Occlusive disease of the great vessels—the innominate artery, the common carotid arteries, and the subclavian arteries—is uncommon when compared to carotid bifurcation, aorto-iliac, and femoral-popliteal artery occlusive disease. The most common etiology of brachiocephalic occlusive disease in the United States is atherosclerosis, followed by Takayasu arteritis and radiation arteritis. Arterial thoracic outlet symptom also may give rise to subclavian-axillary artery aneurysmal and occlusive disease, with or without atheroembolic lesions of the upper extremity. That condition is covered in another chapter.

Symptomatic atherosclerotic brachiocephalic artery occlusive disease is the most common indication for reconstruction of the great vessels. Occlusive disease of the innominate artery is the most common reason for median sternotomy and aortic-origin reconstruction. Subclavian artery occlusive disease, on the other hand, is the most commonly treated of the great vessel lesions. Common carotid artery occlusive disease is the least encountered entity. Patients are usually encountered in their fifth or sixth decades. Men and women are equally divided, and in some series women represent the majority of patients. Significant risk factors include smoking and hypertension. Coronary artery disease is a well-recognized comorbid condition.

Takayasu arteritis is the second most common etiology of great vessel occlusive disease in the United States. It is much more common in women than in men, and, in general, it occurs in women <45 years of age. Types I and III involve the great vessels, and Type III is the most commonly encountered pattern, with disease in the aortic arch and its branches and the distal thoracic and upper abdominal aortas and their branches. Involvement of the coronary arteries at their ostia with arteritis occurs infrequently.

Great vessel occlusive disease caused by radiation arteritis is the third most commonly encountered form in the United States. This relates to external beam radiation to the upper mediastinum and neck for a variety of malignancies. Radiation-induced changes include intimal proliferation and fibrosis and disruption of the elastic lamina. There is an ischemic necrosis of the arterial wall secondary to destruction of the vasa vasorum. Patterns of disease are related entirely to the treatment fields and dosages.

The diagnosis of occlusive disease of the brachiocephalic vessels should be suspected when patients present with cerebral and/or upper-extremity symptoms compatible with ischemia or microembolization. Those cerebral symptoms include lateralizing anterior symptoms, such as transient ischemic attack (TIA), amaurosis fugax , and stroke, as well as nonlateralizing symptoms such as bilateral visual disturbances, ataxia, and vertigo, or any of the myriad manifestations of vertebrobasilar disease. Cerebrovascular symptoms in the presence of brachiocephalic occlusive disease may be of the anterior (carotid) or posterior (vertebral) circulations; because of the peculiar anatomy of the innominate artery and its primary branches, symptoms of both (global ischemia) may be present at the same time with innominate artery stenoses. Global ischemia may also be encountered with multiple great vessel lesions involving the common carotid and subclavian arteries. Neurologic symptoms arising from brachiocephalic great vessel disease are most frequently caused by involvement of the innominate artery.

Upper-extremity ischemic pain with use, arm fatigue, or generalized aching (all classed as “claudication”) or digital ischemia with microemboli or ulcerations, especially if unilateral, are indicative of subclavian or innominate artery occlusive disease. These may be present as solitary symptoms or may present in conjunction with neurologic symptoms.

With the widespread use of internal mammary arteries as conduits to revascularize the coronary arteries, coronary ischemia or “coronary steal” is recognized as a presenting symptom of subclavian (or innominate) artery disease with angina as the chief complaint.

Finally, great vessel disease, and most especially left subclavian artery disease, may be asymptomatic and discovered on physical examination or during workup for carotid bifurcation or coronary artery disease.

A detailed history and physical examination are paramount in the accurate diagnosis of brachiocephalic occlusive disease. Descriptions of the sudden appearance of painful, discolored, bluish lesions of the fingers, especially when unilateral, are indicative of subclavian disease. Global ischemia may be described as light intolerance as well as by vertebrobasilar and localizing anterior cerebrovascular symptoms. The presence of proximal carotid or mediastinal bruits, diminished pulses, inequality of upper-extremity pulses, absence of upper-extremity pulses, or unilateral digital microemboli or
gangrene should alert the clinician to the real possibility of stenotic disease of these vessels. Whereas physical examination of the carotid bifurcations is as often unrewarding as it is helpful, careful physical examination of the origins of the great vessels including palpation and auscultation of the upper mediastinum, chest, proximal neck, and upper extremities can be very predictive of great vessel disease location and, sometimes, severity. The presence of livedo reticularis or microemboli or subungual hemorrhage should alert the clinician to the possibility of microemboli. Palpable radial and ulnar pulses do not rule out these lesions. Claudication is usually encountered with highly stenotic or occlusive lesions of the subclavian or innominate arteries; microemboli are seen with less stenotic lesions. Bilateral digital gangrene or upper-extremity ischemia is more indicative of systemic problems than of vascular occlusive disease and should prompt workup for collagen vascular and rheumatoid disease states.

