The acute aortic syndrome (AAS) includes the following disease entities:
- acute aortic dissection
- intramural hematoma (IMH) of the aorta
- penetrating aortic ulcer (PAU)
- covered aortic rupture
All these disease entities affect the aorta as an organ and have acute thoracic pain as a common leading symptom. The pain quality differs (in PAU often dull, in dissection cutting sharp) and the pain localization (rather back pain; if descending aorta affected, then in addition to thoracic pain also abdominal pain possible). Transitions between the four disease entities are also possible. For example, an initial PAU can lead to an IMH or an IMH can progress to an aortic dissection [1].
The concept of AAS is reflected in the classification of the European Task Force in the European Society of Cardiology (ESC) Guidelines 2014, whereby the classification also considers dissections after trauma and due to iatrogenic aortic lesions [2].
The term penetrating aortic ulcer (PAU) refers to the ulceration of an atherosclerotic plaque that penetrates the internal elastic lamina and often causes a wall hematoma in the surrounding media [3]. PAU is a disease of the severely atherosclerotic aorta [4].
In general, AAS occurs with a frequency of 2.6 to 3.5 cases/100,000 inhabitants per year [5, 6]. With 2–7 % of all AAS, PAU is relatively rare [2]. PAU is mainly localized in the ascending aorta and typically occurs in older patients with arterial hypertension, hyperlipoproteinemia, and aortic sclerosis [7]. It can lead to complications such as aneurysm or pseudoaneurysm formation, aortic dissection, or aortic rupture.
Symptomatic penetrating aortic ulcers have a poor prognosis with a rupture rate of up to 40 % [3]. Urgent surgical or endovascular therapy is therefore generally recommended. There is hardly any data on the natural course of asymptomatic PAU patients. Progression with pseudoaneurysm formation is assumed in 30–50 % of cases. The indication for invasive treatment of asymptomatic PAU remains unclear at present. In general, there is a risk of rupture that depends on the diameter of the ulcer. For a width > 2 cm and a depth of >1 cm, interventional or surgical therapy should be considered according to the literature [3, 8, 9, 10].
Based on previous experience, elective endovascular treatment is recommended for a diameter or depth of the ulcer of ≥ 20 mm. In case of signs of impending rupture (pain, extra-aortic blood), endovascular treatment is urgent. Pain is mentioned as one of the main criteria for urgent surgical intervention [3, 11].
The essential imaging information for PAU is provided by angio-CT, MR angiography, and with limitations by intra-arterial DSA [12, 13]. CT using multi-detector technology (MDCT) depicts calcifications in the area of a PAU better than MR angiography [13]. Regardless, computed tomography is available more promptly than MRI in most clinics.
Although no randomized data are available, given the comorbidity of many patients, endovascular treatment is preferred over open surgical repair whenever possible. Successful endoluminal treatment of PAU has been described by several groups [14-17].