Materiały źródłowe

• #1 Vascular Ring Symptoms, Doctors, Treatments, Advances & More | MediFind

  https://www.medifind.com/conditions/vascular-ring/6261

  Vascular ring is an abnormal formation of the aorta, the large artery that carries blood from the heart to the rest of the body. It is a congenital problem, which means it is present at birth. […] Vascular ring is rare. It accounts for less than 1% of all congenital heart problems. The condition occurs as often in males as females. Some infants with vascular ring also have another congenital heart problem. […] Vascular ring occurs very early during the baby’s development in the womb. Normally, the aorta develops from one of several curved pieces of tissue (arches). The body breaks down some of the remaining arches, while others form into arteries. Some arteries that should break down do not, which forms a vascular ring. […] With vascular ring, some of the arches and vessels that should have changed into arteries or disappeared are still present when the baby is born. These arches form a ring of blood vessels, which encircles and presses down on the windpipe (trachea) and esophagus. […] Several different types of vascular ring exist. In some types, the vascular ring only partially encircles the trachea and esophagus, but it still can cause symptoms.

• #1 HIE Multimedia – Vascular ring

  https://adamcertificationdemo.adam.com/content.aspx?productid=117&pid=1&gid=007318

  Vascular ring is an abnormal formation of the aorta, the large artery that carries blood from the heart to the rest of the body. It is a congenital problem, which means it is present at birth. […] Vascular ring is rare. It accounts for less than 1% of all congenital heart problems. The condition occurs as often in males as females. Some infants with vascular ring also have another congenital heart problem. […] Vascular ring occurs very early during the baby’s development in the womb. Normally, the aorta develops from one of several curved pieces of tissue (arches). The body breaks down some of the remaining arches, while others form into arteries. Some arteries that should break down do not, which forms a vascular ring. […] With vascular ring, some of the arches and vessels that should have changed into arteries or disappeared are still present when the baby is born. These arches form a ring of blood vessels, which encircles and presses down on the windpipe (trachea) and esophagus.

• #1 Vascular rings and slings | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/vascular-rings-and-slings?embed_domain=hackmd.io%2F%40yipuafecsl2jsu8smr5njq%2Fbnjhjgjghjghjgh&lang=us

  Vascular rings and slings refer to the congenital vascular encirclement of the esophagus and/or trachea by anomalous/aberrant vessels. […] Causes of vascular rings and slings commonly include: double aortic arch (the most common symptomatic vascular ring), incomplete double aortic arch, right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum, aberrant right subclavian artery (most common), and pulmonary sling.

• #1 Vascular Rings: Practice Essentials, Anatomy, Pathophysiology

  https://emedicine.medscape.com/article/426233-overview

  The various forms of this anomaly occur very early in embryologic development. They result from the abnormal or incomplete regression of one of the six embryonic branchial arches. Several papers have reported the close association of band 22q11 deletion with anomalies of the aortic arch, as well as other congenital cardiac abnormalities. […] In normal development, each primitive arch either progresses into a functional vascular structure or involutes as follows: […] Abnormal arch development (see Anatomy) results when one or more of the necessary involutions or other changes in embryonic arch structures does not occur. […] The classic double aortic arch develops when involution of the distal right fourth arch does not take place. The fourth right and left arches both persist and join the left-side descending thoracic aorta. The right and left aortic arches encircle the trachea and the esophagus.

• #1 Vascular Rings: Practice Essentials, Anatomy, Pathophysiology

  https://emedicine.medscape.com/article/426233-overview

  When the left fourth branchial arch involutes and the right remains, a right aortic arch is present. Right aortic arch occurs less frequently than 1 in 100,000 times in the general population and may exist in the absence of any other anomalies. Its presence is suggestive of the existence of an associated anomaly. […] Persistence of the right arch with involution of the left creates a situation in which the origins of the left subclavian artery and ductus arteriosus can vary. Several of these configurations can produce a vascular ring. […] In these cases, only partial resorption of the distal left fourth arch occurs. The first brachiocephalic vessel originating from the right arch is the left innominate artery, which, in turn, branches into a left carotid and a left subclavian artery. […] Two extremely rare complete rings occur in the presence of a left aortic arch, and both are associated with a right-side descending thoracic aorta.

