values for the ipsilateral lung side (affected) and the contralateral lung side (non-affected) in (A) VDP (%), (B) QDP (%), (C) VQDmatch (%), (D) DDIV (arb. unit) and (E) DDIQ (arb. unit). In healthy controls, the left lung side was compared to the right lung side. The group level is presented as grey boxplots (median, lower, and upper quartile, whiskers extending to at most 1.5*interquartile range). p values are the results of post hoc analysis of two-way repeated measures ANOVA and corrected for multiple testing. MP-MRI: Matrix pencil decomposition magnetic resonance imaging; small CDH: congenital diaphragmatic hernia with primary closure; large CDH: congenital diaphragmatic hernia with patch or flap repair; VDP: percentage of the lung volume with impaired fractional ventilation; QDP: percentage of the lung volume with impaired relative perfusion; VQDmatch: matched defect in perfusion and ventilation (in percent); DDIV: defect distribution index of ventilation; DDIQ: defect distribution index of perfusion; DP: defect percentage; non aff.: non-affected, contralateral lung side; aff.: affected, ipsilateral lung side
Fig. 4
Fig. 4
Example of MP-MRI images in (A) a healthy control, (B) a patient with a small CDH, and (C) a patient with a large CDH. A: 11.9-year-old, healthy boy (VDPleft (%) = 8.42, VDPright (%) = 10.92, QDPleft (%) = 6.18, QDPright (%) = 7.24). B: 8.7-year-old, female patient with small CDH (primary closure of the diaphragmatic defect) on the anatomically left side, marked by white arrow (VDPaffected (%) = 8.26, VDPnon-affected (%) = 7.39, QDPaffected (%) = 9.97, QDPnon-affected (%) = 6.47). C: 9.9-year-old, female patient with large CDH (flap repair of the diaphragmatic defect) on the anatomically left side, marked by white arrow (VDPaffected (%) = 17.74, VDPnon-affected (%) = 7.84, QDPaffected (%) = 32.11, QDPnon-affected (%) = 1.53). For each case study, morphological images are given in (a). Overlaid on morphological images are: fractional ventilation maps (b), relative perfusion maps (c), and masks representing areas with impaired ventilation and impaired perfusion (d, e). On the heat maps, a change of colour range towards dark blue indicates severe impairment of lung function. Further individual outcome values are provided in Supplemental Table S6. CDH: congenital diaphragmatic hernia; MP-MRI: Matrix pencil decomposition magnetic resonance imaging; VDP: percentage of the lung volume with impaired fractional ventilation; QDP: percentage of lung volume with impaired relative perfusion; non aff.: non-affected, contralateral lung side; aff.: affected, ipsilateral lung side
Fig. 5
Fig. 5
Association of lung function and MP-MRI outcomes of the lung side affected in patients with CDH. Individual values plotted by (A) FEV1 (z-score) and VDP (%), (B) FEV1 (z-score) and QDP (%), (C) FEV1 (z-score) and VQDmatch (%), (D) RV/TLC and VDP (%), (E) RV/TLC and QDP (%), and (F) RV/TLC and VQDmatch (%). Values of both, patients with a small CDH (primary closure of the diaphragmatic defect) and a large CDH (patch or flap repair of the diaphragmatic defect) are included. Lines represent simple linear regression (ρ = Spearman’s correlation coefficient). The Benjamini-Hochberg procedure has been applied to correct p-values for multiple comparisons. MP-MRI: Matrix pencil decomposition magnetic resonance imaging; CDH: congenital diaphragmatic hernia; FEV1: forced expiratory volume in 1 second; VDP: percentage of the lung volume with impaired fractional ventilation; QDP: percentage of lung volume with impaired relative perfusion; VQDmatch: matched defect in perfusion and ventilation (in percent); aff.: affected, ipsilateral lung side; RV: residual volume; TLC: total lung capacity
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