Supra-aortic vessels aneurysms: Diagnosis and prompt intervention
Article Outline
Purpose
Aneurysms involving the supra-aortic vessels are rare but carry serious risk of embolization, thrombosis, and rupture. We describe our experience with the diagnosis, treatment strategies, and outcomes in patients with extended follow-up.
Methods
Data during a 17-year period (January 1990 to December 2007) was analyzed. We assessed age, gender, presenting symptoms, localization, pathologic diagnosis, type of procedures, complications, and survival.
Results
A total of 74 patients were treated for supra-aortic aneurysms. Of all aneurysms treated, 63% were degenerative, 24% iatrogenic, 8% traumatic, 3% genetic, and 1% mycotic. The subclavian artery was most commonly affected (50%, 2/3 in the right side), followed by the common carotid (36%), internal carotid (10%), innominate (3%), and vertebral (1%). At the time of diagnosis, 52 patients (70%) were asymptomatic, but of those symptomatic 68% had an embolic event as a presenting symptom. Embolic episodes were more common in patients with smaller aneurysms (P < .006). Open surgery was performed in 77% of all cases, and the use of endovascular techniques became the predominant treatment modality over the last 4 years. Survival at 30 days was 100%. Five- and 10-year survival rates were 87% and 43%, respectively.
Conclusion
Most cases of supra-aortic aneurysm are asymptomatic and embolization as opposed to rupture represents the greatest risk to the patient. Most cases can be detected prior to symptoms. Endovascular repair is an emerging alternative of treatment and, with the current development of appropriate devices, will likely form the mainstay of therapy in the near future.
Aneurysms involving the supra-aortic arteries (subclavian, innominate, carotid, external carotid, and vertebral arteries below the skull base) represent an uncommon form of aneurysmal disease.1 The first surgical intervention performed on these types of aneurysms was reported in 1805 by Sir Astley Cooper in a patient who had a carotid artery aneurysm that was successfully treated by vessel ligation.2 In subsequent years, 173 aneurysms have been reported (Table I). The etiology of these aneurysms may be similar to those located in the aorta which are commonly degenerative.3 However, other etiologies such as congenital aneurysms,4 traumatic pseudoaneurysm,5, 6 vasculitic disease,7 connective tissue disorders,8 or infections9, 10, 11, 12 are more often diagnosed compromising the supra-aortic vessels. Supra-aortic aneurysms comprise between 0.4 and 4% of all aneurysms.13, 14, 15, 16
Table I. Large series published in the literature for supra-aortic aneurysms⁎
| Author | Year | Number of cases | Follow-up | Localization | ||||
|---|---|---|---|---|---|---|---|---|
| Subclavian | Common carotid | Internal carotid | Innominate | Vertebral | ||||
| Beall, A et al32 | 1962 | 7 | 11 yrs | 0 | 3 | 4 | 0 | 0 |
| Pairolero, P et al33 | 1981 | 31 | 20yrs | 31 | 0 | 0 | 0 | 0 |
| Thomas, T et al46 | 1989 | 5 | 2yrs | 0 | 0 | 0 | 5 | 0 |
| Bower, T et al13 | 1991 | 73 | 40yrs | 41 | 25 | 0 | 6 | 1 |
| Prêtre, R et al34 | 1994 | 14 | 8yrs | 11 | 1 | 0 | 2 | 0 |
| Regina, G et al29 | 1999 | 16 | 10yrs | 6 | 4 | 0 | 6 | 0 |
| Kieffer, E et al25 | 2001 | 27 | 27yrs | 0 | 0 | 0 | 27 | 0 |
| Current Series | 2008 | 74 | 17yrs | 37 | 27 | 8 | 2 | 1 ⁎⁎ |
| Total | 247 | 126 | 60 | 12 | 48 | 2 ⁎⁎ | ||
⁎There are no large series reporting endovascular treatment of supra-aortic aneurysms, just isolated case reports. |
⁎⁎Multiple aneurysms. |
Similar to many other aneurysms, supra-aortic vessel aneurysms pose serious risks including: rupture, thrombosis, and local compression, but also have an associated risk of catastrophic brain embolization and stroke. The incidence of neurologic events had been reported to be as huge as 50%17 with a mortality rate close to 70% in patients left untreated.18 Many variations of presenting symptoms have been described, including dysphagia, hoarseness, Horner's syndrome, supraclavicular pain, transient ischemic attacks (TIA), stroke, or the presence of a palpable mass.19 The use of radiological modalities, especially computed tomography (CT), are essential both to confirm the diagnosis and to plan repair.20, 21, 22, 23 Surgical resection of the aneurysm followed by an interposition has been the standard treatment.6, 24, 25 However, with the rapid evolution of endovascular techniques, novel approaches for the treatment of supra-aortic aneurysms have been described.26, 27, 28 The aim of this study is to examine our experience with aneurysms of the supra-aortic vessels, their presenting symptoms, diagnostic modalities used, type of intervention performed, and outcomes in patients with extended follow-up.
