I. Congenital aortic disease
Disruption of the aortic arch
The aortic arch includes three segments: proximal arch, distal arch and aortic isthmus. An anatomic discontinuity or complete lumen inaccessibility between any two segments is diagnosed as interruption of the aortic arch. Autopsy shows that it accounts for 1% to 4% of precardiac disease, while clinical data indicate that it accounts for 1.3% of severe pediatric precardiac disease, with a prevalence of approximately 0.003/1000 births.
There are 3 types of aortic arch interruption: ① type A, the aortic arch interruption is located in the isthmus, accounting for about 40%; ② type B, the aortic arch interruption is located between the left subclavian artery and the left common carotid artery, accounting for about 55%; ③ type C: the aortic arch interruption is located between the left common carotid artery and the unnamed artery, accounting for about 5%. Hong Hao, Department of Cardiac Surgery, Wuhan Union Hospital
Aortic constriction
Aortic constriction refers to congenital stenosis occurring in the aortic isthmus, and occasionally between the left common carotid artery and the left subclavian artery, or in the thoracic or abdominal aorta. It is a relatively common congenital aortic malformation, accounting for approximately 6.5% of congenital heart disease (CHD).
Aortic constriction has been classified into simple and complex types based on whether it is combined with other cardiovascular malformations (other than ductus arteriosus).
The Bonnet typology of 1903 is now commonly used in clinical practice (although with significant limitations): (1) the anterior ductal type (infantile), in which the constriction is located between the ductus arteriosus and the left subclavian artery; and (2) the posterior ductal type (adult), in which the constriction is located distal to the ductus arteriosus or the attachment of the arterial ligament.
Congenital vascular ring
Congenital vascular rings refer to complete or partial encirclement and compression of the esophagus and/or trachea by the aortic arch and its branch vessels, and mainly include complete and incomplete vascular rings. The disease accounts for 1% to 2% of the composition of congenital heart disease.
Vascular suspensory band
A vascular suspensory band refers to a left pulmonary artery that starts outside the pericardium from the right pulmonary artery, bifurcates left posteriorly through the trachea, and enters the left hilum anterior to the esophagus, forming a suspensory band around the trachea. The disease is rare, with the largest reported case being only 15 (accumulated over 45 years).
Supra-aortic stenosis
Supra-aortic stenosis refers to limited or diffuse stenosis of the aorta occurring over the aortic valve and has three main clinical manifestations: as one of the phenotypes of Williams syndrome (about half of cases), disseminated, or familial.
Most supra-aortic stenoses are confined stenoses, caused by an annular protrusion of the inner wall of the aorta above the aortic valve junctional stop into the aortic lumen, often with an hourglass appearance. Diffuse supra-aortic stenosis usually presents with abnormal thickening of the aortic intima and wall, which may extend to the origin of the innominate artery.
The disease is a complex lesion of the aortic root and the aortic sinus proximal to the stenosis may be dysplastic. The coronary opening may be obstructed not only by thickened aortic tissue but also by the aortic valve. aortic valve thickening is present in 30% of patients with supra-aortic stenosis, but aortic valve stenosis due to leaflet fusion is rare.
II. Acquired aortic disease
Aortic aneurysm
The English word for aneurysm (aneurysm) is of Greek origin and originally meant dilatation. A true aortic aneurysm is an aortic lesion that is more than 1.5 times the diameter of the normal aorta at the site of the lesion, with all layers of the structure intact. A pseudoaneurysm is a limited hematoma around the aorta caused by infection, trauma, surgery, or rupture of an ulcer. The difference between the two is that the arterial wall structure of a pseudoaneurysm is incomplete. Epidemiological data show that the incidence of aortic aneurysms (mainly descending aortic aneurysms) is 10.4 per 100,000 person-years.
Depending on the site of occurrence, aortic aneurysms are classified as ascending aortic aneurysms, aortic arch aneurysms, and thoracic and abdominal aortic aneurysms (descending aortic aneurysms).
Crawford (Crawford) typed thoracic and abdominal aortic aneurysms into 4 types: (i) type I, thoracic and/or partial abdominal aortic involvement; (ii) type II, total descending aortic involvement (including all thoracic and abdominal aorta, which may involve the iliac artery); (iii) type III, distal thoracic and abdominal aortic involvement; and (iv) type IV, abdominal aortic involvement.
Aortic coarctation
Aortic coarctation refers to the entry of blood in the aortic lumen through an endothelial breach into the middle layer of the aortic wall and tearing the middle layer of the aortic wall to form a false lumen. However, aortic coarctation can also occur in the absence of an endothelial rupture. The nomenclature of aortic coarctation is more confusing, such as aortic coarctation, aortic coarctation formation, aortic dissection (separation) and coarctation aneurysm. In recent years, foreign scholars have increasingly applied the term aorticdissection, which has also been recognized by many domestic scholars. Therefore, it is suggested that the term “aortic dissection” be used uniformly in China to facilitate standardization and communication.
Aortic interstitial hematoma refers to the overflow of blood from the arterial lumen into the aortic wall without rupture of the outer membrane of the artery.
Depending on the time of onset, aortic coarctation is divided into two categories: acute (<14 days) and chronic (>14 days).
There are two internationally accepted staging methods for aortic coarctation. DeBakey typing: ① DeBakey type I, aortic coarctation starts from the ascending aorta (or aortic root) and involves most or all of the aorta; ② DeBakey type II, aortic coarctation involves only the ascending aorta; ③ DeBakey type III, aortic coarctation involves only the descending aorta (Ⅲa, involving only the thoracic descending aorta; Ⅲb, involving the thoracic and abdominal aorta ).
Stanford typing, proposed by Daily and others in the 1970s, is divided into 2 types: ①Stanford type A, in which the aortic coarctation involves the ascending aorta (including the aortic root); ②Stanford type B, in which the aortic coarctation involves the descending aorta.
There is also a less commonly used Kirklin typing method, which is not described here. The author has unique insights into the typing of aortic coarctation, which will be described in detail in a later series.
Other Acquired Aortic Diseases
Non-infectious aortic diseases
Multiple aortitis (Takayasu arteritis), leukoaraiosis, cutaneous mucosal lymph node syndrome (Kawasaki disease), and giant cell arteritis may develop into aneurysms, especially multiple aortitis, which more often involves the aortic arch and cephalic-arm trunk and often requires surgical intervention. Other immune diseases, such as ankylosing spondylitis, Wright syndrome, and nodular polyarteritis, may also develop aortic root aneurysms and aortic valve closure insufficiency, requiring surgical treatment.
Aortic trauma
Among aortic trauma, acute traumatic aortic dissection is more common and is defined as a total or partial dissection of the aortic wall that occurs within 14 days of injury. Chronic aortic trauma may result in aortic aneurysm.