Vascular Rings and Slings - December 2005

CXR

Diagnosis: Diagnosis
Vascular Ring- Ring probable double aortic arch

Vascular Rings & Slings
Morning Report
December 13, 2005

Introduction
“Vascular Vascular ring” ring refers to a variety of
congenital vascular anomalies that encircle
and compress the esophagus and trachea
Complete or “true true ring” ring vs. Incomplete
Abnormalities of the aortic arch and its
branching arteries make up most of the
“vascular vascular rings” rings (exception- (exception pulmonary artery sling)

In the embryonic aortic arch,
the ventral and dorsal aorta
are connected by six primitive
aortic arches
Regressions or persistence of
the primitive arches cause the
different vascular ring
anomalies
A “left left” or “right right” aortic arch is
defined by the mainstem mainstem
bronchus bronchus that is crossed by the
aorta arch (not to the side of the
midline the aorta descends)
Embryology
www.childsdoc.org/spring98/vascular

Clinical Presentations
Respiratory Symptoms
(Most Common)
Inspiratory stridor
“noisy noisy breathing” breathing or
wheezing
Chronic cough
Recurrent respiratory
infections
Hoarse cry
ALTE/apnea (infants)
Feeding Symptoms
(More common in older pts)
Gagging or choking w/
food
Recurrent emesis
Dysphagia (typically in older
children/ especially with solid
foods)
Dysphagia
Failure to thrive (food
avoidance)
Symptoms are often made worse w/ feeding and during intercurrent illness

Often Normal
Physical Exam
Poor weight gain (if severe compression)
Pulmonary exam may reveal wheezing, stridor, stridor,
dyspnea, dyspnea,
retractions
Abnormal positioning may be observed
– Lie w/ neck extended, back arched when supine to
minimize tracheal narrowing

Complete
Complete
(encircle and compress the
esophagus and trachea)
– Double aortic arch
– Right aortic arch w/
aberrant left
subclavian (and left
ligamentum arteriosum)
arteriosum
These two anomalies
make up >95% of all
complete vascular rings
Vascular Rings
Incomplete
Incomplete
(compress but do not encircle
the esophagus and trachea)
– Aberrant right
subclavian artery
– Anomalous innominate
artery
– Pulmonary vascular
sling
– Right aortic arch w/
mirror image
branching

Park: Pediatric Cardiology for Practitioners, 4th ed., Copyright © 2002 Mosby, Inc

Normal Aortic Arch Anatomy
(aka innominate
artery)
www.pediatriconcall.com/fordoctor/Diseasesandcondition/Difficultydia.asp

Double Aortic Arch
Most common vascular
ring (40%)
Persistence of both right
and left branchial arches
Infants present early in
life w/ respiratory distress
(stridor stridor) ) and feeding
problems
Right-arch Right arch dominant is
most common (75%)
Usually an isolated
anomaly
www.childsdoc.org/spring98/vascular

Right Aortic Arch w/ Aberrant Left
Second most common vascular
ring (30%)
The ring is formed by the right
aortic arch w/ left sided
ligamentum arteriosum
attached to an aberrant LSA
Present later in life (3-9mo) (3 9mo)
b/c the ring is “looser looser”
compared to double aortic arch
Not often associated w/
congenital heart defects
(except w/ “mirror mirror image
branching” branching type)
Subclavian Artery
www.childsdoc.org/spring98/vascular

Right Aortic Arch Anomalies
Complete Ring Incomplete Ring
www.childsdoc.org/spring98/vascular

Incomplete Vascular Rings
Anomalous Innominate Artery
– The innominate arises from the left side of the aortic arch and
compresses the trachea
– Asymptomatic or causes mild respiratory symptoms
– Barium Esophagram is normal; Bronchoscopy can be diagnostic
– Commonly associated with other cardiac defects
Aberrant Right Subclavian Artery
– RSA arises from the descending aorta and courses behind the
esophagus
– Usually asymptomatic
– Mild feeding problems may occur/swallowing dysfunction
– Usually an isolated anomaly

Anomalous Left Pulmonary Artery
“Pulmonary Pulmonary Vascular Sling” Sling
– Left PA arises from the RPA
and courses behind the
trachea and in front of the
esophagus to enter the hilum
of the left lung
– Respiratory AND feeding
problems may occur-
symptoms may be severe
– Esophagram shows anterior anterior
compression only
– Bronchoscopy is required to
r/o associated tracheal
stenosis w/ complete tracheal
rings
“ring ring-sling sling complex” complex (50%) www.childsdoc.org/spring98/vascular

Diagnosis
CXR (AP and lateral)
– Tracheal narrowing and/or displacement (lateral)
– Aortic arch location
– Tracheal Position: abnormal deviation to the left
(due to the aorta coursing over the right mainstem bronchus) OR
Midline position with double aortic arch
– Atelectasis, Atelectasis,
hyperinflation, or pneumonia may be present
(especially in pulmonary artery slings)
Barium Esophagram
Barium
– Most important and reliable diagnostic tool
Except in anomalous innominate artery
– Posterior (and anterior) compression of esophagus on lateral
Except in pulmonary artery sling sling anterior compression only

CXR
www.uhrad.com/ pedsarc/peds056a.jpg

Tracheal Deviation
www.pediatriconcall.com/fordoctor/Diseasesandcondition/Difficultydia.asp

Barium Esophagram

Other Imaging… Imaging
ECHO
– Recommended to exclude other cardiac defects and visualizes the
vascular ring
– Very useful in diagnosing pulmonary artery sling
CT/MRI
– Identify vascular structures and anatomy of tracheobronchial tree
Angiography
– Considered “gold gold standard”, standard , but rarely needed for diagnosis
Bronchoscopy
– Use as a diagnostic tool is controversial
– Recommended for diagnosis of a vascular sling to r/o concomitant
tracheal rings (and useful for aberrant innominate artery)

Management
Asymptomatic patients need no surgical treatment, even
when anomalies are found incidentally
Medical management is recommended for infants with
mild symptoms
– Careful feeding w/ soft foods
– Aggressive treatment of pulmonary infections
Respiratory distress, h/o recurrent pulmonary infections,
apneic spells, FTT are indications for surgical
intervention

Surgical Management
Double Aortic Arch
– Division of the smaller of the two arches (usually the left arch) arch
Right Aortic Arch w/ Aberrant LSA
– Division of the ligamentum arteriosum
Anomalous Innominate Artery
– Surgical suturing of the artery to the sternum (if >75% tracheal
narrowing)- narrowing) rarely indicated
Aberrant right Subclavian Artery
– Surgical interruption of the artery (rarely indicated)
Anomalous Left Pulmonary Artery (Pulmonary Vascular
Sling)
– Surgical division and reimplantation of the LPA

Complications
Surgical Mortality rate is generally low (