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(Stroke. 1995;26:1935-1940.)
© 1995 American Heart Association, Inc.
Articles |
From the Departments of Neurologic Surgery (W.I.S.) and Neurology (B.M.), Mayo Clinic, Rochester, Minn.
| Abstract |
|---|
|
|
|---|
Summary of Report In the first family, a 63-year-old man suffered an extracranial internal carotid artery (ICA) dissection, and his 43-year-old cousin with BAV suffered an intracranial vertebral artery (VA) dissection. Two other family members had pathologically proven BAV. In the second family, a 31-year-old woman suffered bilateral extracranial ICA and VA dissections. Her father, at age 46, suffered an aortic dissection associated with cystic medial necrosis and BAV. Her paternal uncle died from an aortic dissection at age 59. In the third family, a 39-year-old woman suffered extracranial ICA and VA dissections, and her brother died at age 48 from an aortic dissection associated with a BAV.
Conclusions The familial occurrence of spontaneous arterial dissections and BAV suggests a common developmental defect. The aortic valvular cusps and the arterial media of the aortic arch and its branches are derived from neural crest cells, suggesting that a neural crest defect may be the underlying abnormality in these families.
Key Words: aorta carotid arteries cerebrovascular disorders dissection vertebral arteries
| Introduction |
|---|
|
|
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The etiology and pathogenesis of cervicocephalic arterial dissections are poorly understood, but a primary arteriopathy is often implicated, particularly when the arterial dissection is spontaneous and the patient is young. Nevertheless, the nature of the putative underlying arteriopathy remains unknown in the great majority of patients.4 5 Several studies have implicated a genetically determined arteriopathy in patients with spontaneous cervical artery dissections.6 7 8 9 Among approximately 240 patients with spontaneous cervical artery dissections evaluated at the Mayo Clinic between 1970 and 1992, eight had a family history of arterial dissection. Four of these families have been described previously.6 9
In the present report we describe the familial occurrence of spontaneous arterial dissections and congenitally bicuspid aortic valve (BAV) in three families and propose a common developmental defect in these families. None of these families have been reported previously.
| Description of Families |
|---|
|
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|
Family A (Fig 1
)
Subject III8
A 63-year-old man, the propositus, suffered transient
dysphasia, right hemiparesis, and left monocular blindness. He had
undergone bilateral inguinal hernia repairs at age 23 and had been
known to be hypertensive since age 41. Physical examination was normal.
A CT scan of the head was normal. Echocardiography
showed aortic valve sclerosis with mild aortic
regurgitation, but the exact number of valves could not
be determined. Angiography demonstrated a dissection of the left
extracranial ICA associated with an intimal flap extending into the
petrous portion of the ICA (Fig 2
). Multiple luminal
irregularities were noted in the distal left common carotid artery.
|
|
At age 77, the patient developed a bifrontal headache followed by gait unsteadiness, vertigo, and hiccups. Neurological examination was significant for truncal ataxia only. A head CT was normal. An MR image of the head showed an area of increased T2 signal in the right posterior medulla compatible with infarction. Angiography showed a chronic dissection of the left ICA, but the posterior circulation was normal.
Subject V1
A 43-year-old man, a distant cousin of the propositus,
developed an occipital headache associated with abruptly turning his
head, followed by gait unsteadiness. His medical history was
significant for aortic insufficiency since age 20 years, which was
associated with aortic root dilatation and a BAV, as demonstrated on
echocardiography. His blood pressure was 140/80
mm Hg, and cardiac auscultation revealed a systolic ejection murmur
and a decrescendo diastolic murmur. Neurological
examination showed right peripheral seventh and tenth
cranial nerve pareses, right Horner's syndrome, and ataxia. A cranial
CT scan and examination of cerebrospinal fluid were normal. Angiography
revealed a dissection of the intracranial right vertebral artery (VA)
just distal to the posterior inferior cerebellar artery,
manifested by a high-grade smooth stenosis. The patient
recovered slowly from his dysphagia, necessitating a temporary
gastrostomy. No new vascular events have developed during 2 years of
follow-up.
