Thorac Cardiovasc Surg 2017; 65(S 02): S111-S142
DOI: 10.1055/s-0037-1598989
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Georg Thieme Verlag KG Stuttgart · New York

Feasibility of Dynamic Contrast-Enhanced Magnetic Resonance Lymphangiography (DCMRL) in Fontan Patients

A. Steif
1   Department of Pediatric Cardiology, University Hospital Erlangen, Erlangen, Germany
,
J. Moosmann
1   Department of Pediatric Cardiology, University Hospital Erlangen, Erlangen, Germany
,
R. Cesnjevar
2   Department of Pediatric Cardiac Surgery, University Hospital Erlangen, Erlangen, Germany
,
A. Rüffer
2   Department of Pediatric Cardiac Surgery, University Hospital Erlangen, Erlangen, Germany
,
O. Rompel
3   Department of Radiology, University Hospital Erlangen, Erlangen, Germany
,
A. Schmid
3   Department of Radiology, University Hospital Erlangen, Erlangen, Germany
,
M. Glöckler
1   Department of Pediatric Cardiology, University Hospital Erlangen, Erlangen, Germany
,
S. Dittrich
1   Department of Pediatric Cardiology, University Hospital Erlangen, Erlangen, Germany
› Author Affiliations
Further Information

Publication History

Publication Date:
02 February 2017 (online)

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Objective: We report four Fontan patients in whom we have performed the new technique of dynamic contrast-enhanced MR-lymphangiography (DCMRL) since April 2016. Imbalanced lymphatic homeostasis is discussed as an important factor for developing a failing Fontan circulation. It seems to play a role in developing plastic bronchitis, persistent chylothorax and protein-losing enteropathy. “Conventional” MR (T2-weighted MRI) can detect lymphangiectasis in many patients with Fontan circulation. However, the lymphatic flow cannot be demonstrated using this technique. In this study, we present first results of dynamic contrast-enhanced magnetic resonance lymphangiography.

Methods: Inguinal lymphatic nodes are punctured ultrasound guided with 22–26G. 1 to 4 mL Gadovist or Dotarem are injected by hand into each node at a rate of 0.5 to 1 mL/h. SCAN: T1w VIBE fs coronal and transversal (thorax and abdomen, repetitive) and flash 3D-Sequences.

Table 1

Patient data

Patient no.

Sex

Age [y]

Age at last surgery [y]

Diagnosis

Surgical repair

Symptoms

1

m

6

4.5

PA + VSD (restr.)

TCPC extracard.

Pleural effusions (PLE), edema

2

f

32.4

15.4

TA + VSD

TCPC initial intracard., secondary extracard.

Protein-losing enteropathy

3

f

13.7

8.4

DILV

TCPC extracard.

PLE, edema

4

m

14.7

3.9

HLHS

TCPC extracard.

Protein-losing enteropathy

Results: In all patients the “conventional” T2-MRI showed lymphangiectasis. DCMRL showed detailed pathology in three patients: (1) In P1 retrograde lymphatic flow toward right pleura with effusions in right pleura. (2) In P2 reduced lymphatic flow inguinal bilateral. 3) In P3 Truncus lumbalis with normal size, no lymphatic flow toward cisterna chyli in the thoracic duct. In one patient (P4) occlusion of lymph drainage could be ruled out.

Conclusion: With DCMRL more detailed information can be obtained concerning the lymphatic flow which seems to be important in developing pathologies in Fontan circulation. The method is feasible but further investigation is necessary.