Neurological Symptoms in Type A Aortic Dissections
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Neurological Symptoms in Type A Aortic Dissections
Charly Gaul, MD; Wenke Dietrich, MD; Ivar Friedrich, MD;
Joachim Sirch, MD; Frank J. Erbguth, MD
Background and Purpose—Aortic dissection typically presents with severe chest or back pain. Neurological symptoms
may occur because of occlusion of supplying vessels or general hypotension. Especially in pain-free dissections diagnosis can
be difficult and delayed. The purpose of this study is to analyze the association between type A aortic dissection and
neurological symptoms.
Methods—Clinical records of 102 consecutive patients with aortic dissection (63% male, median age 58 years) over 7.5
years were analyzed for medical history, preoperative clinical characteristics, treatment and outcome with main emphasis on
neurological symptoms.
Results—Thirty patients showed initial neurological symptoms (29%). Only two-thirds of them reported chest pain, and
most patients without initial neurological symptoms experienced pain (94%). Neurological symptoms were attributable
to ischemic stroke (16%), spinal cord ischemia (1%), ischemic neuropathy (11%), and hypoxic encephalopathy (2%).
Other frequent symptoms were syncopes (6%) and seizures (3%). In half of the patients, neurological symptoms were
transient. Postoperatively, neurological symptoms were found in 48% of all patients encompassing ischemic stroke
(14%), spinal cord ischemia (4%), ischemic neuropathy (3%), hypoxic encephalopathy (8%), nerve compression (7%),
and postoperative delirium (15%). Overall mortality was 23% and did not significantly differ between patients with and
without initial neurological symptoms or complications.
Conclusion—Aortic dissections might be missed in patients with neurological symptoms but without pain. Neurological
findings in elderly hypertensive patients with asymmetrical pulses or cardiac murmur suggest dissection. Especially in
patients considered for thrombolytic therapy in acute stroke further diagnostics is essential. Neurological symptoms are
not necessarily associated with increased mortality. (Stroke. 2007;38:292-297.)
Key Words: aortic dissection 䡲 neurological complications 䡲 neurological symptoms
T he incidence of aortic dissection ranges from 5 to 30 cases
per million people per year.1 Aortic dissection can produce
a wide range of symptoms by affecting the outflow of supra-
ary 1, 1998 and June 30, 2005. Both the Department of Cardiotho-
racic Surgery Halle and Nuremberg are responsible for a region of
⬇1 000 000 inhabitants. Patients were identified by screening hos-
pital discharge diagnosis records and surgical databases. Clinical
aortal, abdominal, spinal, extremity, and renal vessels. Typical files of patients with thoracic or thoraco-abdominal aortic dissection
symptoms are chest or back pain and hypotension leading to the fulfilling the criteria of DeBakey type I or II and Stanford type A
differential diagnosis of myocardial infarction. Neurological were reviewed by neurologists (C.G. and W.D.) for neurological
symptoms occur because of occlusion of carotid, vertebral, or symptoms. The Stanford classification consists of 2 types: type A,
spinal arteries, and vasa nervorum of peripheral nerves, or involving the ascending aorta regardless of the entry side location;
because of hypotension and related cerebral perfusion deficit. and type B, involving only the aorta distal to the origin of the left
subclavian artery. The DeBakey classification consists of 3 types:
Most studies that have evaluated neurological symptoms in type I, with involvement of both the ascending and descending aorta;
patients with acute aortic dissection were restricted to a small type II, with involvement of only the ascending aorta; and type III,
number of patients at a single center. They showed a distinct only the descending aorta.1 Neurological symptoms were classified
increased mortality in the presence of neurological symptoms.2,3 into different groups according to Blanco et al (1999).3 Neurological
The aim of the present study was to assess the link between type symptoms of all patients admitted primarily to the emergency room
A aortic dissection and neurological symptoms with regard to or the intensive care unit of both hospitals were diagnosed after
detailed clinical assessment by neurologists and further neuroimag-
perioperative and mid-term outcome. ing if necessary. In patients directly transferred from regional
hospitals for further surgical management, classification was made
Materials and Methods by documented clinical signs and symptoms and neuroimaging
All patients with acute aortic dissection treated in 2 departments of findings. The diagnosis of aortic dissection was confirmed with CT,
cardiothoracic surgery were retrospectively enrolled between Janu- MRI, echocardiography, aortography, intraoperative visualization,
Received July 17, 2006; final revision received August 10, 2006; accepted August 18, 2006.
