Pulmonary artery sling is a rare condition in which the blood vessels between the heart and the lungs have formed incorrectly before birth. It is a type of cardiovascular condition called a

vascular ring 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, whic ...

.The main treatment is surgery.

Symptoms and signs

Symptoms include cyanosis,

dyspnoea Shortness of breath (SOB), also medically known as dyspnea (in AmE) or dyspnoea (in BrE), is an uncomfortable feeling of not being able to breathe well enough. The American Thoracic Society defines it as "a subjective experience of breathing dis ...

and apnoeic spells. Rarely it is asymptomatic and is detected incidentally in asymptomatic adults.

Cause

In pulmonary artery sling, the

left pulmonary artery A pulmonary artery is an artery in the pulmonary circulation that carries deoxygenated blood from the right side of the heart to the lungs. The largest pulmonary artery is the ''main pulmonary artery'' or ''pulmonary trunk'' from the heart, and ...

anomalously originates from a normally positioned

right pulmonary artery A pulmonary artery is an artery in the pulmonary circulation that carries deoxygenated blood from the right side of the heart to the lungs. The largest pulmonary artery is the ''main pulmonary artery'' or ''pulmonary trunk'' from the heart, and ...

. The left pulmonary artery arises anterior to the right main

bronchus A bronchus is a passage or airway in the lower respiratory tract that conducts air into the lungs. The first or primary bronchi pronounced (BRAN-KAI) to branch from the trachea at the carina are the right main bronchus and the left main bronchu ...

near its origin from the

trachea The trachea, also known as the windpipe, is a cartilaginous tube that connects the larynx to the bronchi of the lungs, allowing the passage of air, and so is present in almost all air- breathing animals with lungs. The trachea extends from the ...

, courses between the trachea and the

esophagus The esophagus ( American English) or oesophagus (British English; both ), non-technically known also as the food pipe or gullet, is an organ in vertebrates through which food passes, aided by peristaltic contractions, from the pharynx to ...

and enters the left hilum.

Treatment

It almost always requires surgical intervention. The surgery is usually

open heart surgery Cardiac surgery, or cardiovascular surgery, is surgery on the heart or great vessels performed by cardiac surgeons. It is often used to treat complications of ischemic heart disease (for example, with coronary artery bypass grafting); to c ...

with an incision through the sternum.

History

The first known case of pulmonary artery sling was diagnosed and surgically repaired by

Willis J. Potts Willis John Potts (March 22, 1895 – May 5, 1968) was an American pediatric surgeon and one of the earliest physicians to focus on the surgical treatment of heart problems in children. Potts set up one of the country's first pediatric surgery pr ...

Lurie Children's Hospital Ann & Robert H. Lurie Children's Hospital of Chicago, formerly Children's Memorial Hospital and commonly known as Lurie Children's, is a nationally ranked pediatric acute care children's hospital located in Chicago, Illinois. The hospital has 3 ...

in 1953.

References

External links

Vascular diseases Congenital vascular defects Rare diseases {{circulatory-disease-stub