Subjects IV3 and III7
Subjects IV3 and III7 suffered
ischemic strokes of uncertain etiology at the age of 59 years
and 60 years, respectively. Subject IV3 had severe aortic
valve stenosis with moderate insufficiency and was found to
have a BAV and aortic root dilatation at surgery. Preoperative
echocardiography had failed to demonstrate a BAV.
Subject III7 was found to have a BAV as an incidental
finding at postmortem examination.
Family B (Fig 3
)
Subject III1
A 31-year-old woman, the proposita, developed a
right-sided headache followed 2 weeks later by vertigo, nausea,
left hemifacial numbness, and pulsatile tinnitus. Her medical history
was significant for a right inguinal hernia repair at age 3, and she
had been hypertensive since age 19. General medical examination was
normal. Neurological examination showed absent hearing on the right,
bilateral peripheral seventh cranial nerve pareses, a right
sixth cranial nerve paresis, bilateral hypesthesia in a V2
distribution, left-sided dysmetria, and ataxia. Angiography
demonstrated bilateral extracranial VA dissections, manifested by
occlusion on the right at the C2 level and a high-grade
stenosis on the left extending from the C2 level to the origin
of the basilar artery. The patient recovered from her strokes with only
mild residual ataxia.
|
Seven years later the patient developed diplopia and right periorbital
pain. Neurological examination showed a right third cranial nerve
paresis and mild gait ataxia. MRI showed bilateral subacute
hematomas in the wall of the extracranial ICAs (Fig 4
)
and old bilateral posterior fossa infarcts. Angiography showed
extensive bilateral extracranial ICA dissections. The appearance of the
left VA had normalized, and the right VA showed a moderate degree of
residual irregular stenosis. Her third cranial nerve paresis
resolved, and no new vascular episodes have developed during 2 years of
follow-up.
|
Analysis of the type III procollagen gene in cultured skin fibroblasts was normal.5
Subject II2
A 46-year-old man, the father of the proposita, experienced an
acute onset of chest pain. His medical history was significant for
bilateral inguinal hernia repair at age 19 years and hypertension since
age 34 years. Cardiac auscultation revealed systolic and
diastolic cardiac murmurs consistent with aortic
stenosis and insufficiency. Aortography demonstrated aortic
root dilatation and an aortic dissection. At surgery, a tear was
identified opposite the left subclavian artery, and the dissection was
noted to extend into both common iliac arteries. He underwent a bypass
graft to both groins but developed right leg ischemia
postoperatively, and a femoral saphenous vein bypass graft was
successfully performed.
At age 58 years the patient underwent replacement of his dilated
ascending aorta and aortic valve replacement for aortic
stenosis and insufficiency related to a BAV. Moderately severe
cystic medial necrosis of the resected aortic segment was noted (Fig 5
). One year later he underwent coronary artery
bypass surgery for angina pectoris. He has remained
asymptomatic during an additional 7 years of
follow-up.
|
Analysis of the type III procollagen gene in cultured skin fibroblasts was normal.10
Subject II5
A 59-year-old man, a paternal uncle of the proposita,
developed severe chest pain. He had been diagnosed with labile
hypertension at age 50 years. On admission to the hospital he was
hemodynamically stable. A chest film was normal.
Several hours later he again developed chest pain. Shortly thereafter
he experienced cardiorespiratory arrest. A chest film now showed a
markedly widened mediastinum, diagnostic of aortic
dissection. Resuscitations were unsuccessful, and the patient died. An
autopsy was not performed.
Family C (Fig 6
)
Subject II5
A 39-year-old woman, the proposita, developed a left-sided
headache and several episodes of transient right hemiparesis with
dysphasia. Neurological examination showed left miosis and ptosis. A
head CT was normal. Angiography demonstrated a subcranial dissecting
aneurysm of the left ICA associated with irregular
stenosis of its proximal extracranial portion.
|
No further spells occurred until 9 years later, when she developed two
episodes of transient right hemiparesis and dysphasia. Her blood
pressure was 120/82 mm Hg. Physical examination was normal.