• #1 Vascular Rings: Practice Essentials, Anatomy, Pathophysiology

  https://emedicine.medscape.com/article/426233-overview

  Anomalous innominate artery is a rare cause of tracheomalacia or stenosis. It is exclusively seen in the pediatric population. This condition occurs as a consequence of the abnormal takeoff of the innominate artery, which results in anterior compression of the trachea. […] This abnormality occurs when the left main pulmonary artery arises as a branch of the right pulmonary artery instead of originating from the main pulmonary artery. This is believed to be an abnormality related to sixth aortic arch development.

• #1 Vascular rings – Overview – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/vascular-rings/cdc-20389579

  A vascular ring occurs when a part of the body’s main artery, called the aortic arch, or its branches form a ring around the trachea or esophagus. […] A vascular ring is a heart condition present at birth. That means it’s a congenital heart defect. In this condition, part of the body’s main artery or its branches form a complete ring around the windpipe and the food swallowing tube. […] The exact cause of vascular rings is not known. Congenital heart conditions happen as a baby, also called a fetus, is growing in the womb during pregnancy. […] Some things that may play a role in the development of congenital heart conditions are: Gene changes. Some medicines. Some health conditions. Lifestyle choices, including smoking. Things in your environment. […] What increases the risk of vascular rings is not well known. Possible risk factors for vascular rings and other congenital heart conditions are: Genetics. Changes in some genes have been linked to congenital heart conditions. For example, people with Down syndrome often are born with heart conditions.

• #1 Vascular Ring Double Aortic Arch – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK558938/

  DAA’s exact cause is unknown (nor for any other VR). There have been associations of RAA and DAA with 22q11 deletion in the literature. There is some association of DAA with trisomy 21 and trisomy 18. A left aortic arch with aberrant (retro-esophageal) right subclavian artery is not a ring but is highly associated with trisomy 21. […] The vascular ring is formed when there is a failure of the regression or persistence of some part of the aortic arch. A double aortic arch is formed when the fourth aortic arches persist.

• #1 What Is A Double Aortic Arch?

  https://my.clevelandclinic.org/health/diseases/23432-double-aortic-arch

  A double aortic arch is one type of vascular ring, a group of congenital heart defects that affect 1 in 10,000 babies. […] The double aortic arch belongs to a group of congenital heart defects called vascular rings. […] The exact cause of a double aortic arch isn’t known. Its more common in babies conceived by in-vitro fertilization, but we don’t know why. […] A double aortic arch is linked with other medical conditions. Up to 12% of babies with a double aortic arch have another congenital heart defect. These include ventricular septal defect (VSD) and tetralogy of Fallot. Up to 20% of babies with a double aortic arch can have a genetic disorder such as chromosome 22q11 deletion and trisomy 21.

• #1 Case Based Pediatrics Chapter

  http://hawaii.edu/medicine/pediatrics/pedtext/s07c07.html

  Vascular rings encompass only 1% to 3% of all congenital heart disease. Some vascular rings are associated with other congenital heart lesions while others are isolated defects. Tracheobronchial anomalies are rarely seen with vascular rings but are common in pulmonary artery slings. […] A pulmonary sling is the left pulmonary artery arising from the right pulmonary artery. It is also known as an anomalous pulmonary artery and results from regression/failure of development of the left pulmonary artery. […] In the group of vascular rings and slings as a whole, respiratory symptoms predominate in the initial presentation. The severity of compression determines the severity of symptoms. About 70% to 90% of patients will have respiratory symptoms. Stridor is present in almost all cases. […] The diagnosis of vascular rings is challenging. A high index of suspicion is necessary because of the relatively low incidence of these anomalies in contrast to the common symptoms the patients exhibit.

• #1 Clinical Course of Vascular Rings and Risk Factors Associated With Mortality

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3341422/

  Vascular rings refer to anomalies of the great arteries that cause respiratory or feeding problems. […] Vascular rings are rare congenital anomalies that primarily result from an embryological derangement of the paired aortic arches or branching pulmonary arteries. […] The presence of a complex heart disease significantly influenced mortality (p=0.002). […] The presence of complex heart disease was significantly associated with mortality. […] The factors associated with mortality are shown in Table 4. We found that only the presence of complex heart disease significantly influenced mortality. […] Overall prognosis for a vascular ring was relatively good in this study, though vascular rings combined with complex heart disease showed a poor prognosis. […] In conclusion, vascular rings include several types of anomalies, each with different symptoms and prognosis. The presence of complex heart disease was significantly associated with mortality.

