Congenital heart disease

Quantification of lymphatic burden in patients with Fontan circulation by T2 MR lymphangiography and associations with adverse Fontan status

Eur Heart J Cardiovasc ImagingPublished Retrospective cohort study

Authors' conclusion

Quantification of MR lymphatic burden is a reliable tool to assess the lymphatics post-Fontan and is associated with clinical status.

Study context

Population
Paediatric Fontan patients (<18 years) with clinical cardiac MRI including lymphatic 3D T2 fast spin echo (FSE) imaging, May 2017–October 201927 males
Participants
48 patients
Exposure
Greater vs lower MR lymphatic burden
Outcome
Lymphatic burden (thresholding-based segmentation of 3D T2 FSE MIP image, indexed to body surface area) and its association with clinical status
Methodological detailsblinded independent readers, Median age at MRI 12.9 (9.4-14.7) years; time from Fontan to MRI 9.1 (5.9-10.4) years; follow-up post-Fontan 9.4 (6.6-11.0) years
  • Blinded independent readers
  • Median age at MRI 12.9 (9.4-14.7) years; time from Fontan to MRI 9.1 (5.9-10.4) years; follow-up post-Fontan 9.4 (6.6-11.0) years

Main finding

As reported in the abstract

Correlation with post-Fontan hospital length of stay
rs = 0.416, P = 0.004
Correlation with duration of chest tube drainage
rs = 0.439, P = 0.002
Median lymphatic burden, chylous effusions immediately post-Fontan vs none
178 (118-393) vs. 113 (46-190) mL/m², P = 0.028
Median lymphatic burden, composite adverse Fontan status (n = 13) vs none
435 (137-822) vs. 114 (51-178) mL/m², P = 0.003
Lymphatic burden > 600 mL/m² for late adverse Fontan status: sensitivity
57%
Lymphatic burden > 600 mL/m² for late adverse Fontan status: specificity
95%

Observational data: associations, not effects.

Further findings

Interobserver reliability of lymphatic burden

Intraclass correlation coefficient
0.96 (0.94-0.98)

Original publication

Quantification of lymphatic burden in patients with Fontan circulation by T2 MR lymphangiography and associations with adverse Fontan status. Eur Heart J Cardiovasc Imaging. Published 3 November 2022. doi:10.1093/ehjci/jeac216

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