T2-Weighted MRI following TCPC: Patients with Higher-Grade Changes of Intestinal Lymphatic Vessels Were Younger at SCPC and Have a More Frequent History of Chylothorax

Research is increasingly focusing on pathologies of the lymphatic vessels in patients after TCPC. In this study, we focused on the intestinal lymphatic vessels. We tried to find out if clinical and laboratory parameters are associated with higher-grade changes after TCPC.

We prospectively examined 33 patients aged 19.8 years (14.6; 30.2) [median (Q1; Q3)] after TCPC (follow-up 14.3 years (9.7; 24.9) with a heavily T2-weighted MRI sequence on a 3.0-T scanner. Examinations in coronal orientation were performed with respiratory gating, slice thickness 0.6 mm, TR 2,400 ms, TE 692 ms, FoV 460 mm (covering thoracic and abdominal regions), scan time 14:41 minutes (13:18; 16:30) after a solid meal and a cup of pineapple juice. The findings were classified according to delineation of intestinal lymphatic vessels. Type 1: ≤3 intestinal vessels definable; type 2: 4 to 6 intestinal vessels definable; type 3: >6 intestinal vessels/edematous changes or ascites. The findings were correlated with parameters obtained at the annual routine check-up. Statistical analysis was performed using U-test and chi-square test.

Fifteen patients (group 1) showed type 3 lymphatic abnormalities, of which two had ascites. Eighteen patients (group 2) showed lower grade morphologies (types 1–2). Image quality was assessed based on the ability to delineate the common hepatic duct and did not differ between groups (p = 0.134). Patients in group 1 were younger at MRI examination (17.4; 14.3/18.9 vs. 26.2; 18.2/32.3 years, p = 0.03). SCPC (superior cavopulmonary connection) was performed earlier in group 1 (9.9; 7.9/25.5 vs. 29.2; 13.7/66.6 months, p = 0.018). Group 1 patients showed lower levels for IgG (833; 241/1,170 vs. 1,285; 1,153/1,654 mg/dL, p = 0.02), Lipase (30; 25/34 vs. 38; 34.8/48.8 U/L, p = 0.005), a-Antitrypsin (165; 144/179 vs. 136.5/123/149 mg/dL, p = 0.018), Cystatin C 1.0; 1.0/1.2 vs. 0.95; 0.8/1.3 mg/L, p = 0.023) and TSH (3.33; 2.5/4.3 vs. 2.3; 1.6/3.4 mU/L, p = 0.043). There were no significant differences regarding total protein, NTproBNP, lymphocytes or platelets. A history of chylothorax was present in 7/15 versus 2/18, p = 0.022. PLE occurred in 4/15 versus 1/18, p = 0.092.

In the long-term follow-up after TCPC, patients with more pronounced changes of the intestinal lymphatic vessels were younger at SCPC, and showed more frequently a history of chylothorax and lower IgG values.

Publication History

Article published online:
28 January 2023