• #1 Expertise in the nuances of diagnosis, management and surgery for vascular rings – Mayo Clinic

  https://www.mayoclinic.org/medical-professionals/pediatrics/news/expertise-in-the-nuances-of-diagnosis-management-and-surgery-for-vascular-rings/mac-20530282

  Vascular rings are a group of diagnoses present at birth caused by abnormal development of the brachial arch system, which results in a ring of vessels encircling and constricting the airway and esophagus. […] There is no known cause of or genetic link for vascular rings. […] Symptoms such as difficulty swallowing, difficulty feeding, or wheezing and coughing can appear early in infancy, especially in those with double aortic arch. […] Surgical intervention is nearly always recommended to release vascular rings. […] The subgroup of left arch with aberrant right subclavian, technically a sling, rarely causes significant symptoms. These patients usually do not need intervention, but there is a select group in which the anatomy causes significant dysphagia.

• #1 Long term respiratory morbidity in patients with vascular rings: a review | Italian Journal of Pediatrics | Full Text

  https://ijponline.biomedcentral.com/articles/10.1186/s13052-023-01430-x

  Abnormalities in position and/or branching of the aortic arch can lead to vascular rings that may cause narrowing of the tracheal lumen due to external compression, or constriction of the oesophagus, causing symptoms that vary in relation to the anatomical vascular pattern and the relationship between these structures. […] Vascular rings (VRs) account for 1% of congenital cardiac defects. Their real incidence is difficult to establish as the clinical presentations are non-specific and range from early onset of symptoms to asymptomatic clinical setting into adulthood. […] Though most VRs are isolated, congenital heart defects and chromosomal abnormalities can be associated. […] Extrinsic airway compression (EAC) and malacia are common complications of vascular rings. […] The mere presence of a vascular ring is not an indication in itself for surgical intervention. It is important to know that symptoms in small children may disappear as the child and chest cage grow.

• #2 Vascular Rings | Symptoms & Causes | Freedmans Health

  https://freedmanshealth.org/disease-conditions/symptoms-causes/vascular-rings/

  A vascular ring occurs when the aorta forms abnormally during fetal development. […] A vascular ring is a congenital defect, meaning its present at birth. […] A vascular ring can form in many different ways. […] Vascular rings are rare, occurring in less than 1% of babies. […] The condition can affect any ethnicity. For unknown reasons, its about twice as common in babies assigned male at birth as in babies assigned female at birth.

• #2 Vascular Rings: Causes and Symptoms

  https://my.clevelandclinic.org/health/diseases/23947-vascular-rings

  A vascular ring occurs when the aorta forms abnormally during fetal development. […] For reasons scientists dont understand yet, vascular rings occur during fetal development. Often, parts of the aorta that should disappear during fetal development dont go away.

• #2 Vascular Ring | Thoracic Key

  https://thoracickey.com/vascular-ring/

  Vascular ring reportedly represents less than 1% of all congenital cardiovascular anomalies, but this may be an underestimate because some conditions are asymptomatic. […] Vascular ring refers to a group of anomalies of the aortic arch that cause respiratory symptoms or feeding problems. […] Double aortic arch is the most common vascular ring (40%). This anomaly is caused by a failure of regression of both the right and left fourth branchial arches, resulting in right and left aortic arches, respectively. […] Right aortic arch with left ligamentum arteriosum has several different forms; two common types are described here. […] Anomalous innominate artery occurs in about 10% of patients with vascular ring. […] Aberrant right subclavian artery is the most common arch anomaly (accounting for 0.5% of the general population, but its true incidence may be higher if asymptomatic patients are included). […] Anomalous left PA (also called vascular sling) is a rare anomaly in which the left PA arises from the right PA.

• #2 Multidisciplinary approach to vascular rings and vascular-related aerodigestive compression: a clinical practice review – Chiu – Translational Pediatrics

  https://tp.amegroups.org/article/view/114864/html

  Vascular rings, including double aortic arch and right aortic arch with aberrant left subclavian and left ligamentum, are part of a larger group of vascular-related aerodigestive compression syndromes that also includes innominate artery compression syndrome, dysphagia lusoria, aortic arch anomalies, and aneurysms of either the aorta or pulmonary artery. […] Vascular rings and vascular-related aerodigestive compression syndromes encompass a variety of lesions that involve abnormal development of the aortic arch with resultant vascular compression of either the trachea, the esophagus, or both. True, or complete, vascular rings include double aortic arch and right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum and have a prevalence of approximately 11.3 per 10,000 live births.

