The analysis of echocardiographic results in patients suffering from epidermolysis bullosa.
cardiomyopathy
echocardiography
epidermolysis bullosa
Journal
Postepy dermatologii i alergologii
ISSN: 1642-395X
Titre abrégé: Postepy Dermatol Alergol
Pays: Poland
ID NLM: 101168357
Informations de publication
Date de publication:
Dec 2020
Dec 2020
Historique:
received:
28
05
2020
accepted:
12
08
2020
entrez:
19
2
2021
pubmed:
20
2
2021
medline:
20
2
2021
Statut:
ppublish
Résumé
Cardiac abnormalities revealed in patients suffering from epidermolysis bullosa (EB) include dilated cardiomyopathy (DC) and aortopathy. DC is a rare but serious complication associated with an increased mortality, predominantly observed in recessive dystrophic EB. Echocardiography is the most available diagnostic tool used to detect heart disease in EB patients. To analyse echocardiographic results obtained in Polish EB patients and compare them between the EB group and healthy persons. We analysed retrospectively echocardiograms of 23 patients with EB (14 F, mean age 17.3 years) performed from 2017 to 2019. The incidence of left ventricular (LV) systolic and diastolic dysfunction, right heart disease and congenital heart disease was evaluated. A comparison of echo-parameters between EB patients and 20 matched healthy subjects was performed. We did not find any cases of DC and aortopathy in the EB group. One bicuspid aortic valve case was revealed. Analysis of LV diastolic parameters showed that the mean value of mitral A velocity was significantly higher and the pulmonary venous flow D velocity was lower in the EB group than in controls. Tissue Doppler analysis revealed lower values of E' velocities of mitral annulus in the EB group, what may suggest discreetly slower LV relaxation, however, this will definitely require further research. Although most EB patients do not present cardiac symptoms, there is still a risk of developing cardiomyopathy associated with poor prognosis. It seems reasonable to perform a scheduled echocardiographic screening including LV systolic and diastolic function assessment to detect preclinical cardiac abnormalities.
Identifiants
pubmed: 33603603
doi: 10.5114/ada.2020.102101
pii: 42822
pmc: PMC7874876
doi:
Types de publication
Journal Article
Langues
eng
Pagination
871-878Informations de copyright
Copyright: © 2021 Termedia Sp. z o. o.
Déclaration de conflit d'intérêts
The authors declare no conflict of interest.
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