Right-sided aortic arch

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Right-sided aortic arch
Rechts descendierende Aorta - Roe pa.jpg
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 ]

Contents

Symptoms and signs

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]

Pathophysiology

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 ]

Diagnosis

Chest radiograph showing a right-sided aortic arch, lateral view Rechts descendierende Aorta - Roe seitlich.jpg
Chest radiograph showing a right-sided aortic arch, lateral view

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 ]

Classification

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 ]

Management

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 ]

Epidemiology

Right-sided aortic arch is rare, with a prevalence among adults of about 0.01%.[ citation needed ]

See also

Related Research Articles

Aorta Largest artery in the body

The aorta is the main and largest artery in the human body, originating from the left ventricle of the heart and extending down to the abdomen, where it splits into two smaller arteries. The aorta distributes oxygenated blood to all parts of the body through the systemic circulation.

Artery Blood vessels that carry blood away from the heart

An artery is a blood vessel in humans and most other animals that takes blood away from the heart to one or more parts of the body. Most arteries carry oxygenated blood; the two exceptions are the pulmonary and the umbilical arteries, which carry deoxygenated blood to the organs that oxygenate it. The effective arterial blood volume is that extracellular fluid which fills the arterial system.

In medicine, a pulse represents the tactile arterial palpation of the cardiac cycle (heartbeat) by trained fingertips. The pulse may be palpated in any place that allows an artery to be compressed near the surface of the body, such as at the neck, wrist, at the groin, behind the knee, near the ankle joint, and on foot. Pulse is equivalent to measuring the heart rate. The heart rate can also be measured by listening to the heart beat by auscultation, traditionally using a stethoscope and counting it for a minute. The radial pulse is commonly measured using three fingers. This has a reason: the finger closest to the heart is used to occlude the pulse pressure, the middle finger is used get a crude estimate of the blood pressure, and the finger most distal to the heart is used to nullify the effect of the ulnar pulse as the two arteries are connected via the palmar arches. The study of the pulse is known as sphygmology.

Brachiocephalic artery

The brachiocephalic artery is an artery of the mediastinum that supplies blood to the right arm and the head and neck.

Aortic dissection Injury to the innermost layer of the aorta

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. Also, vomiting, sweating, and lightheadedness may occur. Other symptoms may result from decreased blood supply to other organs, 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.

Subclavian artery Major arteries of the upper thorax, below the clavicle

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.

dextro-Transposition of the great arteries Medical condition

dextro-Transposition of the great arteries, is a potentially life-threatening birth defect in the large arteries of the heart. The primary arteries are transposed.

Aortic aneurysm Notable enlargement of the aorta (heart artery)

An aortic aneurysm is an enlargement (dilatation) of the aorta to greater than 1.5 times normal size. They usually cause no symptoms except when ruptured. Occasionally, there may be abdominal, back, or leg pain. The prevalence of AAA has been reported to range from 2 to 12% and is found in about 8% of men more than 65 years of age. The mortality rate attributable to AAA is about 15,000 per year in the United States and 6,000 to 8,000 per year in the United Kingdom and Ireland. Between 2001 and 2006, there were approximately 230,000 AAA surgical repairs performed on Medicare patients in the United States.

Coarctation of the aorta Medical condition

Coarctation of the aorta, also called aortic narrowing, 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.

Descending thoracic aorta

The descending thoracic aorta is a part of the aorta located in the thorax. It is the third and last part of the thoracic aorta and is a continuation of the aortic arch. It is located within the posterior mediastinal cavity, but frequently bulges into the left pleural cavity. The descending thoracic aorta begins at the lower border of the fourth thoracic vertebra and ends in front of the lower border of the twelfth thoracic vertebra, at the aortic hiatus in the diaphragm where it becomes the abdominal aorta.

Aortic arch

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.

Aortic arches

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 Medical condition

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.

Ortners syndrome Medical condition

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 Medical condition

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.

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

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.

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 Medical condition

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.

References

  1. Białowas J, Hreczecha J, Grzybiak M (2000). "Right-sided aortic arch". Folia Morphol. (Warsz). 59 (3): 211–6. PMID   10974792.
  2. Momma, K.; Matsuoka, R.; Takao, A. (2014). "Aortic Arch Anomalies Associated with Chromosome 22q11 Deletion (CATCH 22)". Pediatric Cardiology. 20 (2): 97–102. doi:10.1007/s002469900414. ISSN   0172-0643. PMID   9986884. S2CID   813344.
  3. Zidere V, Tsapakis EG, Huggon IC, Allan LD (December 2006). "Right aortic arch in the fetus". Ultrasound Obstet Gynecol. 28 (7): 876–81. doi:10.1002/uog.3841. PMID   17066500. S2CID   25566821.
  4. "Spotlight on research | News Centre | King's College London".
  5. Fetuses with right aortic arch: a multicenter cohort study and meta-analysis. D'Antonio F, Khalil A, Zidere V, Carvalho JS. Ultrasound Obstet Gynecol. 2016 Apr;47(4):423-32. doi: 10.1002/uog.15805. Epub 2016 Mar 16. Review.
  6. Correlation of Symptoms with Bronchoscopic Findings in Children with a Prenatal Diagnosis of a Right Aortic Arch and Left Arterial Duct. Vigneswaran TV, Kapravelou E, Bell AJ, Nyman A, Pushparajah K, Simpson JM, Durward A, Zidere V. Pediatr Cardiol. 2018 Apr;39(4):665-673. doi: 10.1007/s00246-017-1804-5. Epub 2018 Jan 6.