Pdf diagnostic role of magnetic resonance imaging in. It is formed by regression of a segment of the embryological left aortic arch and persistence of the right fourth arch or right dorsal aorta. In the present series of 30 patients with laevocardia and complete or partial inversion of the viscera, 40 per cent had a right aortic arch. Abstractanomalies of the aortic arch represent clinically the most important group of congenital lesions in the heart and great vessels.
In livocardia with atrial inversion, the aortic arch is usuallyrightsidedshaherandjohnson,1963. The remaining part of the right dorsal aorta distal to the right 4th primitive arch is. After birth, the distal part of the left sixth aortic arch, which forms the duc tus arteriosus, normally constricts to form the ligamentum arteriosum also known as the. The type of raa reported 1 is generally held to result from regression of segment c of the embryonic left fourth arch 2. These may include aortic arch or arches, arch vessels, pul monary artery branch or branches any of which may be patent or atretic, and ductus. Imaging of aortic arch anomalies and associated findings. Aortic arch anomalies are present in 1% to 2% of the general population and are commonly associated with congenital heart disease, chromosomal defects, and tracheaesophageal compression in postnatal life. The aortic arch and its branches form during the third week of embryogenesis, which involves a complex process.
Variant anatomy of the aortic arch occurs when there is failure of normal aortic development. Pdf magnetic resonance imaging has become the gold standard for imaging the aortic arch with the ability to demonstrate not only the arterial. The purpose of this study was to determine the relative frequency of associated cardiovascular anomalies in individuals with this anomaly. Operation for aortic arch anomalies the annals of thoracic surgery. Aortic arch sidedness refers to which bronchus is crossed by the arch fig 3. Anatomic variations of the aortic arch and its vessels are common in the general population. The coarctation, therefore, is a manifestation of abnormal interruption of this arch system at different locations, if a longer segment is involved, hypoplastic. Magnetic resonance imaging has become the gold standard for imaging the aortic arch with the ability to demonstrate not only the arterial branching pattern, but also the relationship of aorta and its branches to the trachea and bronchi. Imaging is therefore required to elucidate the complex aortic arch anomalies, abnormal branching pattern, their relationship with trachea and esophagus, and. Abnormalities of the arch branching pattern arise by persistence of segments of.
Vascular rings represent 12% of congenital cardiovascular anomalies. Anomalous origin of the right subclavian artery arsa from the aorta distal to the normally positioned left subclavian artery is a relatively frequent congenital anomaly in subjects with left aortic arch. Aortic arch anomalies persistent fifth aortic arch. Aortic arch anomalies coarctation of the aorta interrupted aortic arch echocardiography v. The left dorsal aorta forms the distal aortic arch and the descending thoracic aorta 17,18. Aortic arch anomalies refer to a variety of congenital abnormalities of the position or branching pattern, or both, of the aortic arch table 1 14. Arch anomalies can be associated with symptoms, such as dysphagia lusoria in the setting of left aortic arch with aberrant right subclavian artery. Hunault in 1735,cited by moes wrote pathologic description of anomalous right subclavian artery. Aortic arch abnormalities associated with other congenital. Total aortic arch replacement in patients with arch vessel. A congenital anomaly of the aortic arch is usually an incidental radiological finding in asympto matic patients, except when the anomaly constitutes a vascular ring. Congenital heart abnormalities can obstruct blood flow or redirect the normal flow of blood through andor out of the heart. Congenital aortic arch anomalies result from errors in involution or migration of primitive arches, usually aberrations occur at the level of interruption of the primitive arches 2.
