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Fulminant myocardial bleeding: another clinical course of vascular Ehlers-Danlos Syndrome

Masahide Tokue1, Hidehiko Hara1, Kenji Kurosawa2

  • 1Department of Cariovascular Medicine, Toho University Ohashi Medical Center, Tokyo, Japan.

BMJ Case Reports
|September 25, 2017
PubMed

Insights

Vascular Ehlers-Danlos Syndrome (vEDS) can cause life-threatening arterial and colon issues. This case highlights vEDS as a cause of spontaneous myocardial hemorrhage and sudden death, even after initial misdiagnosis.

Area of Science:

  • Cardiology
  • Genetics
  • Pathology

Background:

  • Vascular Ehlers-Danlos Syndrome (vEDS) is a rare, autosomal dominant disorder.
  • It is characterized by fragile blood vessels and connective tissues, leading to arterial dissections, aneurysms, and ruptures.
  • Gastrointestinal complications, such as colon rupture, are also common.

Observation:

  • A patient presented with sudden chest pain following a spontaneous colon rupture.
  • Initial ECG findings suggested acute myocarditis.
  • Hemodynamic deterioration occurred due to rapid pericardial effusion accumulation, leading to death.

Findings:

  • Autopsy revealed extensive spontaneous myocardial hemorrhage.
  • The hemorrhage was attributed to the inherent tissue fragility characteristic of vEDS.
  • This finding confirmed vEDS as the underlying cause of the fatal event.

Implications:

  • This case underscores the importance of considering vEDS in patients with unexplained arterial or gastrointestinal events.
  • Early diagnosis of vEDS is crucial for risk stratification and management.
  • Increased awareness among clinicians can improve patient outcomes and potentially prevent fatal complications.

Keywords:
adult intensive carecardiovascular medicinegeneticsintensive care

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