Contemporary management of vascular Ehlers-Danlos syndrome

Ying Wei Lum, Benjamin S. Brooke, James Hamilton Black

Research output: Contribution to journalArticle

Abstract

Purpose of review: Vascular Ehlers-Danlos syndrome (EDS) results from mutations in the formation of type III collagen. This leads to various potentially lethal complications including rupture of the arterial vessels, intestinal organs, and the uterus. This review summarizes recent cohort studies that have improved our medical and surgical management of complications associated with vascular EDS. Recent findings: Vascular EDS is associated with a shortened overall survival due to potential complications, namely loss of connective tissue integrity in blood vessels and increased risk of arterial rupture. The traditional approach has been to treat such complications conservatively unless they are life threatening. There have been challenges to this treatment paradigm based on recent reports. Treatment with the beta blocker Celiprolol was shown in a randomized study to be associated with a three-fold decrease in arterial rupture in vascular EDS patients. Furthermore, it was shown by observational studies that elective surgical repair of blood vessels at risk of rupture may be safely undertaken at tertiary referral centers that have expertise in managing connective tissue disorders. Novel approaches using endovascular therapy with coil embolization have also been attempted with good results in the treatment of ruptured pseudoaneurysms, visceral aneurysms, and carotid-cavernous fistulas. Summary: New evidence-based treatments have greatly expanded the medical and surgical management options for patients with EDS. These patients are best managed by multidisciplinary teams of interventionalists, cardiologists, and geneticists in tertiary centers with expertise in managing connective tissue disorders.

Original languageEnglish (US)
Pages (from-to)494-501
Number of pages8
JournalCurrent Opinion in Cardiology
Volume26
Issue number6
DOIs
StatePublished - Nov 2011

Fingerprint

Ehlers-Danlos Syndrome
Blood Vessels
Rupture
Connective Tissue
Celiprolol
Therapeutics
Collagen Type III
False Aneurysm
Tertiary Care Centers
Uterus
Fistula
Observational Studies
Aneurysm
Cohort Studies
Mutation
Survival

Keywords

  • arterial aneurysm
  • arterial dissection
  • connective tissue disorders
  • endovascular
  • management of complications
  • vascular Ehlers-Danlos syndrome

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Contemporary management of vascular Ehlers-Danlos syndrome. / Lum, Ying Wei; Brooke, Benjamin S.; Black, James Hamilton.

In: Current Opinion in Cardiology, Vol. 26, No. 6, 11.2011, p. 494-501.

Research output: Contribution to journalArticle

@article{6335451f16674c36b7f2ac8c3973e2b5,
title = "Contemporary management of vascular Ehlers-Danlos syndrome",
abstract = "Purpose of review: Vascular Ehlers-Danlos syndrome (EDS) results from mutations in the formation of type III collagen. This leads to various potentially lethal complications including rupture of the arterial vessels, intestinal organs, and the uterus. This review summarizes recent cohort studies that have improved our medical and surgical management of complications associated with vascular EDS. Recent findings: Vascular EDS is associated with a shortened overall survival due to potential complications, namely loss of connective tissue integrity in blood vessels and increased risk of arterial rupture. The traditional approach has been to treat such complications conservatively unless they are life threatening. There have been challenges to this treatment paradigm based on recent reports. Treatment with the beta blocker Celiprolol was shown in a randomized study to be associated with a three-fold decrease in arterial rupture in vascular EDS patients. Furthermore, it was shown by observational studies that elective surgical repair of blood vessels at risk of rupture may be safely undertaken at tertiary referral centers that have expertise in managing connective tissue disorders. Novel approaches using endovascular therapy with coil embolization have also been attempted with good results in the treatment of ruptured pseudoaneurysms, visceral aneurysms, and carotid-cavernous fistulas. Summary: New evidence-based treatments have greatly expanded the medical and surgical management options for patients with EDS. These patients are best managed by multidisciplinary teams of interventionalists, cardiologists, and geneticists in tertiary centers with expertise in managing connective tissue disorders.",
keywords = "arterial aneurysm, arterial dissection, connective tissue disorders, endovascular, management of complications, vascular Ehlers-Danlos syndrome",
author = "Lum, {Ying Wei} and Brooke, {Benjamin S.} and Black, {James Hamilton}",
year = "2011",
month = "11",
doi = "10.1097/HCO.0b013e32834ad55a",
language = "English (US)",
volume = "26",
pages = "494--501",
journal = "Current Opinion in Cardiology",
issn = "0268-4705",
publisher = "Lippincott Williams and Wilkins",
number = "6",

