Skip to main content
SCMR 29th Annual Scientific Sessions Main banner
Final Program


Program-At-A-Glance

Late Breaking Clinical Trials

Virtual Recording

Session: Late Breaking Clinical Trials

Abdominal lymphatic derangement is associated with smaller conduits and protein-losing enteropathy in adolescent Fontan patients

Friday, February 6, 2026
11:30 AM - 11:40 AM  
Location: Asia IV and Hall 8 (3rd Floor)
Background: Abdominal lymphatic derangement is a prevalent complication of the Fontan circulation. We aimed to investigate the association between semi-quantitative magnetic resonance imaging (MRI)–derived abdominal lymphatic abnormality scores with clinical and hemodynamic features in adolescent Fontan patients. 

Methods: We retrospectively analyzed Fontan patients aged 10-18 years who underwent fasting cardiac–liver MRI between 2018 and 2022. Portal and retroperitoneal lymphatic abnormalities were graded on a 3-point scale, 1: normal to mild, 2: moderate, and 3: severe (Fig 1). Lymphatic grading and associations with clinical and laboratory parameters were assessed using Kruskal Wallis, Fisher exact probability and Spearman correlation tests. 

Results: Of 86 patients, 52 (60.5%) were male. The mean age at MRI was 14.1±0.3 years. The median duration from Fontan completion was 10.8 years [IQR 8.5-13.0]. Majority of the patients had grade 1 of the portal (42/86, 49%) and retroperitoneal (52/86, 61%) lymphatic grading with the median scores of 2 [IQR1-2] and 1 [IQR1-2], respectively. Six patients (7.0%) had a clinical history of protein-losing enteropathy (PLE) (Table 1). Higher portal and retroperitoneal lymphatic scores were associated with history of PLE (p=0.001 for both), presence of ascites (p< 0.001 for both), lower serum total protein levels (ρ=-0.198, p=0.025 and ρ=-0.483, p=0.001, respectively), and smaller body surface area (BSA) (ρ=-0.284, p=0.029 and ρ=-0.397, p=0.001); while higher retroperitoneal score was associated with smaller Fontan extracardiac conduit minimal diameter (ρ=-0.285, p=0.042) and cross-sectional area (ρ=-0.278, p=0.044) (Fig 2). No associations were observed with age or time from Fontan.

Conclusion: Worse abdominal lymphatic derangement is associated with smaller conduit sizes, presence of PLE and ascites, markers of impaired protein homeostasis, and decreased BSA in adolescent Fontan patients. These findings highlight the central role of lymphatic dysfunction in PLE pathogenesis with impacts on worse somatic growth. MRI lymphatic imaging may be a useful complementary noninvasive biomarker for risk stratification and consideration of potential conduit upsizing.

Table 1. Complete anatomical diagnoses, type of Fontan, and history of adverse events.

 

N (%)

Male (n=86)

52 (60.5)

Diagnosis (n=86)

 

     Tricuspid atresia

25 (29.1)

     Hypoplastic left heart syndrome

23 (26.7)

     Double inlet left ventricle

13 (15.1)

     Atrioventricular septal defect

9 (10.5)

     Double outlet right ventricle

9 (10.5)

     Pulmonary stenosis with hypoplastic right ventricle

3 (3.5)

     Pulmonary atresia-intact ventricular septum

2 (2.3)

     Pulmonary atresia-ventricular septal defect

1 (1.2)

     Double inlet right ventricle

1 (1.2)

Ventricular dominance

 

     Left

44 (51.2)

     Right

38 (44.2)

     Undetermine

3 (3.5)

Heterotaxy (n=86)

4 (4.7)

Type of Fontan (n=86)

 

     ECC

79 (91.9)

     Lateral tunnel

7 (8.1)

ECC size at implantation (n=79)

 

     16 mm

1 (1.2)

     20 mm

14 (17.3)

     22 mm

63 (77.8)

     24 mm

1 (1.2)

     Unknown

12 (2.5)

Clinical History (n=86)

     Arrhythmias

     PLE

     Plastic bronchitis

     Admission for chylothorax

     Thrombosis

     Stroke

     Chronic kidney disease

     Cirrhosis

     PLE, plastic bronchitis, or admission for chylothorax

 

11 (12.8)

6 (7)

1 (1.2)

4 (4.7)

6 (7)

2 (2.3)

2 (2.3)

2 (2.3)

11 (12.8)

MRI findings (n=86)

 

     Age at MRI (years) [mean±SD]

14.1±0.3

     Duration from Fontan completion (years) [median (IQR)]

10.8 (8.5-13.0)

     BSA (m2) [mean±SD]

1.45±0.0

     Portal lymphatic scores

 

            Grade 1

42 (48.8)

            Grade 2

39 (45.4)

            Grade 3

5 (5.8)

     Retroperitoneal lymphatic scores

 

            Grade 1

52 (60.5)

            Grade 2

30 (34.9)

            Grade 3

4 (4.6)

     ECC minimum diameter (mm) [mean±SD]

15.5±0.2

     ECC minimum cross-sectional area (mm2) [mean±SD]

242.2±5.4

BSA: body surface area; ECC: extracardiac conduit; MRI: magnetic resonance imaging; PLE: protein-losing enteropathy. Figure 1. Representative MRI images of portal (A-C) and retroperitoneal (D-F) lymphatic abnormality scores. A and D represent grade 1 normal to mild abnormalities, B and E represent grade 2 moderate abnormalities, and C and F represent grade 3 severe abnormalities.
Figure 2. Violin plots illustrating distribution of patient BSA at MRI (panel A and B), total serum protein (panel C and D), ECC minimal diameter (panel E), and ECC minimal cross sectional area (panel F), across lymphatic abnormality scores. Each point represents an individual data set, the lines represent median and Q1-Q3.