Congenital aortic arch anomalies canadian journal of cardiology. Aortic arch anomalies article pdf available in journal of cardiovascular magnetic resonance 84. Interrupted aortic arch iaa refers to a rare but very serious congenital heart defect in which there is a gap between the ascending and descending segments of the aorta, namely, in the arch of. Atresia of the left arch occurs in approximately 1025% of double aortic arches and we speculate that this particular anatomical variant would have an appearance similar to that of interrupted aortic arch on sagittal imaging but similar to that of simple right arch on transverse imaging. What is the aortic arch and what abnormalities are seen in children with phace syndrome. Interruption of the aortic arch childrens hospital of. A congenital anomaly of the aortic arch is usually an incidental radiological finding in asymptomatic patients, except when the anomaly constitutes a vascular ring that is formed when the abnormally patterned arch vessels completely encircle the trachea and esophagus. Important associations with other cardiac defects will be acknowledged. Aberrant right subclavian artery with left aortic arch. The trachea normally deviates slightly to the side opposite the arch. Abnormal regression of right 4th aortic arch asymptomatic, usually incidental detection on imaging aortic arch anomalies 16. The aim of this article was to assess the role of mri in the diagnosis and management of a variety of complex aortic arch anomalies. Aortic arch definition of aortic arch by the free dictionary.
In this chapter, aortic arch anomalies will be described as they occur in isolation. The fifth aortic arch extends from the vas and inserts into segment c. Because the right ductus arteriosus almost always disappears, this arch anomaly does not developmental aortic arch anomalies in infants and children. Congenital aortic arch anomalies comprise a rare spectrum of cardiovascular diseases. Congenital variants and anomalies of the aortic arch. Developmental aortic arch anomalies in infants and children. It results in a number of heterogenous anomalies of the aorta and its branch vessels.
Based on edwards diagram, four possible areas of involution can be. Fetal sonographic diagnosis of aortic arch anomalies. While certain patterns of aortic arch anomalies are simple positional abnormalities, there are other patterns that form a complete or incomplete vascular ring around the trachea and esophagus. Variant anatomy of the aortic arch radiology reference. Association of chromosome 22q11 deletion with isolated. Developmental aortic arch anomalies in infants and children assessed with ct angiography luis ramosduran 1, john w.
Individuals with both a cardiac defect and an aortic arch anomaly right aortic arch, cervical location, or abnormal branching pattern are more likely to have a. Even among the isolated anomalies of the aortic arch that are included in this study, 10 distinct patterns were found in patients with a 22q11 deletion. The curved portion of the aorta between the ascending and descending portions that gives rise to the brachiocephalic trunk, the left carotid artery, and the left subclavian artery. A nomalies of the aortic arch represent a group of lesions that may occur in isolation or in conjunction with other cardiac defects. Congenital anomalies of the aortic arch include leftsided, rightsided, and double aortic arches, with various branching patterns of the great vessels.
Imaging is therefore required to elucidate the complex aortic arch anomalies, abnormal branching pattern, their relationship with trachea and esophagus, and other associated cardiac and noncardiac pathologies. Aortic arch anomalies include a heterogeneous group of diagnosis, including native disease of the aortic arch alone or in association with intracardiac disease and anomalies secondary to surgical. Although anomalies of the aortic arch and its branches are relatively uncommon malformations, they are often associated with congenital heart disease. Aortic arch anomalies refer to congenital abnormalities of the position or. Left aortic arch with aberrant right subclavian artery is the second most common aortic arch anomaly and is present in 0. Gross anatomy normally, the aorta ascends in the superior medias. A congenital anomaly of the aortic arch is usually an incidental radiological finding in asymptomatic patients, except when the anomaly constitutes a vascular ring that is formed when the abnormally patterned arch vessels completely encircle the trachea and esophagus 1. The various anomalies in aortic arch can be explained. The predictors of left ventricular outflow tract obstruction lvoto after the repair of coarctation of the aorta or interruption of the aortic arch coaiaa with ventricular septal defect have been investigated. Isolated lesions may be clinically significant when the airways are compromised by a vascular ring.
