Early and Late Outcomes of Cardiovascular Surgery in Patients With Ehlers-Danlos Syndrome

Background Cardiovascular surgical outcomes reports are few for vascular type IV of Ehlers- Danlos Syndrome (vEDS) compared to non-vascular types I-III (nEDS). Methods To define cardiovascular surgical outcomes among adult patients (≥18 years) with EDS types, a review of our institution's in-house STS Adult Cardiac Surgery Database-compliant software and electronic medical records from Mayo Clinic (1993–2019) was performed. Outcomes were compared for vEDS patients and nEDS patients. Demographics, baseline characteristics, operative, in-hospital complications and follow-up vital status were analyzed. Results Over the study time frame, 48 EDS patients underwent surgery (mean age 52.6 ± 14.6 years; 48% females). Of these, 17 patients had vEDS and 31 patients had nEDS. Six patients (12.5%) underwent prior sternotomy. Urgent or emergent surgery was performed in 10 patients (20.8%). Aortic (vEDS 76.5% vs. nEDS 16.1%) and mitral procedures (vEDS 11.8% vs. nEDS 48.4%) were the two most common cardiovascular surgeries performed (p < .01 and p = .007, respectively). Cardiopulmonary bypass time (CPB) (165 ± 18 vs. 90 ± 13 min; p = .015) and aortic cross clamp times (140 ± 14 vs. 62 ± 10 min; p < .001) were longer for vEDS patients. There was 1 (2.1%) early and 7 (14.6%) late deaths; 6 among vEDS and 2 among nEDS patients. Survival at 5 (80% vs. 93%), 10 (45% vs. 84%) and 15 years (45% vs. 84%) was lower in patients with vEDS (p = .015 for each comparison). Conclusion Cardiovascular surgeries are significantly more complex with longer bypass and cross clamp times for type IV vEDS compared to nEDS patients. Reduced overall survival underscores the complexity and fragility of vEDS patients.

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Clinical and Genetic Features of Ehlers–Danlos Syndrome Type IV, the Vascular Type

New England Journal of Medicine ◽

10.1056/nejm200003093421001 ◽

2000 ◽

Vol 342 (10) ◽

pp. 673-680 ◽

Cited By ~ 791

Author(s):

Melanie Pepin ◽

Ulrike Schwarze ◽

Andrea Superti-Furga ◽

Peter H. Byers

Keyword(s):

Syndrome Type ◽

Ehlers Danlos Syndrome ◽

Type Iv ◽

Vascular Type ◽

Genetic Features ◽

Danlos Syndrome

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Clinical and Genetic Features of Ehlers–Danlos Syndrome Type IV, the Vascular Type

New England Journal of Medicine ◽

10.1056/nejm200102013440522 ◽

2001 ◽

Vol 344 (5) ◽

pp. 392-392 ◽

Cited By ~ 2

Keyword(s):

Syndrome Type ◽

Ehlers Danlos Syndrome ◽

Type Iv ◽

Vascular Type ◽

Genetic Features ◽

Danlos Syndrome

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Acute brain ischemia as a complication of the Ehlers–Danlos syndrome, the case series

Vascular ◽

10.1177/1708538113505519 ◽

2013 ◽

Vol 22 (5) ◽

pp. 341-345 ◽

Cited By ~ 1

Author(s):

Michal Pajak ◽

Marcin A Majos ◽

Wojciech Szubert ◽

Ludomir Stefanczyk ◽

Agata Majos

Keyword(s):

Brain Ischemia ◽

Case Series ◽

Vascular Complications ◽

Limb Ischemia ◽

Radiology Department ◽

Artery Dissection ◽

Ehlers Danlos Syndrome ◽

Type Iv ◽

Vascular Type ◽

Danlos Syndrome

Vascular type of Ehlers–Danlos syndrome involves many severe complications leading not only to organ-specific symptoms but often ends in a sudden death. The aim of this paper was to present a diagnostic possibilities and its efficiency rate in patients with vascular complications of Ehlers–Danlos syndrome who suffered from artery dissection resulting in acute brain or limb ischemia. We analysed three patients with diagnosed Ehlers–Danlos syndrome who were referred to radiology department for diagnostic imaging of affected vascular beds, each experienced brain ischemia. The paper also aims at offering some general recommendations for patients suffering from possible complications of type IV Ehlers–Danlos syndrome basing on our own experience and available literature data.

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Ehlers-Danlos Syndrome Type IV, Vascular Type, Which Demonstrated a Novel Point Mutation in the COL3A1 Gene

Internal Medicine ◽

10.2169/internalmedicine.49.3435 ◽

2010 ◽

Vol 49 (16) ◽

pp. 1797-1800 ◽

Cited By ~ 6

Author(s):

Rinako Sadakata ◽

Atsushi Hatamochi ◽

Keiji Kodama ◽

Akiko Kaga ◽

Takefumi Yamaguchi ◽

...