• #2 Case Based Pediatrics Chapter

  http://hawaii.edu/medicine/pediatrics/pedtext/s07c07.html

  Vascular rings encompass only 1% to 3% of all congenital heart disease. Some vascular rings are associated with other congenital heart lesions while others are isolated defects. Tracheobronchial anomalies are rarely seen with vascular rings but are common in pulmonary artery slings. […] A pulmonary sling is the left pulmonary artery arising from the right pulmonary artery. It is also known as an anomalous pulmonary artery and results from regression/failure of development of the left pulmonary artery. […] In the group of vascular rings and slings as a whole, respiratory symptoms predominate in the initial presentation. The severity of compression determines the severity of symptoms. About 70% to 90% of patients will have respiratory symptoms. Stridor is present in almost all cases. […] The diagnosis of vascular rings is challenging. A high index of suspicion is necessary because of the relatively low incidence of these anomalies in contrast to the common symptoms the patients exhibit.

• #2 32: Vascular Rings and Slings | Veterian Key

  https://veteriankey.com/32-vascular-rings-and-slings/

  Airway obstruction in children and its accompanying symptoms of stridor and respiratory distress can be caused by intrinsic abnormalities of the tracheobronchial tree and/or extrinsic compression of the airway. Among the causes of the latter are selected abnormalities in the embryologic development of the aorta or pulmonary arteries that may be grouped together as vascular rings and slings. […] The etiology of most vascular rings is unknown. Chromosome 22q11.2 deletions are associated with isolated aortic arch anomalies, including vascular rings, and also with conotruncal cardiac defects and noncardiac abnormalities.

• #2 Prenatal ultrasound scans showing a vascular ring | Great Ormond Street Hospital

  https://www.gosh.nhs.uk/conditions-and-treatments/procedures-and-treatments/prenatal-ultrasound-scans-showing-vascular-ring/

  When we carried out a prenatal ultrasound scan in the Fetal Cardiology clinic, we were able to confirm that your baby has a structurally normal heart but there is an unusual arrangement of the great arteries after they have left the heart called a vascular ring. This arrangement has the potential to form a ring round the trachea (windpipe) and oesophagus (food pipe). […] Doctors think that vascular rings occur early in pregnancy when the baby is developing in the womb. They do not think it was caused by anything you did or did not do during pregnancy. […] The blood vessels in the chest form early in pregnancy as a series of arches. Some of these arches shrink and disappear as pregnancy progresses. Vascular rings develop when some of these arches continue to develop abnormally. […] Some vascular rings occur because of an associated chromosome problem. An example of a chromosome problem is Downs Syndrome or Di George Syndrome.

• #2 Vascular rings | Health Library | Memorial Health System

  https://www.mhsystem.org/health-library/con-20359711/

  A vascular ring is a heart condition present at birth. That means its a congenital heart defect. […] The exact cause of vascular rings is not known. Congenital heart conditions happen as a baby, also called a fetus, is growing in the womb during pregnancy. […] Some things that may play a role in the development of congenital heart conditions are: Gene changes. Some medicines. Some health conditions. Lifestyle choices, including smoking. Things in your environment. […] What increases the risk of vascular rings is not well known. Possible risk factors for vascular rings and other congenital heart conditions are: Genetics. Changes in some genes have been linked to congenital heart conditions. For example, people with Down syndrome often are born with heart conditions. Diabetes. Having type 1 or type 2 diabetes during pregnancy may change how the unborn babys heart forms. But diabetes that develops during pregnancy doesnt typically increase a babys risk of congenital heart conditions. Rubella, also called German measles. Having rubella during pregnancy can cause harmful changes in an unborn babys heart. A blood test can be done before pregnancy to see if youre immune to rubella. A vaccine is available for people who arent immune. Some medicines. Taking some medicines during pregnancy may increase the risk of congenital heart conditions. Examples include lithium (Lithobid) for bipolar disorder and isotretinoin (Claravis, Myorisan, others), which is used to treat acne. Talk with a healthcare professional about the medicines you take. Smoking. If you smoke, quit. Smoking during pregnancy or being around tobacco smoke increases the risk of some congenital heart conditions. Alcohol use. Drinking alcohol during pregnancy may increase the risk of heart conditions in the baby.

