An aneurysm is defined as a persistent, abnormal dilatation of an artery to 1.5 times its normal diameter.
A thoracic aortic aneurysm can involve the ascending aorta or aortic root (60%), aortic arch (10%), descending aorta (40%), or thoracoabdominal aorta (10%) segments*.
Whilst thoracic aneurysms are less common than abdominal aortic aneurysms, they are associated with high mortality. They have an incidence of 6 in 100,000 person-years, with an increasing prevalence with age.
*This classification is important because aneurysms in different locations present with different symptoms, and have different levels of surgical risk.
Aetiology
Thoracic aortic aneurysms develop due to degradation of the tunica media, the layer of the artery which provides tensile strength and elasticity to the wall. As a result, the artery loses structural integrity and dilates, and as the diameter increases, the wall tension rises and further increases the diameter in a vicious cycle.
Figure 1 – Lateral view of the thoracic aorta, including the intercostal branches
The main causes of thoracic aneurysm are:
-
Connective tissue diseases
- Marfan’s syndrome or Ehlers-Danlos syndrome.
-
Bicuspid aortic valve
- Increased risk of thoracic aortic aneurysms is seen in patients with Turners syndrome (from increased risk of bicuspid aortic valve)
- Trauma
- Aortic dissection
- Aortic arteritis, e.g. Takayasu Arteritis
- Tertiary syphilis
Thoracic aortic aneurysms grow at a mean rate of 1-2mm/year. This rate is higher in those with Marfan’s syndrome, descending aneurysms (compared to ascending aneurysms), and a dissected aneurysm (compared to a non-dissected).
Risk Factors
The main risk factors for developing a thoracic aortic aneurysm include family history*, hypertension, atherosclerosis (specifically descending aneurysms), smoking, high BMI, male gender, and advancing age.
*19% of patients have a positive family history
Clinical Features
Typically thoracic aneurysms are asymptomatic and are found incidentally.
In those that are symptomatic, the most common presenting complaint is pain, with the location of the pain potentially localising the aneurysm:
| Site of Aneurysm | Location of Pain |
| Ascending aorta | Anterior chest |
| Aortic arch | Neck |
| Descending aorta | Between the scapulae |
Table 1 – Site of Thoracic Aneurysm vs. Typical Associated Location of Pain
Other symptoms of thoracic aneurysms include:
- Back pain – secondary to spinal compression by descending or thoracoabdominal aneurysm
- Hoarse voice – from damage to the left recurrent laryngeal nerve in arch aneurysms
- Distended neck veins – from SVC compression
- Symptoms of heart failure – from involvement of the aortic valve
- Dyspnoea or cough – secondary to tracheal or bronchial compression
Clinical signs are not commonly found on examination, however chronic disease may present with the signs of aortic root disease or heart failure.
Thoracic aneurysms have a risk of rupture or dissection, which are potentially lethal. An acute aortic syndrome will present with sudden onset pain in the back, chest, neck, and/or abdomen
Differential Diagnosis
As thoracic aneurysms are mostly found incidentally on imaging, other differential diagnosis are rarely considered before the definitive diagnosis is determined.
In symptomatic patients, the scope of symptoms for a thoracic aneurysm is wide. However for those presenting with chest or back pain (the most common presentation), diagnoses of ACS, PE, pneumothorax, and aortic dissection should all be considered.
Investigations
Thoracic aneurysms are diagnosed through imaging. However, initial in-patient and pre-operative testing should include routine bloods (FBC, U&Es, clotting) with a group and save, ECG, and CXR performed.
Imaging
Many thoracic aneurysms are first identified as incidental findings on imaging.
A thoracic aneurysm can be seen on CXR demonstrating a widened mediastinal silhouette, an enlarged aortic knob, and possible tracheal deviation. However, a radiograph is not sensitive enough to make the definitive diagnosis and further imaging is required
A CT chest scan with contrast is the preferred imaging modality for thoracic aneurysms, providing sufficient detail to ascertain the level and the size of the aneurysm*.
Transoesophageal echocardiography (TOE) can be used to good effect to further detect any concurrent aortic insufficiency or dissection; TOE should form part of the routine assessment of patients with Marfan’s disease and suspected thoracic aortic disease.
*Due to the potentially tortuous nature of the thoracic aorta and the use of axial images in CT scans, care must be taken to ensure the actual aortic diameter is being measured, not simply measuring through the aorta off-axis; reconstructing axial imaging into 3D images via CT angiography or MR angiography can overcome this issue
Figure 2 – X-ray of a thoracic aortic aneurysm. A – Arrow marks the lateral border of the aorta. B – after endovascular repair.
Management
Despite limited evidence, patients with a confirmed thoracic aneurysm should be started on medical management*, alongside any concurrent further investigations and definitive management planned.
These patients are at increased cardiovascular risk, therefore should be initiated on statin and antiplatelet therapy to decrease the risk of myocardial infarction. Blood pressure should be controlled and smoking cessation is imperative.
*There is some evidence that suggests beta-blocker therapy in patients with a Marfan’s related thoracic aneurysm can slow the rate of growth, but this has not yet been extrapolated into otherwise healthy patients.
Surgical Management
Surgical management is dependent on the location of the aneurysm, with the threshold for surgery varying according to the health of the patient. Patients suffering from Marfan’s syndrome or having a previous thoracic dissection have a greater risk of dissection and rupture, and threshold levels for intervention are often lower
Surgical Intervention Criteria
Whilst cut off criteria will vary between countries and centres, a rough guide to thoracic aneurysm surgical interventions can be followed
- Ascending Aorta Treated when the diameter >5.5cm, the affected region of the aorta is excised and replaced with a dacron graft. If the aortic root is involved, a Bentall procedure is often performed, using a graft that also contains a prosthetic aortic valve
- Aortic Arch Once the aneurysm is over 5.5cm surgery should be considered; the affected aorta is replaced with a multi-limbed graft, allowing for the branching of the great vessels (these procedures have a high risk of cerebral ischaemia from embolisation)
- Descending Aorta Intervention is indicated when the diameter exceeds 6.0cm. These can be repaired open or with endovascular techniques, yet endovascular techniques have been shown to produce fewer postoperative complications and to have a lower mortality
Prognosis
Development of a second aneurysm is not uncommon in these patients post-intervention, so ongoing imaging studies as an outpatient are required following surgery, either with CT or MRI.
Recent studies demonstrate a perioperative mortality of between 2-17%, with arch aneurysms having the highest mortality, at 25% mortality. Mortality is significantly lower in those undergoing endovascular repair.
In patients who are monitored prior to surgery, the annual risk of rupture or dissection is 2% when <5cm, 3% for 5-5.9cm, and 7% for those 6cm or above.
Figure 3 – Contrast CT scan demonstrating a 7cm thoracic aortic aneurysm (black arrow), which has ruptured. The white arrow demonstrates blood in the thorax.
Key Points
- Most cases of thoracic aneurysm are associated with connective tissue disorders or bicuspid aortic valve
- Thoracic aneurysms are most often identified incidentally in routine imaging, however are best identified through CT imaging with contrast
- Management of thoracic aneurysm depends on location
- The annual risk of rupture or dissection is 2% when the aneurysm is <5cm, 3% for 5-5.9cm, and 7% for those >6cm