Volume 11, Issue 6 pp. 589-597
ORIGINAL ARTICLE

Catheter-measured Hemodynamics of Adult Fontan Circulation: Associations with Adverse Event and End-organ Dysfunctions

Makoto Mori MD

Makoto Mori MD

The Section of Cardiac Surgery, Yale University School of Medicine, New Haven, Conn, USA

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Camden Hebson MD

Camden Hebson MD

Division of Cardiology, Emory University School of Medicine, Atlanta, Ga, USA

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Kayoko Shioda MPH, DVM

Kayoko Shioda MPH, DVM

Department of Epidemiology, Emory University Rollins School of Public Health, Atlanta, Ga, USA

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Robert W. Elder MD

Robert W. Elder MD

The Section of Cardiovascular Medicine, Yale University School of Medicine, New Haven, Conn, USA

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Brian E. Kogon MD

Brian E. Kogon MD

Division of Cardiothoracic Surgery, Emory University School of Medicine, Atlanta, Ga, USA

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Fred H. Rodriguez III, MD

Fred H. Rodriguez III, MD

Division of Cardiology, Emory University School of Medicine, Atlanta, Ga, USA

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Maan Jokhadar MD

Maan Jokhadar MD

Division of Cardiology, Emory University School of Medicine, Atlanta, Ga, USA

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Wendy M. Book MD

Corresponding Author

Wendy M. Book MD

Division of Cardiology, Emory University School of Medicine, Atlanta, Ga, USA

Corresponding Author: Wendy M. Book, MD, Division of Cardiology, Department of Medicine, Emory Adult Congenital Heart Center, Emory University School of Medicine, 1364 Clifton Road NE, Atlanta, GA 30322, USA. Tel: (+1) 404-778-5544; Fax: (+1) 404-778-5035; Email: [email protected]Search for more papers by this author
First published: 02 March 2016
Citations: 46

The authors take responsibility for all aspects of the reliability and freedom from bias of the data presented and their discussed interpretation.

Conflict of interest: The authors report no relationships with industry or ones that could be construed as a conflict of interest.

Abstract

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 events (death or heart transplant) were examined using the Cox proportional hazard analysis. Correlations between the hemodynamic measurements and signs of end-organ dysfunction (MELD-XI, Child-Pugh, VAST score, estimated glomerular filtration rate [eGFR]) were analyzed.

Results

Sixty post-Fontan patients (85% systemic left ventricle, 40% atriopulmonary Fontan, mean age of 28 years, and mean time since Fontan operation of 21.9 years) were included. At baseline, those with an event were statistically younger, had lower transcutaneous oxygen saturations, were more likely to have an atriopulmonary Fontan, and were more likely to have a pacemaker. Eighteen experienced a cardiovascularly significant event. Using univariate analysis to compare the event and nonevent groups, mean CI was 2.8 ± 0.9 vs. 2.4 ± 0.5 L/min/m2 (P = .004), and CVP was 18.6 ± 6.5 vs. 16.1 ± 4.3 mmHg (P = .03). However, the statistical significances did not persist in the multivariate model. Higher CVP and pulmonary capillary wedge pressure (PCWP) were associated with higher MELD-XI and Child-Pugh scores, and the VAST score was only associated with PCWP.

Conclusions

Symptomatic adult Fontan patients who experienced an event manifested with a higher CI and CVP, although the multivariate Cox proportional hazard analysis did not yield any significant associations. The presences of hepatic dysfunction and portal venous outflow obstruction were associated with a higher CVP and PCWP. Renal dysfunction was prevalent but no statistically significant association between the hemodynamic measurements was identified, although trends toward a higher CVP and transpulmonary gradient were identified.

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