Echocardiography showed mild mitral valve prolapse
only. Angiography revealed a soft thrombus arising from the known left
internal carotid dissecting aneurysm, which had not changed in
size, and near complete resolution of the area of stenosis.
Luminal irregularities were now noted in the right extracranial ICA.
The VAs were normal. She was treated with anticoagulation. On repeat
angiography 3 months later, the thrombus had resolved, and the
subcranial ICA dissecting aneurysm had not changed in size (Fig 7
). However, a new dissecting aneurysm of the
left extracranial VA had now developed at the level of C4. No vascular
events were noted during 9 years of further follow-up.
|
Analysis of the type III procollagen gene in cultured skin fibroblasts was normal.5
Subject II1
A 48-year-old man, a brother of the proposita, developed
severe chest pain and transient right hemicorporal numbness. On
examination his blood pressure was 160/100 mm Hg, the right femoral
pulse was absent, and the right leg was cold. Neurological examination
was normal. Two days later he had a seizure followed by
cardiorespiratory arrest. Postmortem examination revealed an intimal
tear of the ascending aorta with hemopericardium and a BAV. The
dissection started immediately above the aortic ring and extended into
the right innominate, left common carotid, left subclavian, and both
common iliac arteries. Microscopic examination confirmed a medial
dissection but showed no underlying abnormalities of the
arterial wall (Fig 8
).
|
| Discussion |
|---|
|
|
|---|
In the three presently described families, affected members had either spontaneous arterial dissection, BAV, or both. In addition, cystic medial necrosis was found in one of the two patients whose tissue was available for microscopic examination. Although echocardiography showed no evidence for BAV in two of our patients with cervical artery dissections, the sensitivity of echocardiography in identifying BAV is only approximately 78%,30 and the presence of a BAV cannot be excluded with certainty. Autosomal dominant inheritance with variable penetrance and expression appears to be the most likely mode of genetic transmission in two of our families. In the third family only two siblings were known to be affected, and the mode of inheritance is uncertain. There was no evidence in our families for such heritable connective tissue disorders as Marfan's syndrome or Ehlers-Danlos syndrome, which predispose to arterial dissections31 and which also have been associated with BAV.32 33 Also, there was no evidence for a mutation in the type III procollagen gene (the underlying defect in Ehlers-Danlos syndrome type IV) in the two families that were tested for such a mutation.5 10
Congenitally BAV is one of the most common forms of congenital heart disease in adults and is found in approximately 1% of the population.34 Some patients with BAV remain asymptomatic throughout life, but most eventually develop aortic insufficiency or aortic stenosis.34 Ischemic stroke due to thrombotic or calcific embolization from a BAV has occasionally been reported.35 36
An association of BAV with aortic coarctation, aortic dissection,
aortic root dilatation, and cystic medial necrosis has been well
established.37 38 39 40 41 42 43 44 45 46 Cervicocephalic arterial
dissections have also been associated with aortic
coarctation9 47 and cystic medial
necrosis.48 49 50 Several studies have shown that BAV is a
frequent finding in patients with aortic dissection, particularly in
young adults.40 41 42 Conversely, among all patients with
BAV, aortic dissection is the cause of death in approximately
5%,39 and aortic root dilatation has been reported in
more than 50% of affected individuals.42 43 45 46 BAV is
found in approximately half of patients with coarctation of the aorta,
and the association of aortic coarctation with aortic dissection may be
mainly a reflection of the presence of BAV.37 38 42 44
Cystic medial necrosis of the aorta is commonly identified in patients
with BAV as well as in those with aortic coarctation, and this is not
related to the presence of arterial
hypertension.40 41 In addition, aortic root dilatation in
patients with BAV has been shown to be unrelated to any
hemodynamic changes caused by the abnormal aortic
valve,43 45 and a common developmental defect is generally
implicated in BAV and its associated aortic abnormalities. The possible
interrelationship of BAV and its associated arterial
abnormalities is presented in Fig 9
.
