Histopathological abnormalities in the central arteries and veins of Fontan subjects

Hays, Brandon; Baker, Michael; Laib, Annie; Tan, Wei; Udholm, Sebastian; Goldstein, Bryan H.; Sanders, Stephen P.; Opotowsky, Alexander R.; Veldtman, Gruschen

doi:10.1136/heartjnl-2017-311838

Cited by 15 publications

(4 citation statements)

References 29 publications

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“…In histopathologic investigations of Fontan patients with a mean age of 30 years, mural remodelling has been demonstrated in the pulmonary artery and in the inferior and superior vena cava. 61 Moreover, in the course of their signalling function, PL regulate angiogenesis, possibly linking their concentrations in serum with altered collateral-vessel formation. 37 A decrease in PL concentrations as measured might mean that PL are present in another form, an activated or oxidised one, which in our assay was not determined.…”

Section: Alteration Of Structural or Functional Lymphaticvessel Or Blmentioning

confidence: 99%

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: Alteration Of Structural or Functional Lymphaticvessel Or Blmentioning

confidence: 99%

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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“…For the past decade, pulmonary vascular disease has been suspected to be the primary mechanism for late Fontan failure. A study based on autopsies described a unique remodeling of the pulmonary arteries with intimal hyperplasia and media regression in patients with late failing Fontan, which has been attributed to long-term non-pulsatile low-shear flow of the Fontan circulation [32,33]. Consequently, most randomized control trials assessing drug effects in Fontan patients have targeted PVR with limited and controversial outcomes.…”

Section: Failing Fontanmentioning

confidence: 99%

Cardiac Drugs in ACHD Cardiovascular Medicine

Ladouceur

Valdeolmillos

Karsenty

et al. 2023

JCDD

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Adult congenital heart disease (ACHD) is a growing population that requires life-long care due to advances in pediatric care and surgical or catheter procedures. Despite this, drug therapy in ACHD remains largely empiric due to the lack of clinical data, and formalized guidelines on drug therapy are currently lacking. The aging ACHD population has led to an increase in late cardiovascular complications such as heart failure, arrhythmias, and pulmonary hypertension. Pharmacotherapy, with few exceptions, in ACHD is largely supportive, whereas significant structural abnormalities usually require interventional, surgical, or percutaneous treatment. Recent advances in ACHD have prolonged survival for these patients, but further research is needed to determine the most effective treatment options for these patients. A better understanding of the use of cardiac drugs in ACHD patients could lead to improved treatment outcomes and a better quality of life for these patients. This review aims to provide an overview of the current status of cardiac drugs in ACHD cardiovascular medicine, including the rationale, limited current evidence, and knowledge gaps in this growing area.

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“…Primary left-right-shunt lesions, congenital obstructions of the left heart, cyanotic heart defects with increased pulmonary flow, and anomalies of the pulmonary artery are the most frequent lesions which can be complicated by a P(A)H, and in which P(A)H may considerably impair the quality and duration of life. Another group includes patients with univentricular hearts after Fontan operation that can develop pulmonary vascular disease (28)(29)(30).…”

Section: Pulmonary Hypertension and Pulmonary Arterial Hypertension Imentioning

confidence: 99%

Improving medical care and prevention in adults with congenital heart disease—reflections on a global problem—part II: infective endocarditis, pulmonary hypertension, pulmonary arterial hypertension and aortopathy

Neidenbach¹,

Niwa²,

Oto³

et al. 2018

Cardiovasc. Diagn. Ther

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Despite relevant residua and sequels, follow-up care of adults with congenital heart disease (ACHD) is too often not performed by/in specialized and/or certified physicians or centers although major problems in the long-term course may develop. The most relevant encompass heart failure, cardiac arrhythmias, heart valve disorders, pulmonary vascular disease, infective endocarditis (IE), aortopathy and non-cardiac comorbidities. The present publication emphasizes current data on IE, pulmonary and pulmonary arterial hypertension and aortopathy in ACHD and underlines the deep need of an experienced follow-up care by specialized and/or certified physicians or centers, as treatment regimens from acquired heart disease can not be necessarily transmitted to CHD. Moreover, the need of primary and secondary medical prevention becomes increasingly important in order to reduce the burden of disease as well as the socioeconomic burden and costs in this particular patient group.

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Histopathological abnormalities in the central arteries and veins of Fontan subjects

Cited by 15 publications

References 29 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

Cardiac Drugs in ACHD Cardiovascular Medicine

Improving medical care and prevention in adults with congenital heart disease—reflections on a global problem—part II: infective endocarditis, pulmonary hypertension, pulmonary arterial hypertension and aortopathy

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