Methods
A consecutive cohort of patients who were diagnosed with supra-aortic vessel aneurysm at the Cleveland Clinic Foundation (CCF) from January 1990 to December 2007 was retrospectively analyzed using both clinical notes and radiological studies. The following variables were taken into consideration: gender, age, pathologic diagnosis, location, presenting symptom, imaging modality used (arteriography, ultrasound [US], CT), size and type of aneurysm, duration and type of procedure, intra-operative blood loss, type of grafts (prosthetic, autogenous vein, or stent graft) and complications. Surgical intervention and follow-up was performed at the discretion of the treating surgeon.
Categorical variables were shown as number (percentage) and continuous variables as median (interquartile range [IQR]). We compared patients' characteristics among open surgery, endovascular surgery, and no interventional treatment groups by using the Fisher exact test for categorical variables and the Wilcoxon rank sum test for continuous variables. Kaplan-Meier curves were generated to assess overall survival. Mortality was determined based on patient medical records and supplemented by querying the Social Security Death Index at the time of analysis. Follow-up times were calculated based on the last clinical contact with the patient at the CCF, or through communication from an outside hospital that provides surrogate follow-up on behalf of the CCF. A P value of < .05 was considered significant. Statistical analyses were performed using S-Plus 7.0 (Insightful Corp, Seattle, Wash).
Results
A total of 74 patients with supra-aortic vessel aneurysms were identified (64% men) with a median age of 69 years (IQR 49-76) and a mean follow-up of 29 months. Six patients did not return for scheduled follow-up studies following their first postoperative visit. Patient characteristics overall and across treatment groups are shown in Table II. The majority (63%) of cases were attributed to atherosclerotic degeneration. Age of presentation is indicative of etiology, as most patients presenting with degenerative aneurysms seems to fall in the >60-year-old age group (Fig 1). In the 40-60-year-old group, degenerative aneurysms were found in 53%, iatrogenic in 29%, traumatic in 12%, and 6% had Ehlers-Danlos syndrome. Table III describes the modality and clinical scenario in which asymptomatic patients were discovered.
Table II. Demographics of patients presenting to our institution with supra-aortic aneurysms
| Characteristic | Total (n = 74) | Open surgery (n = 57) | Endovascular surgery (n = 11) | No surgical treatment (n = 6) | P |
|---|---|---|---|---|---|
| Age (y) | 69(49-76) | 68(50-76) | 70(35-76) | 70(69-76) | .5 |
| Male | 47(64) | 36(63) | 7(64) | 4(67) | .8 |
| Localization of aneurysm | .6 | ||||
| Common carotid | 27(36) | 23(40) | 2(18) | 2(33) | |
| Internal carotid | 8(10) | 7(12) | 1(9) | 0 | |
| Subclavian | 37(50) | 25(44) | 8(73) | 4(67) | |
| Innominate | 2(3) | 2(4) | 0 | 0 | |
| Vertebral⁎ | 1(1) | 1(1) | 0 | 0 | |
| Size(mm) | 30(22-40) | 30(22-38) | 45(40-47) | 22(22-30) | .005 |
| Cause | .05 | ||||
| Atherosclerotic | 47(63) | 35(61) | 7(64) | 5(83) | |
| Iatrogenic | 18(24) | 16(28) | 2(18) | 0 | |
| Ehlers-Danlos | 2(3) | 0 | 2(18) | 0 | |
| Mycotic | 1(1) | 1(2) | 0 | 0 | |
| Traumatic | 6(8) | 5(9) | 0 | 1(17) | |
| Symptoms | .3 | ||||
| Asymptomatic | 52(70) | 38(67) | 9(82) | 5(71) | |
| Dysphagia | 2(3) | 1(2) | 0 | 1(17) | |
| Hoarseness | 2(3) | 2(4) | 0 | 0 | |
| TIA | 9(12) | 7(12) | 2(18) | 0 | |
| Arm claudication | 9(12) | 9(16) | 0 | 0 | |
| Smoking | 34(46) | 26(46) | 5(45) | 3(50) | .9 |
| Diabetes | 4(5) | 4(7) | 0 | 0 | .5 |
| COPD | 6(8) | 4(7) | 2(18) | 0 | .4 |
| CAD | 19(26) | 9(16) | 6(55) | 4(67) | .002 |
| Extra aneurysm | 22(30) | 14(25) | 4(36) | 4(67) | .09 |