|
The left ventricular outflow tract, including the aortic valvular cusps, and the media of the aortic arch and its branches are all derived from cells originating in the neural crest.51 52 53 The neural crest is a transient structure in early embryogenesis,51 and a disorder of neural crest cell migration could possibly explain the findings of arterial dissection, BAV, and cystic medial necrosis in our families. The neural crest has been implicated previously in the development of cervicocephalic arterial dissections.9 54
In conclusion, we describe three families with BAV, cystic medial necrosis, and (or) spontaneous arterial dissections involving the cervicocephalic vessels or the aorta. A common genetically determined developmental defect, possibly involving the neural crest, may be implicated in the familial occurrence of cervicocephalic arterial dissections and BAV.
| Footnotes |
|---|
Received April 24, 1995; revision received July 17, 1995; accepted July 17, 1995.
| References |
|---|
|
|
|---|
2.
Schievink WI, Mokri B, Whisnant JP. Internal
carotid artery dissection in a community: Rochester, Minnesota,
1987-1992. Stroke. 1993;24:1678-1680.
3.
Giroud M, Fayolle H, Andre N, Dumas R, Becker F,
Martin D, Baudoin N, Krause D. Incidence of internal carotid
artery dissection in the community of Dijon. J
Neurol Neurosurg Psychiatry. 1994;57:1443.
4.
Schievink WI, Mokri B, O'Fallon WM. Recurrent
spontaneous cervical-artery dissection. N
Engl J Med. 1994;330:393-397.
5.
Kuivaniemi H, Prockop DJ, Wu Y, Madhatheri SL,
Kleinert C, Early JJ, Jokinen A, Stolle C, Majamaa K, Myllylä VV,
Norrgård Ö, Schievink WI, Mokri B, Fukawa O, ter Berg HWM, de
Paepe A, Lozano AM, Leblanc R, Ryynänen M, Baxter BT, Shikata H,
Ferrell RE, Tromp G. Exclusion of mutations in the gene for type
III collagen (COL3A1) as a common cause of intracranial
aneurysms or cervical artery dissections by sequence
analysis of the coding sequences of type III collagen from 55
unrelated patients. Neurology. 1993;43:2652-2658.
6.
Mokri B, Piepgras DG, Wiebers DO, Houser OW.
Familial occurrence of spontaneous dissection of the internal
carotid artery. Stroke. 1987;18:246-251.
7.
Schievink WI, Mokri B, Michels VV, Piepgras DG.
Familial association of intracranial aneurysms and
cervical artery dissections. Stroke. 1991;22:1426-1430.
8. Majamaa K, Portimojarvi H, Sotaniemi KA, Myllylä VV. Familial aggregation of cervical artery dissection and cerebral aneurysm. Stroke. 1994;25:1704-1705. [Medline] [Order article via Infotrieve]
9.
Schievink WI, Michels VV, Mokri B, Piepgras DG, Perry
HO. A familial syndrome of arterial dissections with
lentiginosis. N Engl J Med. 1995;332:576-579.
10. Tromp G, Wu Y, Prockop DJ, Madhatheri SL, Kleinert C, Early JJ, Zhuang J, Norrgård Ö, Darling RC, Abbott WM, Cole CW, Jaakola P, Ryynänen M, Pearce WH, Yao JST, Majamaa K, Smullens SN, Gatalica C, Ferrell RE, Jimenez SA, Jackson CE, Michels VV, Kaye M, Kuivaniemi H. Sequencing of cDNA from 50 unrelated patients reveals that mutations in the triple-helical domain of type III procollagen are an infrequent cause of aortic aneurysms. J Clin Invest. 1993;91:2539-2545.
11.
Hanley WB, Jones NB. Familial dissecting aortic
aneurysm: a report of three cases within two
generations. Br Heart J. 1967;29:852-858.
12. McKusick VA. Association of congenital bicuspid aortic valve and Erdheim's cystic medial necrosis. Lancet. 1972;1:1026-1027. [Medline] [Order article via Infotrieve]
13. Bixler D, Antley RM. Familial aortic dissection with iris anomalies: a new connective tissue disease syndrome? Birth Defects. 1976;12:229-234.
