| Right-sided aortic arch | |
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| Anterior-posterior chest radiograph showing a right-sided aortic arch |
Right-sided aortic arch is a rare anatomical variant in which the aortic arch is on the right side rather than on the left. During normal embryonic development, the aortic arch is formed by the left fourth aortic arch and the left dorsal aorta. In people with a right-sided aortic arch, instead the right dorsal aorta persists and the distal left aorta disappears.[ citation needed ]
A right-sided aortic arch does not cause symptoms on itself, and the overwhelming majority of people with the right-sided arch have no other symptoms. However, when it is accompanied by other vascular abnormalities, it may form a vascular ring, causing symptoms due to compression of the trachea and/or esophagus. [1]
The causes of right-sided aortic arch are still unknown, 22q11 deletions have been found in some people with this condition. [2] It has also been found in association with other genetic syndromes such as Trisomy 21 (Down syndrome).[ citation needed ]
During pregnancy, prenatal ultrasound may reveal the abnormal course of the arch and this is the most common reason for identification of a right sided aortic arch nowadays. [3] Sometimes, when a right sided aortic arch is seen before birth, it can actually be a double aortic arch, sometimes a fetal MRI scan may be helpful if the ultrasound is not clear. [4]
After birth, a right-sided aortic arch is visualized on chest radiography, by the aortic knob (the prominent shadow of the aortic arch) that is located right from the sternum instead of left. Complex lesions are often assessed by MRI or CT.[ citation needed ]
Several types of right-sided aortic arch exist, the most common ones being right-sided aortic arch with aberrant left subclavian artery and the mirror-image type. The variant with aberrant left subclavian artery is associated with congenital heart disease in only a small minority of affected people. The mirror-image type of right aortic arch is very strongly associated with congenital heart disease, in most cases tetralogy of Fallot.[ citation needed ]
If a right aortic arch is associated with a left sided arterial ductal ligament (a remnant from the foetal circulation which forms a ligament after birth) then a vascular ring is formed around the trachea. Studies show that around 1:4 children show symptoms of a vascular ring. [5] This requires further investigation by specialists. Many children are well. There is evidence to show that symptoms of a vascular ring do not correlate with the appearance of the trachea in these patients so further assessment may be required. [6] This could be in the form of a specialist CT scan which is timed with inspiration and expiration or a free breathing bronchoscopy.[ citation needed ]
If required, repairing a vascular ring formed by a right sided aortic arch usually involves dividing the left sided arterial ductal ligament (this is not a structure that is necessary for the heart circulation as it is not a vessel after birth). This is usually performed by cardiothoracic surgeons from the side of the chest (thoracotomy incision) and does not require the heart to be stopped like many heart surgeries. Some people may have an aberrant left subclavian artery (the artery to the left arm) and this may also require re-implantation as it adds to the complexity of the vascular ring.[ citation needed ]
Right-sided aortic arch is rare, with a prevalence among adults of about 0.01%.[ citation needed ]
The aorta is the main and largest artery in the human body, originating from the left ventricle of the heart, branching upwards immediately after, and extending down to the abdomen, where it splits at the aortic bifurcation into two smaller arteries. The aorta distributes oxygenated blood to all parts of the body through the systemic circulation.
An artery is a blood vessel in humans and most other animals that takes oxygenated blood away from the heart in the systemic circulation to one or more parts of the body. Exceptions that carry deoxygenated blood are the pulmonary arteries in the pulmonary circulation that carry blood to the lungs for oxygenation, and the umbilical arteries in the fetal circulation that carry deoxygenated blood to the placenta. It consists of a multi-layered artery wall wrapped into a tube-shaped channel.
The brachiocephalic artery, brachiocephalic trunk, or innominate artery is an artery of the mediastinum that supplies blood to the right arm, head, and neck.
Aortic dissection (AD) occurs when an injury to the innermost layer of the aorta allows blood to flow between the layers of the aortic wall, forcing the layers apart. In most cases, this is associated with a sudden onset of severe chest or back pain, often described as "tearing" in character. Vomiting, sweating, and lightheadedness may also occur. Damage to other organs may result from the decreased blood supply, such as stroke, lower extremity ischemia, or mesenteric ischemia. Aortic dissection can quickly lead to death from insufficient blood flow to the heart or complete rupture of the aorta.
In human anatomy, the subclavian arteries are paired major arteries of the upper thorax, below the clavicle. They receive blood from the aortic arch. The left subclavian artery supplies blood to the left arm and the right subclavian artery supplies blood to the right arm, with some branches supplying the head and thorax. On the left side of the body, the subclavian comes directly off the aortic arch, while on the right side it arises from the relatively short brachiocephalic artery when it bifurcates into the subclavian and the right common carotid artery.
Vascular surgery is a surgical subspecialty in which vascular diseases involving the arteries, veins, or lymphatic vessels, are managed by medical therapy, minimally-invasive catheter procedures and surgical reconstruction. The specialty evolved from general and cardiovascular surgery where it refined the management of just the vessels, no longer treating the heart or other organs. Modern vascular surgery includes open surgery techniques, endovascular techniques and medical management of vascular diseases - unlike the parent specialities. The vascular surgeon is trained in the diagnosis and management of diseases affecting all parts of the vascular system excluding the coronaries and intracranial vasculature. Vascular surgeons also are called to assist other physicians to carry out surgery near vessels, or to salvage vascular injuries that include hemorrhage control, dissection, occlusion or simply for safe exposure of vascular structures.