| Complications Pre-treatment | 17 | 13 | 2 | 2 | .4 |
| Embolization | 13(76) | 11(85) | 1(50) | 1(50) | |
| Compression | 4(24) | 2(15) | 1(50) | 1(50) | |
| Complications Post-treatment | 16 | 14 | 1 | 1 | .7 |
| Embolization | 4(25) | 4(29) | 0 | 0 | |
| Occlusion | 3(19) | 3(21) | 0 | 0 | |
| Re-intervention | 3(19) | 3(21) | 0 | 0 | |
| Death | 6(38) | 4(29) | 1(100) | 1(100) |
⁎Multiple aneurysms; TIA, transient ischemic attack; COPD, chronic obstructive pulmonary disease; CAD, coronary artery disease. |
Fig 1.
Histogram represents the age at presentation in the three different age groups correlated with etiology.
Table III. Supra-aortic aneurysms found incidentally by complimentary exams
| Computed tomography | 19 |
| Cancer screening or follow-up | 5 |
| Associated aortic aneurysm | 14 |
| Ultrasound | 13 |
| Post-op vascular surgery(CEA) | 13 |
| X-ray | 5 |
| Pace maker review | 2 |
| Congestive heart failure screening | 3 |
| Echocardiogram | 3 |
| Post-op valve replacement | 3 |
The subclavian artery was the vessel most commonly affected (50%), and 2/3 of the cases were on the right side, followed by the common carotid artery with 27 cases (36%), internal carotid with 8 cases (10%), innominate with 2 cases (3%), and vertebral in 1 case with bilateral presentation (1%). Of the 37 subclavian artery aneurysms, 7 had an aberrant right subclavian artery. The median size of all the aneurysms at the time of diagnosis was 30 mm (IQR 22-40). Fifty-two of 74 patients were asymptomatic at the time of diagnosis (70%) and 15 patients (20%) had suffered embolization (9 strokes or TIA and 6 upper-limb embolization). In the subgroup of 9 patients who developed cerebral emboli were, on average, 68-years-old and had a median interval between aneurysm diagnosis and embolic first subsequent event was 61 days (IQR 59-184). The average time between embolic event and treatment was 8 days. The remaining 7 (9%) symptomatic patients presented with arm claudication (three cases), dysphagia (in two cases), or hoarseness (in two cases). An additional 13 embolic events occurred during follow-up prior to treatment, 7 of those were in patients with previous embolization. Overall, 28% of patients with supra-aortic vessel aneurysm developed problems with embolization. Interestingly those patients that had embolization, only 24% (5/21) of them had visible thrombus within the aneurysm sac on CT scan. Also of interest, embolic episodes (strokes, TIA, upper limb embolization, and claudication) were more common in patients with smaller aneurysms (P < .006). There was no statistical significance regarding the location of the aneurysm and the symptoms.
Sixty-eight (92%) of the patients underwent an intervention, of which 57 (77%) underwent an open surgical repair (including all 7 patients with aberrant subclavian), 11 (15%) underwent endovascular repair: 8 subclavian - one of those had a hybrid repair with associated open repair of the vertebral artery (Fig 2), 2 common carotid (Fig 3) and 1 internal carotid artery (Fig 4). In the 11 patients treated with an endovascular approach, 8 had aneurysmal sac size contraction (73%), while 3 patients maintained a stable maximal aneurysm diameter. There were no endoleaks or aneurysm sac growth noted over the follow-up period. A history of either prior aortic aneurysm repair or concomitant aortic aneurysm was noted in 22 patients. One patient with a history of aortic dissection was identified in this series. Complications while awaiting intervention occurred in 17 patients, of which embolization accounted for 13/17 (76%), symptoms from local compression developed in 4/17 (24%). Complications associated with the procedure occurred in 12 patients but with no statistical significant difference between treatment options (P < .7, Table II). Freedom from complications in all patients is shown in Fig 5.
