Purpose
To evaluate lymphatic abnormalities before and after Fontan completion
using noncontrast lymphatic imaging and relate findings with
postoperative outcomes.
Materials and Methods
This study is a retrospective review of noncontrast T2-weighted lymphatic
imaging performed at The Children's Hospital of Philadelphia from
June 2012 to February 2023 in patients with single ventricle physiology.
All individuals with imaging at both pre-Fontan and Fontan stages were
eligible. Lymphatic abnormalities were classified into four types based
on severity and location of lymphatic vessels. Classifications were
compared between images and related to clinical outcomes such as
postoperative drainage and hospitalization, lymphatic complications,
heart transplant, and death.
Results
Forty-three patients (median age, 10 years [IQR, 8–11]; 20 [47%]
boys, 23 [53%] girls) were included in the study. Lymphatic
abnormalities progressed in 19 individuals after Fontan completion
(distribution of lymphatic classifications: type 1, 23; type 2, 11; type
3, 6; type 4, 3 vs type 1, 10; type 2, 18; type 3, 10; type 4, 5;
P
= .04). Compared with individuals showing no
progression of lymphatic abnormalities, those progressing to a
high-grade lymphatic classification had longer postoperative drainage
(median time, 9 days [IQR, 6–14] vs 17 days [IQR, 10–23];
P
= .04) and hospitalization (median time, 13 days
[IQR, 9–25] vs 26 days [IQR, 18–30];
P
=
.03) after Fontan completion and were more likely to develop chylothorax
(12% [three of 24] vs 75% [six of eight];
P
<
.01) and/or protein-losing enteropathy (0% [0 of 24] vs 38% [three of
eight];
P
< .01) during a median follow-up of 8
years (IQR, 5–9). Progression to any type was not associated with
an increased risk of adverse events.
Conclusion
The study demonstrated that lymphatic structural abnormalities may
progress in select individuals with single ventricle physiology after
Fontan completion, and progression of abnormalities to a high-grade
classification was associated with worse postoperative outcomes.
Keywords:
Congenital Heart Disease, Glenn, Fontan, Lymphatic
Imaging, Cardiovascular MRI
Supplemental material is available for this
article.
Published under a CC BY 4.0 license.