Aortic arch anomalies may be classified with the edwards hypothetical double arch, with stratification based upon the point at which the hypothetical arch has been interrupted. Joseph schoepf 1 2, thomas henzler 1 3, paul apfaltrer 1 3 and anthony m. These defects can involve the walls of the heart, the valves of the heart, and the large arteries and veins near the heart. Anatomically, aortic arch anomalies consist of abnormal sidedness or position of the aortic arch which concerns the course of the aortic arch itself and abnormal branching of the arch vessels which concerns the order and pattern of origin of the major branches from the aortic arch. A wide spectrum of congenital anomalies can occur during the formation of the aortic arch, brachiocephalic arteries and ductus arteriosus, most. These include double aortic arch, right aortic arch with left ligamentum arteriosum, aberrant right subcla vian artery, anomalous left subclavian artery, and. Arch anomalies can be associated with symptoms, such as dysphagia lu soria in the setting of left aortic arch with aberrant right subclavian artery.
Developmental aortic arch anomalies in infants and. In a child with interruption of the aortic arch, there is a disconnection a break between the top part of the aortic arch and the lower, descending aorta. The aorta starts at the left ventricle lower chamber of the heart as one large vessel and branches into smaller. Evaluation with the use of multidetector computed tomography congenital anomalies of the aortic arch have clinical importance, as the anomalies may be associated with vascular rings or other congenital cardiovascular diseases. Evaluation of aortic arch anomalies by echocardiography.
Congenital variants and anomalies of the aortic arch are important to recognize as they may be associated with vascular rings, congenital heart disease, and chromosomal abnormalities, and can have important implications for prognosis and management. Aortic arch anomaly an overview sciencedirect topics. The sonographically based detection of aortic arch anomalies lies in the 3. There are many different variations of aortic arch anomalies. Congenital variants and anomalies of the aortic arch rsna. Pulmonary stenosis, atrial septal defects, heterotaxy syndrome, and hypoplastic left heart syndrome have also been reported.
The patent vascular structures surrounding the trachea and esophagus include. Aortic arch anomalies can occur in isolation, in association with congenital heart defects, or in genetic syn dromes eg, 22q11. These anomalies may be isolated or may be associated with other congenital heart diseases. The cardiac phenotype ranges from complex intracardiac and aortic arch anomalies to completely normal anatomy. Double aortic arch occurs due to persistence of the right and left dorsal aortas. Aortic arch anomalies can be classified according to the edwards hypothetical double arch system, although many modifications of this classification system have been proposed. Double aortic arch hunault 1735 first report of clinical syndrome of vascular compressionwolman 1939 kommerel in 1936 xray findings of anamolous right sca first division of a vascular ring gross in 1945 first successful repair of interrupted. We encourage the reader to use the threedimensional models which accompany this chapter to understand the normal development of the great arteries. Multidetector computed tomography mdct angiography enables one. Congenital anomalies of the aortic arch include diverse subgroups of malformations that may be clinically silent or may present with severe respiratory or esophageal symptoms especially when associated with complete vascular rings. The most common aortic arch anomaly is bovine arch, in which all four vessels arise from a common trunk. The aorta arises from the left ventricle and carries oxygenated blood from the heart to the body. Congenital anomalies of the aortic arch are uncommon and may be associated with other congenital cardiovascular diseases.
Left ventricular outflow tract obstruction in aortic arch. Coarctation of the aorta occurs in 5 7% of chd 7th form. Between 1995 and 2005, 90 consecutive patients with interrupted aortic arch n 25 or coarctation of the aorta n 65 and cardiac anomalies underwent biventricular repair. Congenital variants and anomalies of the aortic arch are important to recognize as they may be associated with vascular rings, congenital heart disease chd, and chromosomal abnormalities, and can have important implications for prognosis and management, including surgical and percutaneous interventions. Clinically, classification is most likely to proceed on the basis of anatomy and morphology, where the course of the aorta and arch vessels form the basis for. Aortic arch anomalies are a type of congenital heart condition, which means it is a disease or abnormality that is present from birth.
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