Heart transplantation in the Ehlers-Danlos syndrome

Stephanie M Reinhold1, Brian Lima1, Adnan Khalid1

  • 1Texas A&M Health Science Center College of Medicine (Reinhold); the Division of Cardiology (Khalid, Stoler, Hall) and the Department of Cardiovascular and Thoracic Surgery (Lima, Gonzalez-Stawinski, Chamogeorgakis), Baylor University Medical Center at Dallas and the Baylor Hamilton Heart and Vascular Hospital, Dallas, Texas.

Proceedings (Baylor University. Medical Center)
|October 2, 2015
PubMed

Insights

A woman with Ehlers-Danlos syndrome and aortic aneurysm had a heart attack from coronary artery dissection postpartum. She successfully received a heart transplant, marking a first for Ehlers-Danlos syndrome patients.

Area of Science:

  • Cardiology
  • Cardiovascular Surgery
  • Genetics

Background:

  • Ehlers-Danlos syndrome (EDS) is a group of inherited connective tissue disorders.
  • Aortic aneurysms are a known complication in some types of EDS.
  • Spontaneous coronary artery dissection (SCAD) is a rare cause of myocardial infarction.

Observation:

  • A postpartum patient with EDS and an aortic aneurysm experienced a myocardial infarction.
  • The infarction was caused by spontaneous left circumflex coronary artery dissection.
  • The patient progressed to end-stage heart failure.

Findings:

  • The patient underwent a successful orthotopic heart transplantation.
  • This case represents the first documented heart transplant in an individual with Ehlers-Danlos syndrome.
  • Successful transplantation highlights the feasibility of advanced cardiac procedures in select EDS patients.

Implications:

  • This case expands the understanding of cardiovascular complications in Ehlers-Danlos syndrome.
  • It suggests that heart transplantation is a viable option for end-stage heart failure in EDS patients.
  • Further research is warranted to explore the long-term outcomes and specific considerations for EDS patients undergoing heart transplantation.

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