Keyword(s):

Point Mutation ◽

Syndrome Type ◽

Ehlers Danlos Syndrome ◽

Type Iv ◽

Vascular Type ◽

Col3a1 Gene ◽

Danlos Syndrome

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Clinical and Genetic Features of Ehlers-Danlos Syndrome Type IV, the Vascular Type

Obstetrical & Gynecological Survey ◽

10.1097/00006254-200008000-00004 ◽

2000 ◽

Vol 55 (8) ◽

pp. 469-471 ◽

Cited By ~ 2

Author(s):

Melanie Pepin ◽

Ulrike Schwarze ◽

Andrea Superti-Furga ◽

Peter H. Byers

Keyword(s):

Syndrome Type ◽

Ehlers Danlos Syndrome ◽

Type Iv ◽

Vascular Type ◽

Genetic Features ◽

Danlos Syndrome

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A Critical Analysis of the Perioperative Management of Patients with Ehlers Danlos Type IV (Vascular) Syndrome

Journal of Perioperative Practice ◽

10.1177/175045891702700904 ◽

2017 ◽

Vol 27 (9) ◽

pp. 196-199

Author(s):

J Martin

Keyword(s):

Perioperative Management ◽

Emergency Situation ◽

Joint Hypermobility ◽

Connective Tissue Disorders ◽

Care Needs ◽

Ehlers Danlos Syndrome ◽

Type Iv ◽

Vascular Type ◽

Theatre Staff ◽

Danlos Syndrome

This paper discusses the perioperative care needs of patients with Ehlers Danlos type IV (vascular) syndrome. Ehlers Danlos syndrome (EDS) is a heritable group of connective tissue disorders characterised by varying degrees of tissue, blood vessel and internal organ fragility as well as skin and joint hypermobility (De Paepe & Malfait 2012). In 1997 EDS was revised and classified into six subtypes: classical (Types I and II), hypermobility, vascular (Type IV), kyphoscoliosis and arthrochalasia type. Each classification has been based on the following aspects: diagnostic uniformity, natural history, management, genetics, and the identification of potential areas for research (Beighton et al 1998). Vascular type EDS has serious implications for any form of surgical procedure and it is therefore imperative that theatre staff are fully conversant with the needs of this patient group. Overall awareness needs to be increased, particularly as most patients will most frequently be seen in an emergency situation, as elective procedures are avoided as far as possible.

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Pediatric Innominate Artery Pseudoaneurysm Rupture in Vascular Ehlers-Danlos Syndrome: A Case Report

Clinical Practice and Cases in Emergency Medicine ◽

10.5811/cpcem.2021.3.51787 ◽

2021 ◽

Vol 2 (5) ◽

pp. 226-229

Author(s):

Aimee Vos ◽

Katharine Burns

Keyword(s):

Case Report ◽

Innominate Artery ◽

Final Diagnosis ◽

Connective Tissue Disorder ◽

High Morbidity ◽

Ehlers Danlos Syndrome ◽

Artery Pseudoaneurysm ◽

Type Iv ◽

Vascular Type ◽

Danlos Syndrome

Introduction: Ehlers-Danlos syndrome is a well classified connective tissue disorder recognized by its features of hyperextensibility of joints and hyperelasticity of the skin. However, the rare vascular type (Ehlers-Danlos type IV) is more difficult to identify in the absence, rarity, or subtlety of the classical physical features. Patients presenting to the emergency department (ED) with acute complications of vascular Ehlers-Danlos syndrome may be critically ill, requiring accurate diagnosis and tailored management. Case Report: This report details a case of spontaneous innominate artery pseudoaneurysm rupture in a pediatric patient with previously undiagnosed Ehlers-Danlos syndrome. Initial ED evaluation was followed by urgent operative intervention and subsequent genetic testing to confirm final diagnosis. Conclusion: Due to its high morbidity and mortality, vascular type Ehlers-Danlos syndrome should be considered in the differential for otherwise unexplained spontaneous vascular injury.

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THE BILATERAL ULNAR ARTERY INVOLVEMENT IN TYPE IV EHLERS-DANLOS SYNDROME — A CASE REPORT

Hand Surgery ◽

10.1142/s0218810412720185 ◽

2012 ◽

Vol 17 (02) ◽

pp. 213-216 ◽

Cited By ~ 5

Author(s):

Kazuo Ikeda ◽

Naoki Osamura ◽

Satomi Kasashima

Keyword(s):

Case Report ◽

Ulnar Artery ◽

Ehlers Danlos Syndrome ◽

Type Iv ◽

First Case ◽

Vascular Type ◽

The World ◽

Forearm Compartment Syndrome ◽

Danlos Syndrome ◽

Hereditary Condition

Ehlers-Danlos syndrome Type IV, the vascular type, is a rare hereditary condition. Major arteries are often affected in this type. In this paper, we present the first case report in the world in which forearm compartment syndrome caused by the bilateral ulnar artery rupture in this type. Successful treatment such as incision of the forearm fascia and ligation of the ulnar artery was performed.

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Arterial Switch Operation in a Patient With Ehlers-Danlos Syndrome Type IV

World Journal for Pediatric and Congenital Heart Surgery ◽

10.1177/2150135118769418 ◽

2018 ◽

Vol 11 (4) ◽

pp. NP182-NP185

Author(s):

Raghav Murthy ◽

Nicole L. Herrick ◽

Howaida El-Said ◽

Paul Grossfeld ◽

John Moore ◽

...

Keyword(s):

Arterial Switch Operation ◽

Ductus Arteriosus ◽

Rare Condition ◽

Ehlers Danlos Syndrome ◽

Arterial Switch ◽

Type Iv ◽

Vascular Type ◽

Switch Operation ◽

Long Term Follow Up ◽

Danlos Syndrome

Ehlers-Danlos syndrome vascular type IV is characterized by translucent skin, easy bruising, and fragility of arteries. A full-term female infant presented at four weeks of age with a diagnosis of d-transposition of the great arteries with restrictive atrial septal defect. She successfully underwent emergent balloon atrial septostomy and placement of patent ductus arteriosus (PDA) stent. She required restenting of the PDA and pulmonary artery banding prior to arterial switch procedure. At 16 months of age, the patient successfully underwent arterial switch procedure without complication. This report demonstrates the feasibility of an arterial switch operation along with long-term follow-up of this rare condition.

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