14. Petit H, Bouchez B, Destee A, Clarisse J. Familial form of fibromuscular dysplasia of the internal carotid artery. J Neuroradiol. 1983;10:15-22. [Medline] [Order article via Infotrieve]
15. Warnes CA, Kirkman PM, Roberts WC. Aortic dissection in more than one family member. Am J Cardiol. 1985;55:236-238. [Medline] [Order article via Infotrieve]
16. McManus BM, Cassling RS, Soundy TJ, Wilson JE, Sears TD, Rogler WC, Buehler BA, Wolford JF, Duggan MJ, Byers PH. Familial aortic dissection in absence of ascending aortic aneurysm: a lethal syndrome associated with precocious systemic hypertension. Am J Cardiovasc Pathol. 1986;1:55-67.
17.
Marwick TH, Woodhouse SP, Birchley IN, Strong RW.
Management of familial aortic dissection.
Chest. 1987;92:954-956.
18. Varani E, Magnani G, Magelli C, Ferretti RM, Agosta R, Craincevich T, Magnani B. Aneurisma dissecante dell'aorta: descrizione di casi familiari. Medicina. 1988;8:293-297.
19. Nicod P, Bloor C, Godfrey M, Hollister D, Pyeritz RE, Dittrich H, Polikar R, Peterson KL. Familial aortic dissecting aneurysm. J Am Coll Cardiol. 1989;13:811-819. [Abstract]
20.
Toyama M, Amano A, Kameda T. Familial aortic
dissection: a report of rare family cluster. Br Heart
J. 1989;61:204-207.
21. Disertori M, Bertagnolli C, Thiene G, Ferro A, Bonmassari R, Girardini D, Casarotto D. Aneurisma dissecante dell'aorta a carattere familiare. G Ital Cardiol. 1991;21:849-853. [Medline] [Order article via Infotrieve]
22. Teien D, Finley JP, Murphy DA, Lacson A, Longhi J, Gillis DA. Idiopathic dilatation of the aorta with dissection in a family without Marfan syndrome. Acta Paediatr Scand. 1991;80:1246-1249. [Medline] [Order article via Infotrieve]
23. Somlo S, Rutecki G, Giuffra LA, Reeders ST, Cugino A, Whittier FC. A kindred exhibiting cosegregation of an overlap connective tissue disorder and the chromosome 16 linked form of autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 1993;4:1371-1378. [Abstract]
24. Francke U, Berg MA, Tynan K, Brenn T, Liu W, Aoyama T, Gasner C, Miller DC, Furthmayr H. A Gly1127Ser mutation in an EGF-like domain of the fibrillin-1 gene is a risk factor for ascending aortic aneurysm and dissection. Am J Hum Genet. 1995;56:1287-1296. [Medline] [Order article via Infotrieve]
25.
Emanuel R, Withers R, O'Brien K, Rossi P, Feizi O.
Congenitally bicuspid aortic valves: clinicogenetic study of 41
families. Br Heart J. 1978;40:1402-1407.
26.
Gale AN, McKusick VA, Hutchins GH, Gott VL.
Familial congenital bicuspid aortic valve.
Chest. 1977;72:668-670.
27.
Godden DJ, Sandhu PS, Kerr F. Stenosed bicuspid
aortic valves in twins. Eur Heart J. 1987;8:316-318.
28.
McDonald K, Maurer BJ. Familial aortic valve
disease: evidence for a genetic influence? Eur Heart
J. 1989;10:676-677.
29. Glick BN, Roberts WC. Congenitally bicuspid aortic valve in multiple family members. Am J Cardiol. 1994;73:400-404. [Medline] [Order article via Infotrieve]
30. Brandenburg RO Jr, Tajik AJ, Edwards WD, Reeder GS, Shub C, Seward JB. Accuracy of two-dimensional echocardiographic diagnosis of congenitally bicuspid aortic valve: echocardiographic-anatomic correlation in 115 patients. Am J Cardiol. 1983;51:1469-1473. [Medline] [Order article via Infotrieve]
31. Schievink WI, Michels VV, Piepgras DG. Neurovascular manifestations of heritable connective tissue disorders: a review. Stroke. 1994;25:889-903. [Abstract]
32. Roberts WC, Honig HS. The spectrum of cardiovascular disease in the Marfan syndrome: a clinicomorphologic study of 18 necropsy patients and comparison to 151 previously reported necropsy patients. Am Heart J. 1982;104:115-135. [Medline] [Order article via Infotrieve]
33. Leier CV, Call TD, Fulkerson PK, Wooley CF. The spectrum of cardiac defects in the Ehlers-Danlos syndrome, type I and III. Ann Intern Med. 1980;92:171-178.