Coarctation of the aorta (CoA) is a congenital condition whereby the aorta is narrow, usually in the area where the ductus arteriosus inserts. The word coarctation means "pressing or drawing together; narrowing". Coarctations are most common in the aortic arch. The arch may be small in babies with coarctations. Other heart defects may also occur when coarctation is present, typically occurring on the left side of the heart. When a patient has a coarctation, the left ventricle has to work harder. Since the aorta is narrowed, the left ventricle must generate a much higher pressure than normal in order to force enough blood through the aorta to deliver blood to the lower part of the body. If the narrowing is severe enough, the left ventricle may not be strong enough to push blood through the coarctation, thus resulting in a lack of blood to the lower half of the body. Physiologically its complete form is manifested as interrupted aortic arch.
In anatomy, the left and right common carotid arteries (carotids) are arteries that supply the head and neck with oxygenated blood; they divide in the neck to form the external and internal carotid arteries.
The aortic arch, arch of the aorta, or transverse aortic arch is the part of the aorta between the ascending and descending aorta. The arch travels backward, so that it ultimately runs to the left of the trachea.
The aortic arches or pharyngeal arch arteries are a series of six paired embryological vascular structures which give rise to the great arteries of the neck and head. They are ventral to the dorsal aorta and arise from the aortic sac.
Aberrant subclavian artery, or aberrant subclavian artery syndrome, is a rare anatomical variant of the origin of the right or left subclavian artery. This abnormality is the most common congenital vascular anomaly of the aortic arch, occurring in approximately 1% of individuals.
Ortner's syndrome is a rare cardiovocal syndrome and refers to recurrent laryngeal nerve palsy from cardiovascular disease. It was first described by Norbert Ortner (1865–1935), an Austrian physician, in 1897.
Subclavian steal syndrome (SSS), also called subclavian steal steno-occlusive disease, is a constellation of signs and symptoms that arise from retrograde (reversed) blood flow in the vertebral artery or the internal thoracic artery, due to a proximal stenosis (narrowing) and/or occlusion of the subclavian artery. This flow reversal is called the subclavian steal or subclavian steal phenomenon, regardless of signs/symptoms being present. The arm may be supplied by blood flowing in a retrograde direction down the vertebral artery at the expense of the vertebrobasilar circulation. It is more severe than typical vertebrobasilar insufficiency.
An arterial dissection is a tear within the wall of an artery, which allows blood to separate the wall layers. There are several types. Usually, a tear is in an arterial wall, but a vein wall tear has been documented.
Interrupted aortic arch is a very rare heart defect in which the aorta is not completely developed. There is a gap between the ascending and descending thoracic aorta. In a sense it is the complete form of a coarctation of the aorta. Almost all patients also have other cardiac anomalies, including a ventricular septal defect (VSD), aorto-pulmonary window, and truncus arteriosus. There are three types of interrupted aortic arch, with type B being the most common. Interrupted aortic arch is often associated with DiGeorge syndrome.
A vascular ring is a congenital defect in which there is an abnormal formation of the aorta and/or its surrounding blood vessels. The trachea and esophagus are completely encircled and sometimes compressed by a "ring" formed by these vessels, which can lead to breathing and digestive difficulties.
Abdominal ultrasonography is a form of medical ultrasonography to visualise abdominal anatomical structures. It uses transmission and reflection of ultrasound waves to visualise internal organs through the abdominal wall. For this reason, the procedure is also called a transabdominal ultrasound, in contrast to endoscopic ultrasound, the latter combining ultrasound with endoscopy through visualize internal structures from within hollow organs.
Traumatic aortic rupture, also called traumatic aortic disruption or transection, is a condition in which the aorta, the largest artery in the body, is torn or ruptured as a result of trauma to the body. The condition is frequently fatal due to the profuse bleeding that results from the rupture. Since the aorta branches directly from the heart to supply blood to the rest of the body, the pressure within it is very great, and blood may be pumped out of a tear in the blood vessel very rapidly. This can quickly result in shock and death. Thus traumatic aortic rupture is a common killer in automotive accidents and other traumas, with up to 18% of deaths that occur in automobile collisions being related to the injury. In fact, aortic disruption due to blunt chest trauma is the second leading cause of injury death behind traumatic brain injury.
Double aortic arch is a relatively rare congenital cardiovascular malformation. DAA is an anomaly of the aortic arch in which two aortic arches form a complete vascular ring that can compress the trachea and/or esophagus. Most commonly there is a larger (dominant) right arch behind and a smaller (hypoplastic) left aortic arch in front of the trachea/esophagus. The two arches join to form the descending aorta which is usually on the left side. In some cases the end of the smaller left aortic arch closes and the vascular tissue becomes a fibrous cord. Although in these cases a complete ring of two patent aortic arches is not present, the term ‘vascular ring’ is the accepted generic term even in these anomalies.
Dysphagia lusoria is an abnormal condition characterized by difficulty in swallowing caused by an aberrant right subclavian artery. It was discovered by David Bayford in 1761 and first reported in a paper by the same in 1787.