Fig 2.
A, 3D reconstruction of a computed tomography (CT) scan shows a normal aortic arch with a left subclavian aneurysm and a tortuous aneurysmatic right vertebral artery. B, Same patient postintervention demonstrates a patent left subclavian stent in situ (arrow), a right vertebral to right carotid transposition (arrow) excluding the vertebral aneurysm, and retrograde flow to the left vertebral artery through the posterior cerebral artery (arrow).
Fig 3.
A and B, 3D reconstruction computed tomography (CT) scan demonstrates a large left common carotid aneurysm (anterior/posterior [AP] and lateral views).
Fig 4.
A and B, 3D reconstruction computed tomography (CT) scan shows a patent internal carotid stent (arrow) excluding an internal carotid pseudo-aneurysm from a previous open repair with PTFE graft (arrow) (anterior/posterior [AP] and lateral views).
Fig 5.
Kaplan-Meier curve shows freedom from complications in all patients. The solid line represents the Kaplan-Meier survival curve and dotted lines represent the upper and lower 95% confidence intervals.
In the patients who underwent open surgery, 47 had carotid-subclavian or carotid-carotid bypass, 9 had subclavian to axillary artery interposition, and 1 had thoracotomy with left subclavian ligation and left common carotid to axillary artery bypass using a vein as a conduit. Patients selected for endovascular approach had relative contraindications to open surgical repair: 4 had previous neck surgeries, 4 had previous thoracic surgeries, 2 had connective tissue disease that made open surgery higher risk (both were Ehlers-Danlos type IV – Fig 2), 1 had multiple medical comorbidities with an aneurysm which was too high to access surgically. When the proximal aspect of the innominate right or left common carotid arteries were involved in the repair, an extra-anatomic bypass with polytetrafluoroethylene (PTFE) was created (carotid-subclavian bypass), typically followed by the deployment of a thoracic stent graft. However, in the setting of an isolated left subclavian artery aneurysm, the arterial origin was most commonly covered in the absence of revascularization. The distal flow into the aneurysm was treated with endovascular coils or glue.
Indications for non-operative management were present in 6 patients. These included advanced age, high surgical risk, or refusal of the procedure. In the untreated subset of patients, 2 developed a stroke (33%), of which 1 expired as a result. None of them had aneurysm growth in the period of follow-up of 41 months.
Thirty-day survival was 100% (95% confidence interval [CI] 100-100), and 5- and 10-year survival rates were 87% (95% CI 76-99) and 43% (95% CI 11-100), respectively. The mean survival time was 91 months (standard deviation [SD] 41 months).
There were 6 late deaths (>30 days) in our series (8% of the cases) in which one was procedure-related (retroperitoneal hemorrhage following diagnostic angiogram needed to further access the anatomy and plan the procedure at day 39). Three deaths were due to concomitant thoracic or thoracoabdominal aortic aneurysms (two as a result of rupture, and one following elective repair). The remaining patients died as a result of a stroke and ovarian cancer. Overall survival is demonstrated in Fig 6.
Fig 6.
Kaplan-Meier curve shows overall survival in patients with supra aortic aneurysms. The solid line represents the Kaplan-Meier survival curve and dotted lines represent the upper and lower 95% confidence intervals.
Discussion
Most publications found in the medical literature regarding supra-aortic aneurysms, are either from small series or isolated case reports, which account for 200 cases in the English literature (Table I) in the last 100 years. Aneurysms of the supra-aortic vessels had been reported to be symptomatic in up to 50% of patients.29, 30, 31, 32, 33 However, we found that in contemporary practice they are more commonly discovered following screening or follow-up exams such as CT, x-rays, or US for asymptomatic patients (Table III), with just 25% of patients presenting with symptoms at the time of diagnosis. An association between supra-aortic vessel aneurysm and aortic aneurysm has been reported,34,35 and in accordance with this we found that several of our patients who had a supra-aortic aneurysm were diagnosed during the work-up for other aortic pathology. This included associated thoracic and thoracoabdominal aortic aneurysm as well as thoracic aortic dissection. Once diagnosed, any supra-aortic vessel aneurysm should be considered a clinically significant lesion as it has the potential of significant complications if left untreated. Overall, 28% of our patients suffered from embolization, 15 as a presenting symptom and 6 following the diagnosis but prior to any treatment. In our series, the incidence of aneurysm-related death was 5% (which three were related to an associated aortic aneurysms and one as a stroke in a patient with no intervention) and permanent disability as a consequence of stroke was 5%.
