Ventricular‐Arterial Function and Coupling in the Adult Fontan Circulation

Saiki, Hiroshi; Eidem, Benjamin W.; Ohtani, Tomohito; Grogan, Martha; Redfield, Margaret M.

doi:10.1161/jaha.116.003887

Cited by 41 publications

(26 citation statements)

References 46 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…[37][38][39] In addition, osteopontin has been found to have predictive value for poor outcomes in heart failure with preserved ejection fraction, 40 which in some aspects may be more suitable for the model of circulatory failure in the Fontan population. 41 The osteoprotegerin is a potent inhibitor of osteoclast differentiation and activity, and the observed increase in the Fontan group may represent a reactive process aiming to protect against bone loss from osteoclast remodelling associated with increased parathormone and borderline low vitamin D, found to be at the upper and lowest normal ranges, respectively, in our study. 42,43 This study has several limitations, one of them being the small sample size that did not allow us to detect differences between Fontan and controls, but this does not change the fact that the osteopenia was more pronounced in the same sample of patients.…”

Section: Discussionsupporting

confidence: 49%

Early characteristics of bone deficits in children with Fontan palliation

et al. 2020

View full text Add to dashboard Cite

Background:This is a cross-sectional study aiming to understand the early characteristics and background of bone health impairment in clinically well children with Fontan circulation.Methods:We enrolled 10 clinically well children with Fontan palliation (operated >5 years before study entrance, Tanner stage ≤3, age 12.1 ± 1.77 years, 7 males) and 11 healthy controls (age 12.0 ± 1.45 years, 9 males) at two children’s hospitals. All patients underwent peripheral quantitative CT. For the Fontan group, we obtained clinical characteristics, NYHA class, cardiac index by MRI, dual x-ray absorptiometry, and biochemical studies. Linear regression was used to compare radius and tibia peripheral quantitative CT measures between Fontan patients and controls.Results:All Fontan patients were clinically well (NYHA class 1 or 2, cardiac index 4.85 ± 1.51 L/min/m2) and without significant comorbidities. Adjusted trabecular bone mineral density, cortical thickness, and bone strength index at the radius were significantly decreased in Fontan patients compared to controls with mean differences −30.13 mg/cm3 (p = 0.041), −0.31 mm (p = 0.043), and −6.65 mg2/mm4 (p = 0.036), respectively. No differences were found for tibial measures. In Fontan patients, the mean height-adjusted lumbar bone mineral density and total body less head z scores were −0.46 ± 1.1 and −0.63 ± 1.1, respectively, which are below the average, but within normal range for age and sex.Conclusions:In a clinically well Fontan cohort, we found significant bone deficits by peripheral quantitative CT in the radius but not the tibia, suggesting non-weight-bearing bones may be more vulnerable to the unique haemodynamics of the Fontan circulation.

show abstract

Section: Discussionsupporting

confidence: 49%

Early characteristics of bone deficits in children with Fontan palliation

et al. 2020

View full text Add to dashboard Cite

show abstract

“…However, our observations support that in a subset of symptomatic adult Fontan patients afterload is elevated, as shown by increased Ea and SVR indices. Similar findings have been reported using non‐invasive haemodynamics . As these patients also had low CI, it would be natural to think that increased afterload would lead to marked reduction in cardiac output in a single ventricle circulation.…”

Section: Discussionmentioning

confidence: 99%

“…Systemic (Qs) and pulmonary (Qp) flows were calculated by Fick principle with estimated oxygen consumption (VO 2 ) calculated using the LaFarge formula; CI corresponds to Qs divided by body surface area. Patients were then divided into four different haemodynamic profiles based on normal or high FP (< 15 or ≥ 15 mmHg, respectively) and low or normal CI (< 2.5 or ≥ 2.5 L/min/m 2 , respectively), according to previously used thresholds . For Qp calculation, the arterial saturation or an assumed pulmonary vein saturation of 95% was used, whichever highest, irrespective of the presence of venovenous collaterals (VVC).…”

Section: Methodsmentioning

confidence: 99%

Haemodynamic profiles in adult Fontan patients: associated haemodynamics and prognosis

Miranda

Borlaug

Hagler

et al. 2019

European J of Heart Fail

View full text Add to dashboard Cite

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.

show abstract

“…Elevated PVRI and low CI were defined as PVRI>2 WU*m 2 and CI<2.5 L/min/m 2 based upon partition values used in previous studies. 11, 15 …”

Section: Methodsmentioning

confidence: 99%

Hemodynamics of Fontan Failure

Egbe

Connolly

Miranda

et al. 2017

Circ: Heart Failure

100

View full text Add to dashboard Cite

Background Non-pulsatile 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 that when coupled with low cardiac index (CI), this would identify patients at increased risk of Fontan failure. Methods and Results 261 adult Fontan patients underwent cardiac catheterization; age 26±3years, men 146(56%), atriopulmonary Fontan 144(55%). Patients were divided into 2 groups: those with high PVRI (>2 WU*m2) and low CI <2.5 L/min/m2 (Group 1, n=70, 30%), and those with normal PVRI and/or normal CI (Group 2, n=182, 70%). Fontan failure was defined by the composite of all-cause mortality, listing for heart transplantation, or initiation of palliative care. There were 68(26%) cases of Fontan failure during a mean follow-up of 8.6±2.4 years. As compared to Group 2, freedom from Fontan failure was significantly lower in Group 1: 66% vs 89% at 5 years. The combination of high PVRI and low CI was an independent risk factor for Fontan failure, HR 1.84 (95% CI 1.09–2.85). Conclusions When coupled with low CI, even mild elevations in PVRI identify patients at high risk of Fontan failure. This suggests that pulmonary vascular disease is a key mechanism underlying Fontan failure and supports further studies to understand the pathophysiology and target treatments to pulmonary vascular tone in this population.

show abstract

Ventricular‐Arterial Function and Coupling in the Adult Fontan Circulation

Cited by 41 publications

References 46 publications

Early characteristics of bone deficits in children with Fontan palliation

Early characteristics of bone deficits in children with Fontan palliation

Haemodynamic profiles in adult Fontan patients: associated haemodynamics and prognosis

Hemodynamics of Fontan Failure

Contact Info

Product

Resources

About