• #2 Double aortic arch – UF Health

  https://ufhealth.org/conditions-and-treatments/double-aortic-arch

  Double aortic arch is a common form of a group of defects that affect the development of the aorta in the womb. These defects cause an abnormal formation called a vascular ring (a circle of blood vessels). […] Double aortic arch is very rare. Vascular rings make up a small percentage of all congenital heart problems. Of these, a little more than half are caused by double aortic arch. […] A double aortic arch may occur in other congenital heart defects, including: Tetralogy of Fallot, Truncus arteriosus, Transposition of the great arteries, Ventricular septal defect.

• #2 Vascular Ring Quiz: Check Possibility & Treatment with Ubie AI Symptom Checker

  https://ubiehealth.com/diseases/vascular-ring

  A vascular ring is a malformation of the aortic arch, which is the main blood vessel leading from the heart. Due to this malformation, the aortic arch and its branches may partially or completely encircle the windpipe (trachea), the esophagus (the tube connecting the mouth to the stomach), or both, causing breathing and digestive issues. […] Surgery is typically required to treat vascular rings that press against the trachea or esophagus and to prevent complications. Individuals with vascular rings may need lifelong care and regular follow-up appointments with specialists to monitor their conditions.

• #2 Vascular ring – Knowledge and References – Taylor & Francis

  https://taylorandfrancis.com/knowledge/Medicine_and_healthcare/Cardiology/Vascular_ring/

  A vascular ring is a rare congenital condition in which an anomalous configuration of the aortic arch or associated vessels surrounds the trachea and esophagus, forming a complete compressing ring around them. This anomaly affects both males and females and is caused by abnormal regression or persistence of one of the six embryonic aortic arches. […] The most common vascular ring is caused by a double aortic arch encircling the trachea and oesophagus causing compression of these structures. A right aortic arch combined with an aberrant subclavian artery passing behind the oesophagus with a duct or ligament can also complete a ring. […] Several other related vascular anomalies involving aortic arch vessels do not form complete rings but have been grouped descriptively with vascular rings because they can produce similar symptoms related to compression of the trachea and or esophagus.

• #3 Vascular rings and slings – UpToDate

  http://www.uptodate.com/contents/vascular-rings

  Vascular rings are congenital anomalies of the aortic arch that result in compression of the tracheobronchial tree and/or esophagus, leading to respiratory and gastrointestinal symptoms. They can be classified as either complete (when both the trachea and esophagus are fully encircled by a vascular anomaly) or incomplete (without full encirclement of both structures). […] The different forms of vascular rings and slings and their clinical manifestations, diagnosis, and treatment are discussed here. […] Beginning at the fourth week of embryogenesis, the aortic arch develops from six symmetrical paired aortic arch vessels and the paired dorsal aortae. During the next few weeks of embryogenesis, remodeling and rearrangement of these structures result in the formation of the normal left aortic arch.

• #4 Vascular Ring | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/vascular-ring

  A vascular ring occurs when a childs aorta the bodys largest blood vessel or its branches form abnormally, so that they encircle and constrict the trachea and usually the esophagus. This compression can cause breathing and swallowing problems. […] Vascular rings are caused by malformed blood vessels. But the vessels themselves arent really the issue, and some people without symptoms may live their entire lives not even realizing that they have a vascular ring. Instead, symptoms occur when a vascular ring puts pressure on a childs esophagus, trachea, or both. […] Normally, the aorta develops from one in a series of symmetrical arches. By the end of the second month of fetal development, the other arches are naturally broken down or formed into arteries. When a vascular ring occurs, certain arches that should have disappeared still remain and form a ring structure.

• #5 Vascular Ring Symptoms, Diagnosis and Repair

  https://resources.healthgrades.com/right-care/vascular-conditions/vascular-ring

  A vascular ring is a rare vascular irregularity when the aortic arch presses on the trachea and esophagus. It is usually a result of a congenital defect present at birth. […] A vascular ring is formed in utero. Usually, by the end of the second month of fetal development, the branches of the aortic arches have formed into arteries. However, when a vascular ring forms, the branches remain in place and form a ring. […] Vascular rings are not genetic and do not appear to run in families. Vascular rings usually form early on in fetal development.

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