From Department of Neurology (C.G.), Martin-Luther-University Halle-Wittenberg, Germany; Department of Neurology (W.D., F.J.E.), Nuremberg
Municipal Hospital, Germany; Department of Cardiothoracic Surgery (I.F.), Martin-Luther-University Halle-Wittenberg, Germany; Department of
Cardiothoracic Surgery (J.S.), Nuremberg Municipal Hospital, Germany.
Correspondence to Charly Gaul, MD, Department of Neurology, Martin-Luther-University Halle-Wittenberg, Ernst-Grube-Straße 40, 06097
Halle/Saale, Germany. E-mail Charly.Gaul@gmx.de
© 2007 American Heart Association, Inc.
Stroke is available at http://www.strokeaha.org DOI: 10.1161/01.STR.0000254594.33408.b1
292
Downloaded from http://stroke.ahajournals.org/ by guest on May 20, 2015Gaul et al Neurological Symptoms in Type A Aortic Dissections 293
and postmortem examination according to the recommendations of patients. Among the patients with neurological symptoms at
the Task Force on Aortic Dissection of the European Society of the onset of dissection, only two-thirds gave a history of pain,
Cardiology.4 Analysis included general medical history and preop- whereas most patients without neurological symptoms
erative clinical characteristics with main emphasis on neurological
symptoms. Treatment and outcome were analyzed, focusing on (94.4%) experienced initial pain (P⫽0.001***). Out of 102
perioperative neurological complications. Outcome of patients was patients, 30 (29.4%) showed neurological symptoms as the
determined at discharge from the acute hospital and after 6 months. initial manifestation of aortic dissection (mean age, 57.2
The latter determination was made by contacting the rehabilitation years; male/female-ratio 1.3:1). DeBakey type II aortic dis-
centers or the general practitioner. Data analysis was performed section was less frequently observed among patients with
using statistical analysis software SPSS 12.0. Using univariate
neurological symptoms (23.3% versus 45.8% with/without
analysis, frequency and distribution of demographic or comorbidity
variables and presenting symptoms were compared in patients with neurological symptoms; P⫽0.045*). The observed neurolog-
and without signs of neurological dysfunction. 2 cross-tabulations ical symptoms were classified as: (1) ischemic stroke (per-
were applied as appropriate with a significance level of 0.05. sistent or transient, eg, stroke, or transient ischemic attack);
(2) spinal cord ischemia; (3) ischemic neuropathy; and (4)
Results hypoxic encephalopathy (Figure 1). Ischemic neuropathy
Demographics, Predisposing Conditions, resulted from occlusion of the aortic bifurcation (N⫽4), iliac
and Etiology of femoral arteries (N⫽6), and subclavian artery (N⫽1).
