Limited amount of evidence suggests the presence of systemic vasculopathy in pulmonary arterial hypertension (PAH), whereas there hardly any evidence regarding patients with Eisenmenger syndrome. Nailfold video-capillaroscopy (NVC) is an established, non-invasive imaging technique for the assessment of the microcirculation. The aim of this study is to investigate the presence of capillaroscopic abnormalities in patients with the Eisenmenger syndrome.
We present preliminary data regarding capillaroscopic abnormalities in patients with Eisenmenger syndrome treated in a PH expert center in Greece. All patients and healthy controls underwent NVC, performed on all fingers of both hands, excluding thumbs, using Optilia Digital Capillaroscope. Capillary quantitative and qualitative parameters were measured.
NVC was performed in 15 patients with Eisenmenger syndrome, [mean age 41.5±14.4 years, 66.7% female, 40% with a ventricular septal defect) and 28 healthy controls [mean age 42.5±13.5 years, 71.4% female]. Significant capillaroscopic abnormalities were detected in patients compared to healthy controls. Capillary density was significantly decreased in patients with Eisenmenger syndrome vs. healthy controls (8.8±0.9 loops/mm vs. 9.7±0.8 loops/mm, p=0.002). Patients with Eisenmenger syndrome presented also increased mean capillary width (38.3±6.4μm vs. 29.5±4.4 μm, p<0.001), increased loop diameter (16.7±2.8μm vs 11.5±2.2μm, p<0.001) and increased mean number of irregularly enlarged capillaries >50μm/mm [0.5 (IQR 1.3) vs. 0.2 (IQR 0.6), p=0.01]. Furthermore, capillary shape abnormalities, microhemorrhages and signs of thrombosis were more frequent in Eisenmenger patients compared to healthy controls [2.7±1.0 abnormal capillaries/mm vs. 1.2±0.8 abnormal capillaries/mm, p<0.001, 0.12 (IQR: 0.37) microhemorrhages/mm vs. 0.0 (IQR:0.0) microhemorrhages/mm, p<0.001, 2.6±1.4 thrombosed capillaries/mm vs. 1.5±0.7 thrombosed capillaries/mm, p=0.014].
These data support for the first time the hypothesis of systemic microvascular involvement in Eisenmenger syndrome in addition to the well-known pulmonary vasculopathy.