Imaging the Lymphatic System in Fontan Patients

Cate, Floris E.A. Udink ten; Tjwa, Eric T.

doi:10.1161/circimaging.119.008972

Cited by 10 publications

(3 citation statements)

References 18 publications

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“…12 Assuming that the Fontan circulation imposes a state of (subtle) chronic HF associated with chronic low-level systemic inflammation, increased vascular permeability, and increased production of interstitial fluid, the chronic challenge to the lymphatic system is evident, with poor drainage especially into the Fontan-specific high-pressure systemic venous system. 52 Besides lymphangiectasia, lymphatic collaterals form and tortuosity and dilation of the thoracic duct occur in Fontan patients; these interestingly have also been reported in children before Fontan surgery, but might be exacerbated by the Fontan circulation. 52 Lymphatic dysfunction can contribute to deterioration of organ function in Fontan patients, with lymphoedema, plastic bronchitis, PLE, and liver dysfunction.…”

Section: Discussionmentioning

confidence: 91%

“…52 Besides lymphangiectasia, lymphatic collaterals form and tortuosity and dilation of the thoracic duct occur in Fontan patients; these interestingly have also been reported in children before Fontan surgery, but might be exacerbated by the Fontan circulation. 52 Lymphatic dysfunction can contribute to deterioration of organ function in Fontan patients, with lymphoedema, plastic bronchitis, PLE, and liver dysfunction. The two latter disorders can be seen as major reasons for the alterations in PL, acylcarnitine, and HDL-C levels that we found; even if our patients exhibited no overt signs of PLE and even if lipidomic variables did not correlate with serum total protein or albumin levels, enteral lipid uptake might be altered, in combination with altered hepatic metabolism of lipids and of lipoproteins, which are important carriers of PL.…”

Section: Discussionmentioning

confidence: 91%

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Targeted metabolomic analysis of serum phospholipid and acylcarnitine in the adult Fontan patient with a dominant left ventricle

Michel

Dubowy

Zlamy

et al. 2020

Therapeutic Advances in Chronic Disease

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Background: Patients with a Fontan circulation have altered cholesterol and lipoprotein values. We analysed small organic molecules in extended phopsholipid and acylcarnitine metabolic pathways (‘metabolomes’) in adult Fontan patients with a dominant left ventricle, seeking differences between profiles in baseline and Fontan circulations. Methods: In an observational matched cross-sectional study, we compared phosphatidylcholine (PC), sphingomyelin (SM), and acylcarnitine metabolomes (105 analytes; AbsoluteIDQ® p180 kit (Biocrates Life Sciences AG, Innsbruck, Austria) in 20 adult Fontan patients having a dominant left ventricle with those in 20 age- and sex-matched healthy controls. Results: Serum levels of total PC ( q-value 0.01), total SM ( q-value 0.0002) were significantly lower, and total acylcarnitines ( q-value 0.02) were significantly higher in patients than in controls. After normalisation of data, serum levels of 12 PC and 1 SM Fontan patients were significantly lower ( q-values <0.05), and concentrations of 3 acylcarnitines were significantly higher than those in controls ( q-values <0.05). Conclusion: Metabolomic profiling can use small specimens to identify biomarker patterns that track derangement in multiple metabolic pathways. The striking alterations in the phospholipid and acylcarnitine metabolome that we found in Fontan patients may reflect altered cell signalling and metabolism as found in heart failure in biventricular patients, chronic low-level inflammation, and alteration of functional or structural properties of lymphatic or blood vessels. Trial registration number: ClinicalTrials.gov Identifier NCT03886935

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Section: Discussionmentioning

confidence: 91%

Section: Discussionmentioning

confidence: 91%

Targeted metabolomic analysis of serum phospholipid and acylcarnitine in the adult Fontan patient with a dominant left ventricle

Michel

Dubowy

Zlamy

et al. 2020

Therapeutic Advances in Chronic Disease

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“…1,[3][4][5][6] Lymphopenia in Fontan patients is common among those with protein-losing enteropathy or plastic bronchitis which rare complications hypothesised to be related to a lymphatic leak from the gastrointestinal or bronchial tract. [7][8][9] However, recent reports suggest a high prevalence of lymphopenia even in Fontan patients in the absence of a manifest lymphatic leak syndrome. 10 Further, lymphopenia appears to be associated with lower platelet count, suggesting a relationship with splenomegaly and Fontan-associated liver disease commonly seen in Fontan patients secondary to chronically elevated central venous pressure.…”

Section: Introductionmentioning

confidence: 99%

Lymphopenia in adults after the Fontan operation: prevalence and associations

et al. 2020

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Lymphopenia is common in adults who have had a Fontan operation although its aetiology and clinical implications remain unknown. Previous work suggests an association between lymphopenia and both liver disease and splenomegaly. The objective of this study was to assess the prevalence of lymphopenia in adults with a Fontan circulation and evaluate its associations with risk factors and clinical outcomes. Using a retrospective cohort study design, we studied 73 adult Fontan patients (age 25.0 ± 8.4 years) who had a complete blood count and abdominal imaging performed. Patients with protein-losing enteropathy were excluded. Clinical data were extracted from hospital records. The mean white blood cell count was 6580 ± 220/ml with a mean lymphocyte count of 1223 ± 508/ml. Lymphopenia, defined as lymphocyte count <1000/ml, was present in 23 (32%) patients. Patients with lymphopenia had a lower total white blood cell count (5556 ± 2517 versus 7136 ± 1924/ml, p = 0.009) and a lower platelet count (162 ± 69 versus 208 ± 69 k/ml, p = 0.008). Lymphopenia was also associated with findings of portal hypertension, including splenomegaly (36 versus 14%, p = 0.04), varices (22 versus 6%, p = 0.04), and ascites (39 versus 14%, p = 0.02). Lymphopenia did not correlate with any cardiac imaging, haemodynamic or exercise testing variables. In conclusion, lymphopenia is common in adult Fontan patients and is associated with markers of portal hypertension. Larger studies are needed to better define the relationship between lymphopenia and clinical outcomes.

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Cardiac lymphatics and cardiac lymph flow in health and disease

Alexander

Yun

2020

Lymphatic Structure and Function in Health and Disease

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Imaging the Lymphatic System in Fontan Patients

Cited by 10 publications

References 18 publications

Targeted metabolomic analysis of serum phospholipid and acylcarnitine in the adult Fontan patient with a dominant left ventricle

Targeted metabolomic analysis of serum phospholipid and acylcarnitine in the adult Fontan patient with a dominant left ventricle

Lymphopenia in adults after the Fontan operation: prevalence and associations

Cardiac lymphatics and cardiac lymph flow in health and disease

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