Innominate artery aneurysms had been described as the most common supra-aortic aneurysm.19,36-38 Due to their rarity, a few reports have analyzed these aneurysms as an entire group.39-41 Kieffer et al42 reported one of the largest series of innominate artery aneurysms treated with surgical repair. Seventeen patients (63%) had associated aortic aneurysms, 14 patients were asymptomatic at the time of operation, and the most common underlying etiologies were vasculitis in 7 patients, degenerative disease in 6, and syphilis in 5. In contrast to prior reports, our series noted a predominance of subclavian artery aneurysms, followed by the common carotid and then the internal carotid. Although historically innominate, aneurysms were commonly reported, there were 2 patients (3%) in our series that were treated for isolated aneurysms of the innominate artery, which may relate to the predominant degenerative etiology in 63% of patients. A concomitant history of aortic aneurysms was noted in 22 patients (30%), supporting the relationship between these two entities.
Advances in surgical techniques over the last century have provided feasible ways to treat the majority of supra-aortic aneurysms by using an interposition graft, which has been established as a gold standard treatment. However, the open surgical approach for aneurysms of the proximal supra-aortic vessels is invasive and may involve a median sternotomy with or without cervical extension. Open repairs become even more complex in the settings of a supra-aortic vessel aneurysm associated with aortic arch pathology, necessitating the use of deep hypothermic circulatory arrest (DHCA). The estimated perioperative mortality rates for emergency and elective repair of aneurysms involving the aortic arch is 50% and 4%, respectively.43 In the last decade, stent graft technology has evolved remarkably and has now been applied as an alternative way of treatment for supra-aortic aneurysms.
The later patients in our series were all treated with commercially-available stent grafts combined with balloon angioplasty. Our results suggest that such devices can be used safely and efficiently to exclude true aneurysms or pseudoaneurysms of the supra-aortic arteries with very low complication rates when comparing to open surgical repair. Although aneurysms of the distal supra-aortic vessels were frequently amenable to pure endovascular repair, more proximal aneurysms often required hybrid procedures, sometimes involving extra-anatomical bypass of the neck vessels followed by thoracic endografting.44,45 The advantages of this technique are the avoidance of aortic cross-clamping and the need for DHCA.
Although our study is a descriptive retrospective analysis from a single institution, it is the largest series published up to date and the largest including endovascular treatment. Because of the rare nature of this entity, the development of randomized control trials for its treatment is unrealistic, and clinicians rely upon these types of publications to help guide management decisions. There are, however, limitations to our work. A retrospective analysis carries an innate selection bias that relies on the clinical suspicion and reporting practices of clinicians outside of a standardized study environment. The subgroups reported (non-operative treatment and endovascular repair) are quite small, and thus may be subject to type II error when intergroup comparisons are made. Furthermore, this is a series from a highly specialized referral care center, and patients who attend the CCF may be different than those who present to general tertiary care centers, which affect the generalizability of the results. We also suspect that recent improvements in imaging modalities have lead to a better assessment of the aortic arch vessels and earlier detection of these types of aneurysms. Thus, the patients and population earlier in the series may differ from more recent experience. Despite these caveats, we believe that when diagnosed, patients with any type of supra-aortic vessel aneurysm should undergo either open, endovascular, or hybrid repair as the risk of devastating complications is there and the repair can be achieved with low mortality and morbidity rates.
In conclusion, after a thorough review of 17 years of experience with supra-aortic vessel aneurysms at a single center, we have found that most cases are asymptomatic at presentation if patients with concomitant thoracic aortic pathology are imaged and that embolization poses the major risk. Embolization phenomenon were more common in patients with smaller size aneurysms. This may cause physicians to advocate prompt intervention not just based only on the size but on the presence of a supra-aortic aneurysm itself. Recent experience suggests that endovascular therapy is an emerging alternative of treatment with good results and low mortality and with the current development of appropriate devices, will likely form the mainstay of therapy in the near future.
Author contributions
References
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Competition of interest: none.
PII: S0741-5214(08)01449-3
doi:10.1016/j.jvs.2008.08.088
© 2009 The Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.
