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

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.