Typical features were signs of acute upper or lower limb
Demographics ischemia as localized severe pain and paresthesia, pulse
Data of 102 patients (median age, 58 years; range, 18 to 78 deficit, and, in severe cases, motor deficit. In contrast, acute
years) were enrolled. Men were affected more frequently than paraplegia and complete sensory loss below level Th12 in 1
women (1.7:1). The peak incidence in men was in the sixth patient with no signs of lower limb ischemia was most likely
and seventh decades, whereas women were affected mostly in attributable to spinal cord ischemia caused by aortic dissec-
the eighth decade. tion distending from aortic valve to aortic bifurcation, occlud-
Type of Dissection, Predisposing Conditions, and Etiology ing spinal arteries. Transient global amnesia (TGA) was
All patients had Stanford type A dissection; DeBakey type I identified in 2 patients. Both patients showed no pure TGA,
was identified in 60.8% of patients. The most common but showed TGA-plus-syndrome with additional focal neu-
predisposing conditions were hypertension (in 65.3% of rological signs such as mild left-side motor deficit, slight
patients), obesity (35.6%), and cigarette smoking (31.7%). anisocoria, and mild facial paresis; therefore, they were
Marfan syndrome was present in 17.7% of patients (mean classified as having ischemic stroke. Eight of 30 patients
age, 36.7 years; range, 18 to 68 years). Histopathologic (26.7%) presented a combination of different neurological
symptoms: hemiparesis and tonic-clonic seizure or syncope
diagnostics revealed arteriosclerosis in 36.3% of the patients
(3 times), transient ischemic attack and syncope (twice),
as the most frequent finding; in 27.5% no changes could be
ischemic neuropathy and seizure (once), and TGA-plus-
detected.
syndrome (twice). In half of all patients, neurological symp-
toms were only transient.
Initial Symptomatology
Presenting Symptoms and Signs Special Neurological Diagnostics and Findings
The majority of patients (86.1%) presented with initial chest CT scans of the brain at the time of presentation were
or back pain. Major pain in the neck or head was noted in 2 performed in 9 patients, 8 of them with neurological symp-
Figure 1. Classification of initial neurological
symptoms in 30 out of 102 patients with
acute type A aortic dissection.
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TABLE 1. Involvement of the Supra-Aortic Branches and Frequency of Initial and Postoperative Neurological Symptoms
Supra-aortic Vessel Involvement Supra-aortic Vessel Involvement Not Known
Total 102 (100%) Present (44; 43.1%) not Present (53; 52%) (5; 4.9%) P
Initial (preoperative) neurological symptoms
With neurological symptoms 30 (29%) 17 (56.7%) 11 (36.7%) 2 (6.7%) 0.178
Preoperative stroke/TIA 16 (15.7%) 10 (62.5%) 5 (31.3%) 1 (6.1%) 0.169
Postoperative neurological symptoms
With neurological Complication 47 (47.5%) 20 (42.5%) 26 (55.3%) 1 (2.1%) 0.838
Postoperative stroke/TIA 14 (14.1%) 5 (35.7%) 9 (64.3%) 0 0.371
Postoperative cerebral hypoxia 8 (8.1%) 5 (62.5%) 2 (25%) 1 (12.5%) 0.698
No. of patients (%) presented.
TIA indicates transient ischemic attack.
toms, and as expected showed no signs of acute ischemic Therapy, Complications, and Outcome
stroke. Doppler and duplex sonography of the extracranial Surgical Therapy
arteries was performed in one-sixth of all patients (N⫽17), 9 Supracommissural replacement of the ascending aorta was
of them with neurological symptoms, and revealed dissection performed in 46 patients (with intact or resuspendable aortic
in 5 patients. valves), whereas 52 patients (without resuspendable aortic
Ischemic stroke (16 patients) was most frequently referable valves) received a composite replacement. Incidence of
to the carotid circulation (N⫽13; 81.2%), predominantly neurological complications was similar between both groups
right-sided (69.2%). Only 2 of the 9 patients with right (48.1% versus 41.3%; P⫽1.000).