34. Roberts WC. Cardiovascular abnormalities usually silent until adulthood. In: Roberts WC, ed. Adult Congenital Heart Disease. Philadelphia, Pa: FA Davis Co; 1987:631-691.
35.
Pleet AB, Massey EW, Vengrow ME. TIA, stroke,
and the bicuspid aortic valve. Neurology. 1981;31:1540-1542.
36.
O'Donoghue ME, Dangond F, Burger AJ, Suojanen JN,
Zarich S, Tarsy D. Spontaneous calcific embolization to the
supraclinoid internal carotid artery from a regurgitant bicuspid aortic
valve. Neurology. 1993;43:2715-2717.
37.
Becker AE, Becker MJ, Edwards JE. Anomalies
associated with coarctation of the aorta: particular reference to
infancy. Circulation. 1970;41:1067-1075.
38.
Scovil JA, Nanda NC, Gross CM, Lombardi AC, Gramiak R,
Lipchick EO, Manning JA. Echocardiographic
studies of abnormalities associated with coarctation of the
aorta. Circulation. 1976;53:953-956.
39. Fenoglio JJ Jr, McAllister HA Jr, DeCastro CM, Davia JE, Cheitlin MD. Congenital bicuspid aortic valve after age 20. Am J Cardiol. 1977;39:164-169. [Medline] [Order article via Infotrieve]
40.
Edwards WD, Leaf DS, Edwards JE. Dissecting
aortic aneurysm associated with congenital bicuspid aortic
valve. Circulation. 1978;57:1022-1025.
41. Larson EW, Edwards WD. Risk factors for aortic dissection: a necropsy study of 160 cases. Am J Cardiol. 1984;53:849-855. [Medline] [Order article via Infotrieve]
42. Lindsay J Jr. Coarctation of the aorta, bicuspid aortic valve and abnormal ascending aortic wall. Am J Cardiol. 1988;61:182-184. [Medline] [Order article via Infotrieve]
43. Pachulski RT, Weinberg AL, Chan K-L. Aortic aneurysm in patients with functionally normal or minimally stenotic bicuspid aortic valve. Am J Cardiol. 1991;67:781-782. [Medline] [Order article via Infotrieve]
44. Roberts CS, Roberts WC. Dissection of the aorta associated with congenital malformation of the aorta. J Am Coll Cardiol. 1991;17:712-716. [Abstract]
45. Hahn RT, Roman MJ, Mogtader AH, Devereux RB. Association of aortic dilatation with regurgitant, stenotic, and functionally normal bicuspid aortic valves. J Am Coll Cardiol. 1992;19:283-288. [Abstract]
46.
Sadee AS, Becker AE, Verheul HA, Bouma B, Hoedemaker G.
Aortic valve regurgitation and the congenitally
bicuspid aortic valve: a clinico-pathological correlation.
Br Heart J. 1992;67:439-441.
47.
Schievink WI, Mokri B, Piepgras DG. Spontaneous
dissections of cervicocephalic arteries in childhood and
adolescence. Neurology. 1994;44:1607-1612.
48. Brice JG, Crompton MR. Spontaneous dissecting aneurysms of the cervical internal carotid artery. Br Med J. 1964;2:790-792.
49.
Boström K, Liliequist B. Primary
dissecting aneurysm of the extracranial part of the internal
carotid and vertebral arteries: a report of three cases.
Neurology. 1967;17:179-186.