Ultrasound may reveal increased proximal common carotid flow velocities, increased subclavian artery velocities, or occlusion of these vessels, as well as reversal of flow in the vertebral artery. However, the bony structures of the upper mediastinum—ribs, sternum, and clavicles—limit the precision and usefulness of ultrasound in the diagnosis of great vessel disease. Arch and four-vessel arteriography has been the sine qua non of diagnosis. It allows precise delineation of the lesions and planning of operation. It also allows differentiation between atherosclerosis and Takayasu arteritis. It is probably being supplanted by computed tomographic angiography (CTA) with color reconstructions. CTA does not carry the risk of stroke that conventional arteriography does, and its clarity and detail allow precise diagnosis and operative planning. The advent of 64-image computed tomography (CT) will only increase the usefulness of this modality in the future. Patients with great vessel disease and neurologic symptoms deserve CT of the brain in addition to their arterial studies. Studies in patients with upper-extremity ischemia should include distal forearm, hand, and digital views, as well.

Indications for operative repair of brachiocephalic occlusive lesions include the cerebrovascular symptoms described above, as well as upper-extremity symptoms in the presence of the appropriate great vessel occlusion or stenosis. Concomitant carotid bifurcation lesions are repaired at the same time. In patients with multiple proximal lesions and a carotid bifurcation lesion, without ipsilateral proximal common carotid or innominate artery stenosis, it is recommended that the carotid bifurcation lesion be repaired first. If symptoms are alleviated by that operation, then the necessity for a median sternotomy and proximal reconstruction is avoided. Approximately threequarters of patients undergoing innominate artery reconstruction might be expected to have neurologic symptoms, with approximately 50% being attributable to the anterior circulation, 40% to the posterior, and 10% to both. Combined upper-extremity and neurologic symptoms can be expected to be present in 20% to 40% of patients. Unilateral microemboli to an extremity are an especially urgent cause for intervention; the subclavian artery and innominate artery are much more prone to embolize distally than other sites, and the extent of distal ischemia is independent of the degree of stenosis. Patients with coronary steal syndrome are offered either carotid-subclavian artery bypass graft or catheter-based intervention. Subclavian artery transposition is not recommended for those patients because of the prolonged coronary ischemia consequent to clamping and division of the artery proximal to the internal mammary artery. Bypass grafting, on the other hand, is accomplished with clamps distal to the internal mammary artery, allowing the native circulation to remain uninterrupted until the reconstructed artery is opened. Patients with asymptomatic innominate artery and common carotid artery lesions are usually not offered operation, as the natural history of these lesions is unknown. Retrospective studies would indicate that the morbidity and mortality are greater than those for carotid bifurcation disease and, therefore, extrapolation of data from studies concerning carotid bifurcation natural and operative history is not valid. Exceptions to that nonoperative rule include patients needing coronary artery bypass grafting who have stenotic brachiocephalic lesions and patients with concomitant carotid bifurcation disease that has progressed past an 80% stenotic level and requires operation in its own right. In addition, consideration should be given to operation for especially young, otherwise healthy, patients with multiple tight stenoses.

Asymptomatic subclavian artery occlusive disease is the most commonly encountered of the great vessel occlusive lesions. The left subclavian artery is involved approximately 70% of the time, and the right is involved in the remaining cases. Isolated subclavian lesions by themselves rarely cause claudication severe enough to warrant intervention. In most cases of symptomatic left subclavian artery occlusive disease with claudication, multiple lesions of the great vessels, vertebral arteries, or carotid bifurcations are present as well as the subclavian lesion. In the largest series, multiple great vessel lesions are present in anywhere from two-thirds to three-quarters of patients encountered. The reversal of vertebral blood flow seen with left subclavian artery lesions is not an indication in itself for operation and represents a normal collateral flow pattern. It is important to distinguish radiographic (or ultrasonographic) vertebral steal from a symptomatic steal. The former is a finding and not an indication for repair.

Microemboli from an isolated subclavian or innominate artery lesion, on the other hand, are indications for operation. Such lesions are often present in the absence of other great vessel disease.

The most common cause of early and late death in most series remains coronary artery disease. Patients with nonreconstructible coronary lesions should be considered for medical and/or catheter-based therapy if feasible.

In addition, there are subsets of patients who do not respond as well to direct transsternal reconstruction as do the majority of patients. Patients with renal insufficiency have an increased peri-operative combined stroke and death rate. Patients with thrombophilia have an increased peri-operative stroke rate and an increased late reconstruction thrombosis rate. Patients with radiation arteritis have a greater risk of late stroke and death primarily due to an increased late infection rate.