}

TY - JOUR

T1 - Contemporary management of vascular Ehlers-Danlos syndrome

AU - Lum, Ying Wei

AU - Brooke, Benjamin S.

AU - Black, James Hamilton

PY - 2011/11

Y1 - 2011/11

N2 - Purpose of review: Vascular Ehlers-Danlos syndrome (EDS) results from mutations in the formation of type III collagen. This leads to various potentially lethal complications including rupture of the arterial vessels, intestinal organs, and the uterus. This review summarizes recent cohort studies that have improved our medical and surgical management of complications associated with vascular EDS. Recent findings: Vascular EDS is associated with a shortened overall survival due to potential complications, namely loss of connective tissue integrity in blood vessels and increased risk of arterial rupture. The traditional approach has been to treat such complications conservatively unless they are life threatening. There have been challenges to this treatment paradigm based on recent reports. Treatment with the beta blocker Celiprolol was shown in a randomized study to be associated with a three-fold decrease in arterial rupture in vascular EDS patients. Furthermore, it was shown by observational studies that elective surgical repair of blood vessels at risk of rupture may be safely undertaken at tertiary referral centers that have expertise in managing connective tissue disorders. Novel approaches using endovascular therapy with coil embolization have also been attempted with good results in the treatment of ruptured pseudoaneurysms, visceral aneurysms, and carotid-cavernous fistulas. Summary: New evidence-based treatments have greatly expanded the medical and surgical management options for patients with EDS. These patients are best managed by multidisciplinary teams of interventionalists, cardiologists, and geneticists in tertiary centers with expertise in managing connective tissue disorders.

AB - Purpose of review: Vascular Ehlers-Danlos syndrome (EDS) results from mutations in the formation of type III collagen. This leads to various potentially lethal complications including rupture of the arterial vessels, intestinal organs, and the uterus. This review summarizes recent cohort studies that have improved our medical and surgical management of complications associated with vascular EDS. Recent findings: Vascular EDS is associated with a shortened overall survival due to potential complications, namely loss of connective tissue integrity in blood vessels and increased risk of arterial rupture. The traditional approach has been to treat such complications conservatively unless they are life threatening. There have been challenges to this treatment paradigm based on recent reports. Treatment with the beta blocker Celiprolol was shown in a randomized study to be associated with a three-fold decrease in arterial rupture in vascular EDS patients. Furthermore, it was shown by observational studies that elective surgical repair of blood vessels at risk of rupture may be safely undertaken at tertiary referral centers that have expertise in managing connective tissue disorders. Novel approaches using endovascular therapy with coil embolization have also been attempted with good results in the treatment of ruptured pseudoaneurysms, visceral aneurysms, and carotid-cavernous fistulas. Summary: New evidence-based treatments have greatly expanded the medical and surgical management options for patients with EDS. These patients are best managed by multidisciplinary teams of interventionalists, cardiologists, and geneticists in tertiary centers with expertise in managing connective tissue disorders.

KW - arterial aneurysm

KW - arterial dissection

KW - connective tissue disorders

KW - endovascular

KW - management of complications

KW - vascular Ehlers-Danlos syndrome

UR - http://www.scopus.com/inward/record.url?scp=80054687808&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=80054687808&partnerID=8YFLogxK

U2 - 10.1097/HCO.0b013e32834ad55a

DO - 10.1097/HCO.0b013e32834ad55a

M3 - Article

C2 - 21852761

AN - SCOPUS:80054687808

VL - 26

SP - 494

EP - 501

JO - Current Opinion in Cardiology

JF - Current Opinion in Cardiology

SN - 0268-4705

IS - 6

ER -