50. Chang CM, Ng HK, Leung SY, Fong KY, Yu YL. Fatal bilateral vertebral artery dissection in a patient with cystic medial necrosis. Clin Neurol Neurosurg. 1991;93:309-311. [Medline] [Order article via Infotrieve]
51. Le Douarin NM. The Neural Crest. Cambridge, Mass: Cambridge University Press; 1981.
52. Rosenquist TH, Beall AC, Modis L, Fishman R. Impaired elastic matrix development in the great arteries after ablation of the cardiac neural crest. Anat Rec. 1990;226:347-359. [Medline] [Order article via Infotrieve]
53. Kappetein AP, Gittenbergerde Groot AC, Zwinderman AH, Rohmer J, Poelmann RE, Huysmans HA. The neural crest as a possible pathogenetic factor in coarctation of the aorta and bicuspid aortic valve. J Thorac Cardiovasc Surg. 1991;102:830-836. [Abstract]
54. Schievink WI, Piepgras DG, Nichols DA. Spontaneous carotid-jugular fistula and carotid dissection in a patient with multiple intracranial arachnoid cysts and hemifacial atrophy: a generalized connective tissue disorder? Case report. J Neurosurg. 1995;83:546-549.[Medline] [Order article via Infotrieve]
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V. T. Nkomo, M. Enriquez-Sarano, N. M. Ammash, L. J. Melton III, K. R. Bailey, V. Desjardins, R. A. Horn, and A. J. Tajik Bicuspid Aortic Valve Associated With Aortic Dilatation: A Community-Based Study Arterioscler Thromb Vasc Biol, February 1, 2003; 23(2): 351 - 356. [Abstract] [Full Text] [PDF] |
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J. P. Veinot Non-uniform aortic medial degenerative changes associated with aortic valve disease Eur. J. Cardiothorac. Surg., December 1, 2002; 22(6): 1036 - 1037. [Full Text] [PDF] |
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M. Bauer, M. Pasic, R. Meyer, N. Goetze, U. Bauer, H. Siniawski, and R. Hetzer Morphometric analysis of aortic media in patients with bicuspid and tricuspid aortic valve Ann. Thorac. Surg., July 1, 2002; 74(1): 58 - 62. [Abstract] [Full Text] [PDF] |
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W. I. Schievink Spontaneous Dissection of the Carotid and Vertebral Arteries N. Engl. J. Med., March 22, 2001; 344(12): 898 - 906. [Full Text] [PDF] |
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F. J. Kirkham, M. Prengler, D. K.M. Hewes, and V. Ganesan Risk Factors for Arterial Ischemic Stroke in Children J Child Neurol, May 1, 2000; 15(5): 299 - 307. [Abstract] [PDF] |
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M. de Sa, Y. Moshkovitz, J. Butany, and T. E. David HISTOLOGIC ABNORMALITIES OF THE ASCENDING AORTA AND PULMONARY TRUNK IN PATIENTS WITH BICUSPID AORTIC VALVE DISEASE: CLINICAL RELEVANCE TO THE ROSS PROCEDURE J. Thorac. Cardiovasc. Surg., October 1, 1999; 118(4): 588 - 596. [Abstract] [Full Text] [PDF] |
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S Nistri, M D Sorbo, M Marin, M Palisi, R Scognamiglio, and G Thiene Aortic root dilatation in young men with normally functioning bicuspid aortic valves Heart, July 1, 1999; 82(1): 19 - 22. [Abstract] [Full Text] [PDF] |
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D. Bonderman, E. Gharehbaghi-Schnell, G. Wollenek, G. Maurer, H. Baumgartner, and I. M. Lang Mechanisms Underlying Aortic Dilatation in Congenital Aortic Valve Malformation Circulation, April 27, 1999; 99(16): 2138 - 2143. [Abstract] [Full Text] [PDF] |
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W. I. Schievink, B. Mokri, D. G. Piepgras, and J. D. Kuiper Recurrent Spontaneous Arterial Dissections : Risk in Familial Versus Nonfamilial Disease Stroke, April 1, 1996; 27(4): 622 - 624. [Abstract] [Full Text] |
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