hemispheric stroke showed unilateral dissection of the in-
nominate artery, whereas both carotid arteries were involved Postoperative Neurological Symptoms and Outcome
in 7 patients. Involvement of one or more main branches of Neurological postoperative complications were observed in a
the aortic arch (confirmed by CT or MR angiography, carotid total of 47 patients (47.5%), including 17 patients with initial
Duplex, or intraoperative visualization) occurred in 43.1% of neurological symptoms and additional postoperative neuro-
all patients and 56.7% of patients with initial neurological logical complications. Complications were also divided into
symptoms (P⫽0.178; Table 1). Dissection of the innominate different groups (Figure 2). The observed nerve compression
and carotid arteries was present in 41 patients, involvement of syndromes included Horner syndrome, cardiovocal syndrome
the subclavian artery was present in 25 patients. Preoperative or combinations of these syndromes. Eight of the 47 patients
stroke or transient ischemic attack was only found in 22.7% (17%) with postoperative neurological complications showed
of all patients with dissection of the supra-aortic vessels. various combinations of different neurological symptoms
Figure 2. Classification of 47 postoperative neurological complications in 102 patients with acute type A aortic dissection.
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TABLE 2. Outcome with Special Consideration of Initial and Postoperative Neurological Symptoms
Outcome
Asymptomatic Need of Help Need of Permanent Care Death Total
Total 57 (57.6%) 14 (14.1%) 5 (5.1%) 23 (23.2%) 99*
Patients with initial neurological symptoms 13 (44.8%) 4 (13.8%) 3 (10.3%) 10 (30%) 30
Patients with postoperative neurological 20 (45.5%) 10 (22.7%) 5 (11.4%) 9 (20.5%) 44
complications
*Outcome data of 3 patients were not available.
(spinal and ischemic stroke; ischemic neuropathy and tran- Discussion
sient ischemic attack or postoperative delirium caused by
surgical intervention [DSM IV; 293.0], nerve compression Clinical Features of Aortic Dissections
In aortic dissections, neurological symptoms are often dra-
syndrome and transient ischemic attack, postoperative delir-
matic and may dominate the clinical picture and mask the
ium, or ischemic neuropathy).
underlying condition. The frequency of neurological involve-
Overall in-hospital mortality was 22.6%; patients with
ment varies from 17% to 40% (Table 3).5,6 Many neurological
initial neurological symptoms had a mortality rate of 30%
findings have supposedly been overlooked because of incom-
(P⫽0.300). The mortality rate in the 16 patients with preop-
pleteness of neurological examination in critically ill patients.
erative stroke (38.9%) was higher than that of patients
Remarkably, pain is not an obligatory symptom of aortic
without preoperative stroke (19%, P⫽0.116) but did not
dissection. A total of 13.9% of patients in the present study
reach statistical significance. Perioperative stroke was not noted no pain, matching to the reported ranges of pain-free
related to higher mortality (26.7% versus 21.8% with/without dissections between 5% and 15%.6,7 Approximately half of
perioperative stroke; P⫽0.740). In patients with preoperative the patients who did not report pain solely showed neurolog-
hypotension ⬍110 mm Hg (systolic blood pressure) the ical symptoms. Furthermore, neurological symptoms are
perioperative stroke rate of 20% was higher than in patients often evanescent, fluctuating, and fully remitted before ad-
with normotension (9.3%) but did not reach significance mission to the emergency room. Neurological manifestations
(P⫽0.154). Fifty-seven patients (72% of all survivors) were usually appear at or soon after the onset of dissection. Rapid
asymptomatic at discharge, 5 patients were in need of improvement in such cases is probably the result of only
permanent care, and 14 patients were in need of help in daily transient arterial occlusion at the moment of propagation of
life (Table 2). Although no correlation between occurrence of the dissection. Symptoms of ischemic stroke were the most
postoperative neurological complications and death could be common initial neurological finding. In the present series,
observed (Table 2; P⫽0.478), patients without neurological strokes were more frequently hemispheric compared with
complications were more frequently independent in daily life. vertebral-basilar and predominantly right sided (69.2%)
Significant independent determinants of perioperative or matching to the frequency of 71% reported by Chase et al.8
early death were (mortality with versus without presence of This dominance of right hemispheric stroke despite mostly
the predictor): preoperative hypotension (31.1% versus bilateral carotid dissection perhaps could be explained by
13.0%; P⫽0.047*), preoperative renal impairment (58.3% different mechanical dynamics in the progression of the
versus 17.2%; P⫽0.004**), and history of renal insufficiency dissecting hematoma. Involvement of the major branches of
(43.8% versus 17.6%; P⫽0.042*). Logistic regression anal- the aortic arch was found in 43.1% of all patients in the
ysis showed that preoperative renal impairment and history of present study, most frequently affecting the innominate and
renal insufficiency were best predictors for hospital death: carotid arteries because of their proximity to the aortic arch.
