Endothelial glycocalyx in adult patients with congenital heart disease, with or without heart failure

Background

Endothelial glycocalyx covers the endothelial surface and it is correlated with endothelial properties and function. Thus, is well established in assessing endothelial function. It has not been studied in patients (pts) with adult congenital heart disease (ACHD).

Purpose

We aimed to investigate endothelial glycocalyx in ACHD pts thus, the correlation of endothelial dysfunction with heart failure syndrome (HF) in this population.

Methods

In 56 pts, (mean age: 38±12 years) with ACHD (23 with tetralogy of Fallot, of whom 15 with HF as defined by NYHA>I or NTproBNP>125pg/ml, or HF medication; 17 pts with systemic right ventricle, of whom 13 with HF and 16 pts with congenital left outflow obstructive disease, 6 of whom with HF) and 39 controls with similar age, sex and smoking habits, we assessed perfused boundary region (PBR) of

the sublingual microvessels with a diameter 5-25μm using Sidestream Dark Field camera (Microscan). Increased PBR indicates impaired glycocalyx integrity.

Results

Compared with controls, all ACHD pts had a higher PBR 5-25 (2.1±0.3 versus 1.97±0.17μm, p=0.014) (figure). ACHD pts WITH HF had a higher mean age than ACHD pts WITHOUT HF (42.7 ±12.1 versus 34±10.9 years, p=0.011). ACHD pts with HF had a trend for a higher PBR 5-25 values compared to ACHDpts patients without HF, though this did not reach statistical significance (2.09±0.30 versus 2.07±0,32μm, p=0,789). When compared to the control group, ACHD pts with HF had significantly higher

PBR 5-25 (2.09±0.3 versus 1.97±0.17μm, p=0.034). ACHD pts without HF had a trend for higher PBRvalues compared to controls, albeit without reaching statistical significance (2.07±0.32 versus 1.97±0.17μm, p=0.206).

Pts with ACHD with HF vs pts with ACHD without HF had:

1. Worse systematic ventricular GLS (-13.7±4.27 versus -17.9±3.67, p=0.001)

2.Worse systematic atrium conduit strain and reservoir strain S_CD (-13.8±7 versus -18.5±5.2, p=0.17

and S_R 22.3±10.9 versus 30.9±7.5, p=0.002 respectively)

Conclusions

Endothelial glycocalyx is impaired in all ACHD pts and particularly in ACHD heart failure patients. Endothelial dysfunction appears to be a continuous process triggered by fixed and dynamic parameters in relation to the congenital heart disease. Further research is needed to determine if endothelial glycocalyx could be an index for treatment initiation and/or a therapeutic target in this group of young patients.For image description, please refer to the figure legend and surrounding text.