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. 2018 Nov 15:271:312-316.
doi: 10.1016/j.ijcard.2018.05.039.

Venous congestion and pulmonary vascular function in Fontan circulation: Implications for prognosis and treatment

Alexander C Egbe  1 , Yogesh N V Reddy  1 , Arooj R Khan  1 , Mohamad Al-Otaibi  1 , Emmanuel Akintoye  2 , Masaru Obokata  1 , Barry A Borlaug  3
Affiliations

Venous congestion and pulmonary vascular function in Fontan circulation: Implications for prognosis and treatment

Alexander C Egbe et al. Int J Cardiol. .

Abstract

Background: Elevation in central venous pressure (CVP) plays a fundamental pathophysiologic role in Fontan circulation. Because there is no sub-pulmonary ventricle in this system, CVP also provides the driving force for pulmonary blood flow. We hypothesized that this would make Fontan patients more susceptible to even low-level elevation in pulmonary vascular resistance index (PVRI), resulting in greater systemic venous congestion and adverse outcomes.

Methods: Adult Fontan patients and controls without congenital heart disease undergoing clinical evaluation that included cardiac catheterization and echocardiography were examined retrospectively. Outcomes including all-cause mortality and the development of Fontan associated diseases (FAD, defined as protein losing enteropathy, cirrhosis, heart failure hospitalization, arrhythmia, or thromboembolism) were assessed from longitudinal assessment.

Results: As compared to controls (n = 82), Fontan patients (n = 164) were younger (36 vs 45 years, p < 0.001), more likely to be on anticoagulation or antiplatelet therapy, and more likely to have atrial arrhythmia or cirrhosis. There was a strong correlation between CVP and PVRI in the Fontan group (r = 0.79, p < 0.001), but there was no such relationship in controls. Elevated PVRI identified patients at increased risk for FAD (HR 1.92, 95% CI 1.39-2.41, p = 0.01), and composite endpoint of FAD and/or death (HR 1.89, 95% CI 1.32-2.53, p = 0.01) per 1 WU∗m2 increment.

Conclusions: Systemic venous congestion, which is the primary factor in the pathogenesis of FAD and death, is related to even low-level abnormalities in pulmonary vascular function. Multicenter studies are needed to determine whether interventions targeting pulmonary vascular structure and function can improve outcomes in the Fontan population.

Keywords: Central venous pressure; Fontan associated diseases; Fontan failure; Pulmonary vascular resistance.

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Conflict of interest statement

Conflict of Interest

None

Figures

Figure 1
Figure 1
Figure 1A: Linear correlation of central venous pressure (CVP) vs pulmonary vascular resistance index (PVRI) in Fontan patients Figure 1B: Linear correlation of central venous pressure (CVP) vs pulmonary artery wedge pressure (PAWP) in Fontan patients
See this image and copyright information in PMC

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