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Peripheral microangiopathy in Eisenmenger syndrome: A nailfold video capillaroscopy study
Alexandra Arvanitaki1, George Giannakoulas2, Eva Triantafyllidou3
1First Department of Cardiology, AHEPA University Hospital, Medical School, Aristotle University of Thessaloniki, 1 St. Kyriakidi Street, 54636 Thessaloniki, Greece; Fourth Department of Internal Medicine, Hippokration University Hospital, Medical School, Aristotle University of Thessaloniki, 49 Konstantinoupoleos Street, 54642 Thessaloniki, Greece; Department of Cardiology III, Adult Congenital and Valvular Heart Centre, University Hospital Muenster, Albert-Schweitzer-Campus 1, 48149 Muenster, Germany; Adult Congenital Heart Centre, National Centre for Pulmonary Arterial Hypertension, Royal Brompton Hospital, Imperial College, London, UK.
Background:
Eisenmenger syndrome (ES) comprises a severe phenotype of pulmonary arterial hypertension characterized by angiopathy of the lung circulation. The aim of the present study was to demonstrate the presence of systemic microvascular abnormalities in patients with ES using nailfold video-capillaroscopy (NVC) and to identify potential correlations of nailfold capillaroscopic characteristics with non-invasive markers of systemic organ function.
Methods:
Α cross-sectional NVC study was performed in 17 consecutive patients with ES and 17 healthy controls matched for age and sex. NVC quantitative (capillary density, capillary dimensions, haemorrhages, thrombi, shape abnormalities) and qualitative (normal, non-specific or scleroderma pattern) parameters were evaluated.
Results:
Patients with ES [median age 40 (18-65) years, 11 women] presented reduced capillary density [8.8 (7.2-10.2) loops/mm vs. 9.9 (8.3-10.9) loops/mm, p = .004] and increased loop width [15.9 (10.3-21.7) μm vs. 12.3 (7.6-15.2) μm, p < .001], while they had significantly more abnormal capillaries than healthy controls [2.5 (0.9-5.4) abnormal loops/mm vs. 1.0 (0.0-1.7) abnormal loops/mm, p < .001]. NVC shape abnormalities in ES were positively correlated with NT-proBNP (r = 0.52, p = .03) and were negatively associated with estimated glomerular filtration rate (r = -0.60, p = .02). Additionally, capillary loop diameter was positively correlated with increased haemoglobin levels (r = 0.55, p = .03) and negatively correlated with reduced peripheral oxygen saturation (r = - 0.56, p = .02).
Conclusions:
This study supports the hypothesis of peripheral microvascular involvement in ES parallel to pulmonary microangiopathy detected by NVC. Further longitudinal studies are needed to confirm our preliminary results.
