Abstract
Background
Patients with Fontan circulation are known to have increased systemic vascular resistance
(SVR) however the underlying mechanisms are uncertain. We therefore further investigated
the haemodynamic and vascular profile of Fontan patients.
Methods
Eighteen adult subjects aged 25±1 years who had undergone the Fontan procedure in their childhood (at age 6±1 years) and not in clinical failure at the time of study were assessed for: 1) autonomic
function, including direct muscle sympathetic nerve activity (MSNA) recording and
sympathetic and cardiac baroreflex function, 2) endothelial function by means of reactive
hyperaemia using the Endopat peripheral arterial tonometry (PAT) technique and plasma
endothelin concentration and gene expression, 3) pulse wave reflections (digital and
central augmentation index (AI)) and 4) haemodynamic changes to head-up tilt. Data
were compared to that obtained in a group of 23 age- and weight-matched healthy subjects.
Results
Fontan participants presented with elevated MSNA compared with controls (40±5 vs 27±3 bursts per 100 heartbeats), decreased cardiac baroreflex function (16.0±3.3 versus 30.9±3.7 ms·mm Hg−1), normal sympathetic baroreflex function, decreased endothelial function (PAT ratio=0.35±0.09 vs 0.77±0.11), and increased digital (5.9±3.0% vs −9.7±2.3%) and central (1.4±2.7% vs −10.2±3.9%) AI. Ten minute head-up tilt (60°) induced greater reductions in cardiac output
(CO) and stroke volume (SV) in Fontan patients (CO: −28% vs −11%, SV: −40% vs −25%).
Conclusion
Adult Fontan patients have increased MSNA and altered endothelial function that are
likely to contribute to their known increased SVR. Therapies aiming at reducing the
peripheral resistances should target endothelial function and sympathetic activity.
Keywords
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Article info
Publication history
Published online: April 23, 2012
Accepted:
April 1,
2012
Received:
March 2,
2012
Identification
Copyright
© 2012 Elsevier Ireland Ltd. Published by Elsevier Inc. All rights reserved.
