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Rare disease
CASE REPORT
Fulminant myocardial bleeding: another clinical course of vascular Ehlers-Danlos Syndrome
  1. Masahide Tokue1,
  2. Hidehiko Hara1,
  3. Kenji Kurosawa2,
  4. Masato Nakamura1
  1. 1Department of Cariovascular Medicine, Toho University Ohashi Medical Center, Tokyo, Japan
  2. 2Department of Medical Genetics, Kanagawa Children’s Medical Center, Yokohama, Japan
  1. Correspondence to Dr Masahide Tokue, masahide.tokue{at}med.toho-u.ac.jp

Summary

Vascular Ehlers-Danlos Syndrome (vEDS) is a dominantly inherited connective tissue disorder characterised by colon rupture and arterial aneurysm, dissection and rupture. A patient was diagnosed with vEDS after a spontaneous colon rupture when he was brought to our institute because of sudden chest pain. An ECG revealed wide regional ST elevation, which was initially suggestive of acute myocarditis. On the second day, haemodynamics suddenly deteriorated because of a rapid accumulation of bloody pericardial effusion, and the patient died. Autopsy revealed an excessive spontaneous myocardial haemorrhage owing to fragility, which suggested an underlying disease—vEDS.

  • adult intensive care
  • intensive care
  • cardiovascular medicine
  • genetics

This is an Open Access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/

https://doi.org/10.1136/bcr-2017-220786

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    Footnotes

    • Contributors MT is the first author and the corresponding author too. HH was responsible for reviewing article and performed emergent coronary angiogram. KK was involved in the genetic screening. MN was responsible for the final approval of the version to be submitted.

    • Competing interests None declared.

    • Patient consent Obtained from guardian.

    • Provenance and peer review Not commissioned; externally peer reviewed.