Haemodynamic profiles in adult Fontan patients: associated haemodynamics and prognosis

Published on Jun 1, 2019in European Journal of Heart Failure11.627
· DOI :10.1002/EJHF.1365
William R. Miranda14
Estimated H-index: 14
(Mayo Clinic),
Barry A. Borlaug76
Estimated H-index: 76
(Mayo Clinic)
+ 2 AuthorsAlexander C. Egbe20
Estimated H-index: 20
(Mayo Clinic)
Sources
Abstract
AIMS: The Fontan operation is associated with significant long-term morbidity and mortality, posing challenges in management. No haemodynamic classification has been established to aid management and prognostication of patients with Fontan palliation. Our aim was to assess haemodynamic profiles for Fontan patients and evaluate their relationships to prognosis. METHODS AND RESULTS: Eighty-four adult Fontan patients without obstruction or significant valve disease undergoing cardiac catheterization for Fontan-related co-morbidities were divided into four different haemodynamic profiles based on normal/high Fontan pressures (FP) (< 15 or ≥ 15 mmHg) and low/normal cardiac index (CI) (< 2.5 or ≥ 2.5 L/min/m2 ). Fourteen patients had low CI/high FP (17%), 24 low CI/normal FP (29%), 20 normal CI/high FP (24%), and 26 normal CI/normal FP (31%). Demographic and clinical data were similar among groups except for a trend towards a higher prevalence of cirrhosis in the normal CI/high FP (50%) group. Systemic vascular afterload, assessed by systemic vascular resistance and effective arterial elastance, was higher in patients with low CI/high FP and low CI/normal FP as compared to normal CI profiles. Pulmonary vascular resistance indices were higher in patients with low CI/high FP than all other profiles. The normal CI/high FP haemodynamic profile was an independent predictor of mortality (hazard ratio 4.1, 95% confidence interval 1.2-11.5). Heterotaxy and protein-losing enteropathy were also predictors of mortality on the multivariate model. CONCLUSION: The proposed haemodynamic classification provides prognostic information in adults post-Fontan with patients in the normal CI/high FP profile having worse survival. Whether this classification can guide management in these patients deserves further investigation.
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References30
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#1Phillip M. Trusty (Georgia Institute of Technology)H-Index: 10
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Last. Ajit P. Yoganathan (Georgia Institute of Technology)H-Index: 82
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Abstract Objective The staged Fontan procedure has shown promising short-term outcomes in patients with single ventricles. However, Fontan-associated liver disease is a marked problem as patients age. The purpose of this study is to investigate the relationship between hemodynamics and liver fibrosis in patients undergoing the Fontan. Methods A total of 33 patients undergoing the Fontan with liver fibrosis were included in this study. Cardiac magnetic resonance and phase-contrast cardiac magneti...
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#1Biagio Castaldi (UNIPD: University of Padua)H-Index: 13
#2Giulia Bordin (UNIPD: University of Padua)H-Index: 3
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INTRODUCTION: The Fontan procedure is the palliative procedure for single ventricle physiology. Pulmonary resistance plays a key role in the success of this operation. There are conflicting data concerning the impact of pulmonary vasodilators on survival and functional capacity among Fontan patients. AIM: The aim of this retrospective, single-center, nonrandomized study was to investigate the potential effect of pulmonary vasodilators on pulmonary vasculature in Fontan patients. METHOD: Twenty-s...
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#1Alexander C. Egbe (Mayo Clinic)H-Index: 20
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Background Nonpulsatile pulmonary blood flow in Fontan circulation results in pulmonary vascular disease, but the potential relationships between pulmonary vascular resistance index (PVRI) and Fontan failure have not been studied. The objective was to determine whether the absence of subpulmonary ventricle in the Fontan circulation would make patients more vulnerable to even low-level elevations in PVRI, and when coupled with low cardiac index, this would identify patients at increased risk of F...
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Background Elevated central venous pressure (CVP), low cardiac output, and mild hypoxia are common early and late after Fontan operations. However, the association of these characteristics with late mortality is unclear. We aimed to elucidate the hemodynamic determinants of mortality after Fontan operation. Method We evaluated early (group early; 0.5-5years postoperatively, n=387) and late (group late; ≥15years postoperatively, n=161) Fontan hemodynamics that included CVP (mm Hg), cardiac index ...
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Abstract Objectives To evaluate the effects of endothelin inhibitors (ERAs) on hemodynamic and functional parameters in patients post-Fontan procedure with high pulmonary vascular resistance (PVR). Methods Among our cohort of patients with Fontan circulation, 8 children, 8 adolescents, and 8 adults had PVR ≥2 WU*m 2 . These patients were treated with ERAs (minors with bosentan, adults with macitentan) and reevaluated after 6 months. Pre- and posttreatment hemodynamic variables were assessed by c...
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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 c...
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Background In adult Fontan patients, ventricular or arterial dysfunction may impact homeostasis of the Fontan circulation and predispose to heart failure. We sought to characterize ventricular‐arterial (VA) properties in adult Fontan patients. Methods and Results Adult Fontan patients (n=170), including those with right (SRV, n=57) and left (SLV, n=113) dominant ventricular morphology, were compared to age, sex, and body size matched controls (n=170). Arterial function, load‐insensitive measures...
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Background In heart failure, a high systemic vascular resistance index (SVRI), high central venous pressure (CVP), and low cardiac index (CI) predict poor outcomes. Conversely, late hemodynamic manifestations of failing Fontan circulation and associations with end-organ dysfunction are not well understood. Methods A retrospective review of right-heart catheterization data of adult Fontan patients between 2002 and 2014 was conducted. Relationships between hemodynamic variables and serious adverse...
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Background There are limited long-term, single-cohort, follow-up studies available about patients after the Fontan operation. Objectives This study sought to determine the long-term outcome of all patients who had a Fontan operation at the Mayo Clinic. Methods Records of all patients who had a modified Fontan operation between 1973 and 2012 were reviewed. A follow-up questionnaire was mailed to all patients alive at the time of the study. Results Overall, 10-, 20-, and 30-year survival for 1,052...
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