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. 2025 Jul 1;25(1):229.
doi: 10.1186/s12880-025-01803-0.

Imaging and evaluation of cervicothoracic lymphatic drainage pathways in single ventricle patients with Fontan circulation using the mDixon steady state MR angiography

Monika Huhndorf #  1 , Patrick Langguth #  1 , Hatim Seoudy  2   3 , Olav Jansen  1 , Anselm Uebing  4   3 , Jan Hinnerk Hansen  4   3 , Inga Voges  5   6
Affiliations

Imaging and evaluation of cervicothoracic lymphatic drainage pathways in single ventricle patients with Fontan circulation using the mDixon steady state MR angiography

Monika Huhndorf et al. BMC Med Imaging. .

Abstract

Background: Single ventricle (SV) Fontan patients are at risk for the development of pathological cervicothoracic lymphatic drainage pathways that are involved in the development of serious conditions such as plastic bronchitis or chylothorax. Visualization and categorization of cervicothoracic lymphatic drainage pathways might therefore help to stratify prognosis and to individualize therapy and follow-up for Fontan patients. This study aimed to show that the 3-dimensional (3D) modified Dixon (mDixon) steady state magnetic resonance (MR) angiography, commonly used to image cardiovascular anatomy, can visualize cervicothoracic lymphatic drainage pathways in high resolution in Fontan patients.

Methods: 3D mDixon steady state MR angiography of 88 pediatric and young adult patients with a Fontan circulation were retrospectively analysed. The pattern of cervicothoracic lymphatic pathways and image quality according to diagnostic value were assessed. Furthermore, ventricular volumes, mass and ejection fraction from cine imaging were measured.

Results: Image quality was assessed as very good or good in > 90% of the cases. Six patients had a lymphatic complication of which five (83.3%) had a higher cervicothoracic lymphatic pathway type (type 3 or 4).

Conclusions: 3D mDixon steady state MR angiography is an established method to assess cardiovascular anatomy and function in Fontan patients. The method also allows to visualize and evaluate the cervicothoracic lymphatic anatomy with high image quality. 3D mDixon steady state MR angiography is therefore particularly useful to comprehensively assess Fontan patients, a patient group prone not only to cardiac but also lymphatic failure.

Keywords: 3D mDixon steady state magnetic resonanc angiography; Fontan circulation; Lymphatic vessels; Single ventricle.

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Conflict of interest statement

Declarations. Ethics approval and consent to participate: The study has been performed in accordance with the Declaration of Helsinki and has been approved by the ethics committee of the medical faculty of the Kiel University (CAU) (date: 20/11/2023, reference number: AZ D623/23). A general informed consent was obtained from all parents or legal guardians, as appropriate. Consent for publication: NA. Competing interests: The authors declare no competing interests.

Figures

Fig. 1
Fig. 1
Types of cervicothoracic lymphatic abnormalities. The thoracic duct is marked with white arrows, while lymphatic abnormalities as described as type 1–4 are marked with white stars. (A) type 1 - little or no presumed lymphatic channels within the supraclavicular region and mediastinum, (B) type 2 - abnormally increased lymphatic channels within the supraclavicular region without extension into the mediastinum, (C) type 3 – abnormal supraclavicular lymphatics with extension into the mediastinum and (D) type 4 - abnormal supraclavicular lymphatics with extension into the mediastinum and in an interstitial pattern into the lung parenchyma according to Biko et al. [3]
See this image and copyright information in PMC

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