Abstract
Ehlers-Danlos Syndrome Type IV is an illness that often leads to premature death due to arterial rupture or dissection and is characterized by very fragile connective tissue. This report documents the death of a 30-year-old man with Ehlers-Danlos Syndrome Type IV from myocardial rupture and cardiac tamponade following a myocardial infarction. We believe that Ehlers-Danlos Syndrome Type IV contributed to the coronary atherosclerosis and myocardial rupture in this young man and that this disease led indirectly to his death by myocardial infarction, an unusual cause of death in this syndrome.
Issue Section:
Research Papers
References
1.
Beighton
P
, De Paepe
A
, Steinmann
B
, Tsipouras
P
, Wenstrup
RJ
. Ehlers-Danlos syndromes: revised nosology, Villefranche, 1997. Ehlers-Danlos National Foundation (USA) and Ehlers-Danlos Support Group (UK)
. Am J Med Genet
0148-7299 1998
;77
:31
–37
.2.
Ross
MH
, Romrell
LJ
, Kaye
GI
. Histology: a text and atlas
. 3rd ed. Philadelphia
: Lippincott, Williams and Wilkins
, 1995
.3.
Robins
SP
, Farquharson
C
. Connective tissue components of the blood vessel wall in health and disease
. In: Stehbens
WE
, Lie
JT
, editors. Vascular pathology
. London
: Chapman and Hall
, 1995
;89
–128
.4.
Liu
X
, Wu
H
, Byrne
M
, Krane
S
, Jaenisch
R
. Type III collagen is crucial for collagen I fibrillogenesis and for normal cardiovascular development
. Proc Natl Acad Sci USA
0027-8424 1997
;94
:1852
–6
.5.
Jarmulowicz
M
, Phillips
WG
. Vascular Ehlers-Danlos syndrome undiagnosed during life
. J R Soc Med
0035-9157 2001
;94
:28
–30
.6.
Pepin
M
, Schwarze
U
, Superti-Furga
A
, Byers
P
. Clinical and genetic features of Ehlers-Danlos Syndrome type IV, the vascular type
. N Engl J Med
0028-4793 2000
;342
:673
–80
.7.
Prockop
DJ
, Kuivaniemi
H
, Tromp
G
, Ala-Kokko
L
. Inherited disorders of connective tissue
. In: Braunwald
E
, Fauci
A
, Kasper
D
, Hauser
S
, Longo
D
, & Jameson
J
, editors. Harrison's principles of internal medicine
. 15th ed. New York
: McGraw-Hill
, 2001
, 2290
–300
.8.
Cotran
RS
, Kumar
V
, Collins
T
. Robbins pathologic basis of disease
. 6th ed. Philadelphia
: WB Saunders Company
, 1999
.9.
Athanassiou
AM
, Turrentine
MA
. Myocardial infarction and coronary artery dissection during pregnancy associated with type IV Ehlers-Danlos syndrome
. Am J Perinatology
0735-1631 1996
;13
:181
–3
.10.
Choudhury
L
, Marsh
J
. Myocardial infarction in young patients
. Am J Med
0002-9343 1999
;107
:254
–61
.11.
Gordon
MB
, Libby
P
. Atherosclerosis
. In: Lilly
LS
, editor. Pathophysiology of heart disease: a collaborative project of medical students and faculty
. 3rd ed. Philadelphia
: Lippincott Williams and Wilkins
, 2003
;111
–30
.12.
Caulfield
JB
, Janicki
JS
. Structure and function of myocardial fibrillar collagen
. Technology and Health Care
0928-7329 1997
;5
:95
–113
.13.
Bloom
S
, Peric-Golia
L
. Geographic variation in the incidence of myocardial calcification associated with acute myocardial infarction
. Human Pathology
0046-8177 1989
;20
:726
–31
.14.
Lapatto-Reiniluoto
O
, Vaalamo
M
, Takkunen
O
, Manttari
M
. Left ventricular calcification following resuscitation
. J Intern Med
0954-6820 2000
;248
:85
–87
.
This content is only available via PDF.
Copyright © 2005 by ASTM
You do not currently have access to this content.