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Vascular ring

Right aortic arch with aberrant subclavian and left ligamentum arteriosus; Congenital heart defect - vascular ring; Birth defect heart - vascular ring

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.

Causes

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.

Symptoms

Some children with a vascular ring never develop symptoms. However, in most cases, symptoms are seen during infancy. Pressure on the windpipe (trachea) and esophagus can lead to breathing and digestive problems. The more the ring presses down, the more severe the symptoms will be.

Breathing problems may include:

  • High-pitched cough
  • Loud breathing (stridor)
  • Repeated pneumonias or respiratory infections
  • Respiratory distress
  • Wheezing

Eating may make breathing symptoms worse.

Digestive symptoms are rare, but may include:

Exams and Tests

The health care provider will listen to the baby's breathing to rule out other breathing disorders such as asthma. Listening to the child's heart through a stethoscope can help identify murmurs and other heart problems.

The following tests can help diagnose vascular ring:

  • Chest x-ray
  • Computed tomography (CT) scan of the heart and major blood vessels
  • Camera down the throat to examine the airways (bronchoscopy)
  • Magnetic resonance imaging (MRI) of the heart and major blood vessels
  • Ultrasound examination (echocardiogram) of heart
  • X-ray of blood vessels (angiography)
  • X-ray of the esophagus using a special dye to better highlight the area (esophagram or barium swallow)

Treatment

Surgery is usually performed as soon as possible on children with symptoms. The goal of surgery is to split the vascular ring and relieve pressure on the surrounding structures. The procedure is usually done through a small surgical cut in the left side of the chest between the ribs.

Changing the child's diet may help relieve the digestive symptoms of vascular ring. The provider will prescribe medicines (such as antibiotics) to treat any respiratory tract infections, if they occur.

Children who do not have symptoms may not need treatment but should be carefully watched to make sure the condition does not become worse.

Outlook (Prognosis)

How well the infant does depends on how much pressure the vascular ring is putting on the esophagus and trachea and how quickly the infant is diagnosed and treated.

Surgery works well in most cases and often relieves symptoms right away. Severe breathing problems may take months to go away. Some children may continue to have loud breathing, especially when they are very active or have respiratory infections.

Possible Complications

Delaying surgery in serious cases can lead to serious complications, such as damage to the trachea and death.

When to Contact a Medical Professional

Contact your provider if your baby has symptoms of vascular ring. Getting diagnosed and treated quickly can prevent serious complications.

Prevention

There is no known way to prevent this condition.

References

Bryant R, Yoo S-J. Vascular rings, pulmonary arterial sling, and related conditions. In: Wernovsky G, Anderson RH, Kumar K, et al, eds. Anderson's Pediatric Cardiology. 4th ed. Philadelphia, PA: Elsevier; 2020:chap 47.

Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM. Other congenital heart and vascular malformations. In: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Textbook of Pediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020:chap 459.

Valente AM, Dorfman AL, Babu-Narayan SV, Krieger EV. Congenital heart disease in the adolescent and adult. In: Libby P, Bonow RO, Mann DL, Tomaselli GF, Bhatt DL, Solomon SD, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 12th ed. Philadelphia, PA: Elsevier; 2022:chap 82.

Text only

  • Congenital heart defects (CHD) overview

    Congenital heart defects (CHD) overview

    Animation

  •  

    Congenital heart defects (CHD) overview - Animation

    Congenital heart disease describes defects of the heart or its vessels that are present at birth. They are the most common types of birth defect, affecting 1 out of every 120 babies. Some heart defects are severe, but many are not, ranging from simple defects with no symptoms, to complex defects with severe life-threatening symptoms. Each year, more than 35,000 babies in the United States are born with congenital heart defects. Most are simple defects that are easily corrected or do not need treatment. However, a small number of babies are born with complex heart defects and require special medical attention soon after birth. Over the past few decades, the diagnosis and treatment of these complex defects has greatly improved. Thanks to new and effective treatments, almost all children with complex heart defects grow to adulthood and can live active, productive lives. Most of these patients will nonetheless continue to need special heart care throughout their lives. Living with a congenital heart disease can also impact health insurance, employment, pregnancy, contraception, and the risk of infection during routine health procedures. Today, approximately 1 million American adults are living with congenital heart defects.

  • Vascular ring

    Vascular ring - illustration

    Vascular ring is a term used to describe a number of abnormal formations of the aorta, the large artery that carries blood from the heart to the rest of the body, or of the pulmonary artery. The abnormal vessel(s) forms a ring, which encircles and may press down on the windpipe (trachea) or the esophagus. The additional pressure on the windpipe (trachea) and esophagus can lead to breathing and swallowing problems.

    Vascular ring

    illustration

  • Congenital heart defects (CHD) overview

    Animation

  •  

    Congenital heart defects (CHD) overview - Animation

    Congenital heart disease describes defects of the heart or its vessels that are present at birth. They are the most common types of birth defect, affecting 1 out of every 120 babies. Some heart defects are severe, but many are not, ranging from simple defects with no symptoms, to complex defects with severe life-threatening symptoms. Each year, more than 35,000 babies in the United States are born with congenital heart defects. Most are simple defects that are easily corrected or do not need treatment. However, a small number of babies are born with complex heart defects and require special medical attention soon after birth. Over the past few decades, the diagnosis and treatment of these complex defects has greatly improved. Thanks to new and effective treatments, almost all children with complex heart defects grow to adulthood and can live active, productive lives. Most of these patients will nonetheless continue to need special heart care throughout their lives. Living with a congenital heart disease can also impact health insurance, employment, pregnancy, contraception, and the risk of infection during routine health procedures. Today, approximately 1 million American adults are living with congenital heart defects.

  • Vascular ring

    Vascular ring - illustration

    Vascular ring is a term used to describe a number of abnormal formations of the aorta, the large artery that carries blood from the heart to the rest of the body, or of the pulmonary artery. The abnormal vessel(s) forms a ring, which encircles and may press down on the windpipe (trachea) or the esophagus. The additional pressure on the windpipe (trachea) and esophagus can lead to breathing and swallowing problems.

    Vascular ring

    illustration

 

Review Date: 2/23/2022

Reviewed By: Thomas S. Metkus, MD, Assistant Professor of Medicine and Surgery, Johns Hopkins University School of Medicine, Baltimore, MD. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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