Diagnosis of occult diastolic dysfunction late after the Fontan procedure using a rapid volume expansion technique

Averin, Konstantin; Hirsch, Russel; Seckeler, Michael D.; Whiteside, Wendy; Beekman, Robert H.; Goldstein, Bryan H.

doi:10.1136/heartjnl-2015-309042

Cited by 52 publications

(32 citation statements)

References 30 publications

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“…Impedance to egress from the hepatic venous system may be related to specific flow characteristics downstream within the Fontan connection, unique to each individual. There are data to suggest that a fluid bolus at the time of catheterization may acutely increase Fontan pressure, and further study evaluating this relationship using computational flow dynamics and power loss analysis in combination with quantitative characterization of liver fibrosis may offer additional insights …”

Section: Discussionmentioning

confidence: 99%

Hepatic Fibrosis Is Universal Following Fontan Operation, and Severity is Associated With Time From Surgery: A Liver Biopsy and Hemodynamic Study

Goldberg

Surrey

Glatz

et al. 2017

JAHA

222

195

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BackgroundCongestive hepatopathy is a recognized complication of Fontan physiology. Data regarding the incidence of hepatopathy and risk factors are lacking.Methods and ResultsLiver biopsies and cardiac catherizations were performed as part of an evaluation offered to all patients ≥10 years after Fontan. Quantitative determination of hepatic fibrosis was performed using Sirius red staining with automated calculation of collagen deposition per slide (%CD). Biopsies from included subjects were compared to stained specimens from controls without known fibrotic liver disease. Patient characteristics, echocardiographic findings, and hemodynamic measures were evaluated as potential risk factors. The cohort consisted of 67 patients (31 female) at mean age of 17.3±4.5 years and mean time from Fontan of 14.9±4.5 years. Right ventricular morphology was present in 37 subjects. Median %CD by Sirius red staining was 21.6% (range 8.7% to 49.4%) compared to 2.6% (range 2.2% to 3.0%) in controls. There was a significant correlation between time from Fontan and degree of Sirius red staining (r=0.33, P<0.01). Serum liver enzymes and platelet count did not correlate with %CD. The median inferior vena cava pressure was 13 mm Hg (range 6‐24 mm Hg) and did not correlate with %CD. There was no difference in %CD based on ventricular morphology or severity of atrioventricular valve insufficiency.ConclusionsIn this cohort of predominantly asymptomatic children and adolescents electively evaluated after a Fontan operation, all exhibited evidence for hepatic fibrosis as measured by collagen deposition in the liver. Time from Fontan was the only factor significantly associated with collagen deposition. These findings demonstrate that liver fibrosis is an inherent feature of Fontan physiology and that the degree of fibrosis increases over time.

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Section: Discussionmentioning

confidence: 99%

Hepatic Fibrosis Is Universal Following Fontan Operation, and Severity is Associated With Time From Surgery: A Liver Biopsy and Hemodynamic Study

Goldberg

Surrey

Glatz

et al. 2017

JAHA

222

195

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“…These changes are likely associated with reduced vessel compliance and, consequently, diminished stressed volume of the venous vasculature. It is tempting to speculate that the recent observations of elevated Fontan pressure in response to intravenous volume challenge6 may in part reflect the diminished venous capacitance. Regional variations in vessel compliance may potentially contribute to turbulent flow and energy loss observed in these vessels at rest and particularly during exercise 21…”

Section: Discussionmentioning

confidence: 99%

“…Although late survival has improved over time,1patients continue to encounter morbidity. The mechanisms behind failure of the Fontan circulation remain poorly understood; some hypotheses as to primary drivers include abnormal ventriculoarterial coupling,2 elevated pulmonary vascular resistance,3 power loss within the Fontan circuit,4 ventricular preload deprivation,5 diastolic dysfunction6 and endothelial dysfunction 7–13…”

Section: Introductionmentioning

confidence: 99%

Histopathological abnormalities in the central arteries and veins of Fontan subjects

Hays

Baker

Laib

et al. 2017

Heart

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“…Diastolic dysfunction is often quoted as a late effect of the Fontan circulation [54][55][56][57][58][59][60][61], but it remains poorly understood and is likely to be multifactorial. Moreover, measurement of diastolic function using echocardiography may well be inaccurate in a circulation that is, by its nature, preload-depleted.…”

Section: Effects Of the Lack Of A Subpulmonary Ventriclementioning

confidence: 99%

Managing adult Fontan patients: where do we stand?

Clift

Celermajer

2016

Eur Respir Rev

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The Fontan operation is performed as a palliative procedure to improve survival in infants born with a functionally univentricular circulation. The success of the operation is demonstrated by a growing adult Fontan population that exists with this unique physiology. Late follow-up has demonstrated expected and unexpected sequelae, and has shown multisystem effects of this circulation. This review discusses the challenges of managing the late complications in terms of understanding this unique physiology and the innovative therapeutic interventions that are being investigated. The challenge remains to maintain quality of life for adult survivors, as well as extending life expectancy.

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Diagnosis of occult diastolic dysfunction late after the Fontan procedure using a rapid volume expansion technique

Cited by 52 publications

References 30 publications

Hepatic Fibrosis Is Universal Following Fontan Operation, and Severity is Associated With Time From Surgery: A Liver Biopsy and Hemodynamic Study

Hepatic Fibrosis Is Universal Following Fontan Operation, and Severity is Associated With Time From Surgery: A Liver Biopsy and Hemodynamic Study

Histopathological abnormalities in the central arteries and veins of Fontan subjects

Managing adult Fontan patients: where do we stand?

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