Circulatory Response to Rapid Volume Expansion and Cardiorespiratory Fitness in Fontan Circulation

Thomas Möller, Vibeke Klungerbo, Simone Diab, Henrik Holmstrøm, Elisabeth Edvardsen, Guro Grindheim, Henrik Brun, Erik Thaulow, Alvaro Köhn-Luque, Assami Rösner, Gaute Døhlen, Thomas Möller, Vibeke Klungerbo, Simone Diab, Henrik Holmstrøm, Elisabeth Edvardsen, Guro Grindheim, Henrik Brun, Erik Thaulow, Alvaro Köhn-Luque, Assami Rösner, Gaute Døhlen

Abstract

The role of dysfunction of the single ventricle in Fontan failure is incompletely understood. We aimed to evaluate hemodynamic responses to preload increase in Fontan circulation, to determine whether circulatory limitations in different locations identified by experimental preload increase are associated with cardiorespiratory fitness (CRF), and to assess the impact of left versus right ventricular morphology. In 38 consecutive patients (median age = 16.6 years, 16 females), heart catheterization was supplemented with a rapid 5-mL/kg body weight volume expansion. Central venous pressure (CVP), ventricular end-diastolic pressure (VEDP), and peak systolic pressure were averaged for 15‒30 s, 45‒120 s, and 4‒6 min (steady state), respectively. CRF was assessed by peak oxygen consumption (VO2peak) and ventilatory threshold (VT). Median CVP increased from 13 mmHg at baseline to 14.5 mmHg (p < 0.001) at steady state. CVP increased by more than 20% in eight patients. Median VEDP increased from 10 mmHg at baseline to 11.5 mmHg (p < 0.001). Ten patients had elevated VEDP at steady state, and in 21, VEDP increased more than 20%. The transpulmonary pressure difference (CVP‒VEDP) and CVP were consistently higher in patients with right ventricular morphology across repeated measurements. CVP at any stage was associated with VO2peak and VT. VEDP after volume expansion was associated with VT. Preload challenge demonstrates the limitations beyond baseline measurements. Elevation of both CVP and VEDP are associated with impaired CRF. Transpulmonary flow limitation was more pronounced in right ventricular morphology. Ventricular dysfunction may contribute to functional impairment after Fontan operation in young adulthood.ClinicalTrials.gov identifier NCT02378857.

Keywords: Fontan circulation; Hemodynamics; Preload challenge; Transpulmonary gradient; Univentricular congenital heart defects; Ventricular function.

Conflict of interest statement

None.

© 2021. The Author(s).

Figures

Fig. 1
Fig. 1
Flowchart of patient enrollment in the present study. CPET cardiopulmonary exercise test, HC heart catheterization, HT heart transplantation
Fig. 2
Fig. 2
Heart rate response to rapid volume expansion (RVE) in non-pacemaker patients (N = 31)
Fig. 3
Fig. 3
Central venous pressure before and after rapid volume expansion (RVE)
Fig. 4
Fig. 4
Ventricular end-diastolic pressure before and after rapid volume expansion (RVE). Central illustration: Rapid volume expansion by intravenous saline bolus unmasks limitations of the Fontan circulation by pressure rise upstream from the blood flow restriction(s)

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Source: PubMed

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