Knowledge of both variables allowed correct prediction of In the preoperative stroke subgroup, the frequency of
death in 79.6%. supra-aortic involvement was 62.5%, and thus clearly higher
TABLE 3. Initial Neurological Symptoms in the Present Study in Comparison With Literature
Present Study Blanco et al3 Aĺvarez et al2 Meszaros et al5 Fann et al24 IRAD6
No. of included patients 102 with type A 24 with type I 90 with type A 75 with type A or B 174 with type A 289 with type A
dissection dissection dissection dissection dissection dissection
Neurological Symptoms 30 (29.4%) 6 (25%) 21 (23%) 30 (40%) * 17%
Ischemic stroke 16 (15.7%) 2 (8.3%) 12 (13.3%) 24 (32%) 17 (6%) (6.1%)
Spinal cord ischemia 1 (1%) 1 (4.2%) 8 (8.9%) 2 (2.7%) (5%) *
Ischemic neuropathy 11 (10.8%) 1 (4.2%) 5 (5.6%) 4 (5.3%) * *
Hypoxic encephalopathy 2 (2%) 2 (8.3%) * * * *
Syncope 6 (5.9%) * 3 (3.3%) * * (12.7%)
*No data available.
N (%) presented.
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compared with the total study population. In one-third of all symptoms in the present study, consistent with literature
patients with preoperative stroke, however, ischemic stroke (Table 3).6,8,16
was not caused by extension of the dissection toward the
supra-aortic vessels, but was caused by other etiological Operative Therapy of Aortic Dissection and
mechanisms such as thromboembolism or severe hypoten- Perioperative Neurological Symptoms
sion. Furthermore, involvement of the supra-aortic vessels Supracommissural replacement and composite replacement,
does not necessarily lead to cerebral ischemia. Only 22.7% of each isolated or combined with replacement of the aortic
all patients with dissection of the supra-aortic vessels present- arch, was applied to an equal number of patients. Matching to
ed with preoperative stroke or transient ischemic attack. previous studies,17,18 incidence of neurological complications
Diagnosis of both acute ischemic stroke and aortic dissec- was similar between groups. The overall frequency of post-
tion is difficult when the presenting signs are atypical. operative neurological complications (47.5% of all patients)
Among our patients we observed a 76-year-old woman in this series was a little higher compared with literature
presenting with acute right hemispheric stroke and without (Table 4), probably resulting from the exact recording and
other clear signs or symptoms of the underlying aortic assignment by neurologists in the present study. Interest-
dissection in whom cardiac tamponade and intrapleural hem- ingly, more than half of patients with initial neurological
orrhage developed after thrombolytic therapy with recombi- symptoms showed additional new postoperative neurological
nant tissue plasminogen activator (rt-PA) and died. Only 2 complications.
