
What are aortic syndromes?
Aortic syndromes are:
- Aortic dissection
- Penetrating aortic ulcer
- Intramural hematoma
Aortic dissection
Acute aortic dissection is the most devastating aortic pathology, more frequent than the ruptured aneurysm. Without treatment most patients die.
During the dissection, a tear is created in the inner layer of the aorta, causing blood to separate the inner and outer layers, creating instability of the wall. This instability can lead to rupture of the aorta or reduced blood supply to the various organs (brain, intestines), upper and lower extremities.
Type A dissection starts at the ascending aorta while type B dissection starts at the descending thoracic aorta. Acute dissection is a life-threatening emergency that must be treated promptly.
Most patients are men aged 60-70, however women and younger patients may develop dissection.
Risk factors
The most common risk factor is hypertension in 72% of the patients.
Other risk factors are:
- Hereditary genetic syndromes (Marfan syndrome, Ehlers-Danlos syndrome)
- Bicuspid aortic valve
- Coarctation
- Chromosomal abnormalities (Turner syndrome, Noonan syndrome)
- Preeclampsis
- Trauma
Marfan syndrome is a connective tissue disease and affects the blood vessels, bones, heart, lungs and is the cause of 50% of dissection cases in young patients under 40 years of age.
Symptoms
The main symptom is pain that can be located in the chest, back, lower back or abdomen. Most patients present with hypertension. They may also have symptoms of stroke, mesenteric ischemia (reduced blood supply to the intestine), upper or lower extremity ischemia with loss of peripheral pulses and pain or numbness of the limbs.
Diagnosis and Treatment
The diagnosis is made by the physical examination, the history, and then CT angiography is usually performed.
The first line treatment is immediate control of hypertension. Depending on the location of the dissection (type A or B) surgery is decided accordingly. In type A disection type A, it starts in the ascending aorta, surgery is performed by the cardiac surgery team.
In type B dissections the patient is admitted to an intensive care unit for continuous monitoring of vital signs. Depending on the symptoms, it is decided whether immediate surgery is needed or not. In case of symptoms of reduced blood supply to the intestines (abdominal pain that does not improve with conservative treatment) or to the lower extremities or some worrisome findings on CT angiography, urgent surgery is recommended.
Most often, surgical repair involves placement of an endovascular stent (TEVAR) which is performed by puncturing the femoral arteries in the groins. Depending on the other findings, it may be decided to create a bypass or place a stent for the reperfusion of the intestines or the lower extremities.
If the symptoms subside only with conservative management, the patient is monitored and after the appropriate control of the pressure is discharged from the hospital. The patient is then closely monitored and has a repeat CT angiogram to decide the next step of treatment.
It is important to note that 25-50% of patients with dissection will develop an aortic aneurysm. New studies have shown that surgical repair with coverage of the dissection with an endograft (TEVAR) in certain groups of patients increases survival and protects against complications, such as aneurysm rupture. The decision on the time of surgery is discussed with the patient.
Aortic intramural hematoma
The development of hematoma (blood collection) in the wall of the aorta is also an emergency in which the patient may experience severe pain in the chest, back or abdomen. Intramural hematoma is caused by the collection of blood within the layers of the aortic wall usually after rupture of the small blood vessels.
As in the dissection, depending on the location in the aorta, it is divided into type A and type B. Similar treatment to the treatment of the dissection is also applied to the intramural hematoma depending on its symptoms and location.
Penetrating aortic ulcer
Penetrating aortic ulcer occurs in areas of the aorta with atherosclerotic plaques, where the plaque “breaks” and causes a discontinuity in the aortic wall and its layers.
The majority of ulcers occur in the descending thoracic aorta more often in men aged 70-80 years.
Symptoms may include:
- sharp pain in the chest, back, abdomen (if the ulcer is located in the abdominal aorta)
- Hemoptysis or hematemesis may occur if the ulcer ruptures into the bronchi or esophagus.
In some patients it may be an incidental finding on imaging.
Diagnosis is usually made by computed tomography/angiography or magnetic angiography. A transesophageal ultrasound may also be performed in which cardiac function can be assessed at the same time.
Treatment is mainly determined by the symptoms, size and characteristics of the ulcer. Blood pressure control is important and is the first step.
The treatment is discussed in detail with the Vascular Surgeon. If surgical treatment is decided, it most often involves placement of an endovascular graft by puncturing the femoral arteries in the groin areas and using special catheters and wires.
The operation is discussed in detail with the patient, and the possible complications as well.
