eISSN: 2299-0046
ISSN: 1642-395X
Advances in Dermatology and Allergology/Postępy Dermatologii i Alergologii
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6/2020
vol. 37
 
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abstract:
Original paper

The analysis of echocardiographic results in patients suffering from epidermolysis bullosa

Katarzyna Kurnicka
1
,
Katarzyna Osipowicz
2
,
Olga Dzikowska-Diduch
1
,
Katarzyna Wertheim-Tysarowska
3
,
Cezary Kowalewski
2
,
Piotr Pruszczyk
1

  1. Department of Internal Medicine and Cardiology, Medical University of Warsaw, Warsaw, Poland
  2. Department of Dermatology and Immunodermatology, Medical University of Warsaw, Warsaw, Poland
  3. Department of Medical Genetics, Institute of Mother and Child, Warsaw, Poland
Adv Dermatol Allergol 2020; XXXVII (6): 871–878
DOI: https://doi.org/10.5114/ada.2020.102101
Online publish date: 2021/01/06
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Introduction
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.

Aim
To analyse echocardiographic results obtained in Polish EB patients and compare them between the EB group and healthy persons.

Material and methods
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.

Results
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.

Conclusions
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.

keywords:

epidermolysis bullosa, echocardiography, cardiomyopathy
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