other patients treated with rt-PA for acute ischemic stroke The frequency of perioperative stroke (14.1%) in the
resulting from aortic dissection were previously published.9,10 present study was a little higher than previously reported but
Because aortic dissection is a rare cause of ischemic stroke, was not related to higher mortality.18,19,20 In contrast to the
the aggressiveness of screening for this entity has to be predominantly right-sided preoperative strokes, perioperative
questioned. An emergent chest X-ray should be considered as strokes affected equally both hemispheres and were often
part of acute ischemic stroke protocols as well as paying bilateral. Occurrence of postoperative neurological complica-
attention to physical examination findings.11 tions, especially perioperative stroke, did not correlate with
supra-aortic involvement as well. This suggests that other
TGA-Like Symptoms in Patients With perioperative factors such as hypothermic circulatory arrest,
Aortic Dissection suboptimal cerebral protection, or microemboli seem to be
Two patients with TGA-like symptoms caused by aortic responsible for perioperative stroke. Preoperative hypoten-
dissection were classified as ischemic stroke because of the sion in acute type A dissection was described as a predispos-
accompanying focal symptoms. TGA-like symptoms are ing factor toward perioperative stroke (P⬍0.05).20 In the
remarkable in 2 respects. First, TGA may extinguish impor- present study, a higher frequency of stroke in patients with
tant details of a patient’s recent history. One of our patients preoperative hypotension was found, but this did not reach
could not report the sudden onset of severe chest pain, which significance (P⫽0.154).
was the reason for the emergency call, because the memory of Postoperative confusion, agitation was observed in 14.7%
the episode was lost in amnesia. Second, the accompanying of patients, comparable to the reported frequency after car-
minor neurological deficits suggest an arterial-ischemic eti- diopulmonary bypass and surgery of ascending aorta.18,19,21
ology of TGA as the result of aortic dissection.12,13 In 2 The specific cause of postoperative delirium is unclear but is
comparable, recently published patient reports, authors pos- likely associated with subclinical microemboli or generalized
tulated that the acute pain of aortic dissection triggered a cerebral malperfusion.19
stress reaction responsible for TGA.14,15 There is conflicting evidence concerning the question
Disorders of consciousness or syncopes and epileptic whether presence of neurological symptoms is an indepen-
seizures frequently occur at the onset of dissecting aneurysm. dent predictor of poor outcome or in-hospital death in patients
They were present in half of all patients with neurological with acute type A aortic dissection. Although our study, the
TABLE 4. Postoperative Neurological symptoms in the Present Study in Comparison With Literature
Present Study Blanco et al3 Fann et al24 IRAD6
No. of included patients 99 with type A 24 with type I 174 with type A 1078 with type A and B
dissection dissection dissection dissection
Neurological symptoms 47 (47.5%) 4 (16.5%) * 223 (22.8)
Ischemic stroke 14 (14.1%) 0 (0%) (4%) 0 (0%)
Spinal cord ischemia 4 (4%) 0 (0%) * *
Ischemic neuropathy 3 (3%) 1 (4.2%) * *
Hypoxic encephalopathy 8 (8.1%) 3 (12.5%) * *
Nerve compression syndrome 7 (7.1%) 0 (0%) * *
Postoperative delirium 15 (14.7%) * * *
*No data available.
N (%) presented.
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IRAD,6 and Fann et al.24 revealed no such correlation, other 6. Hagan PG, Nienaber CA, Isselbacher EM, Bruckman D, Karavite DJ,
studies identified preoperative neurological deficit as a sig- Russman PL, Evangelista A, Fattori R, Suzuki T, Oh JK, Moore AG,
Malouf JP, Pape LA, Gaca C, Sechtem U, Lenferink S, Deutsch HJ,
nificant predictor of hospital death.18,22,23 The overall in- Diedrichs H, Marcos y Robles J, Llovet A, Gilon D, Das SK, Armstrong
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Conclusion Surgical management of acute type A aortic dissection with a complaint
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1053–1056.
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Additionally, in case of aphasia, unconscious or TGA patients dissection: caution in the thrombolytic era. Stroke. 1999;30:477– 478.
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A Evolving strategies for treatment of acute aortic dissection type A.
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The authors thank Prof Dr S. Zierz for critical review of the 18. Ehrlich MP, Ergin A, McCullough JN, Lansmann SL, Galla JD, Bodian
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Charly Gaul, Wenke Dietrich, Ivar Friedrich, Joachim Sirch and Frank J. Erbguth
Stroke. 2007;38:292-297; originally published online December 28, 2006;
doi: 10.1161/01.STR.0000254594.33408.b1
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