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(Stroke. 1995;26:1935-1940.)
© 1995 American Heart Association, Inc.
Articles |
Familial Aorto-Cervicocephalic Arterial Dissections and Congenitally Bicuspid Aortic Valve
From the Departments of Neurologic Surgery (W.I.S.) and Neurology (B.M.), Mayo Clinic, Rochester, Minn.
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Abstract |
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 Top Abstract IntroductionDescription of FamiliesDiscussionReferences |
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Background A primary arteriopathy is often implicated in the etiology of spontaneous cervicocephalic arterial dissections, but its exact nature usually remains unknown. We describe the familial occurrence of spontaneous arterial dissections and congenitally bicuspid aortic valve (BAV) and propose a common developmental defect in these families.
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
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Introduction |
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TopAbstractIntroductionDescription of FamiliesDiscussionReferences |
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Dissections of the cervicocephalic arteries are an important cause of ischemic stroke in young and middle-aged people.1 The annual incidence of spontaneous dissection of the extracranial internal carotid artery (ICA), the most frequently involved vessel, is approximately 2.5 to 3.0 per 100 000 population.2 3
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.
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Description of Families |
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TopAbstractIntroductionDescription of FamiliesDiscussionReferences |
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Clinical characteristics of the affected family members are summarized in the Table
.
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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.
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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.
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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.
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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.
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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.
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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.
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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).
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Discussion |
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TopAbstractIntroductionDescription of FamiliesDiscussionReferences |
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Numerous families with arterial dissections have been described,6 8 9 11 12 13 14 15 16 17 18 19 20 21 22 23 24 including 10 with at least one member with involvement of the carotid or vertebral arteries.6 8 9 14 18 23 Of all these families, the presence of BAV was only noted in a father and son with aortic dissection and cystic medial necrosis reported by McKusick.12 Several families with BAV but without arterial dissection have also been described,25 26 27 28 29 and screening for asymptomatic BAV has been recommended for first-degree relatives.29 A familial aggregation of BAV and spontaneous cervicocephalic arterial dissections has not been previously appreciated.
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.
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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.
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Footnotes |
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Reprint requests to Dr Wouter I. Schievink, Department of Neurologic Surgery, Mayo Clinic, 200 First St SW, Rochester, MN 55905.
Received April 24, 1995; revision received July 17, 1995; accepted July 17, 1995.
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