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Vascular Ring Double Aortic Arch

Abstract
The term "vascular ring" (VR) refers to the vascular structures that encircle and compress the esophagus and trachea, causing respiratory and gastrointestinal symptoms. VRs are further divided into two broad categories: complete or incomplete.  Complete VRs encircle the trachea and esophagus entirely. These include double aortic arch (DAA) and right aortic arch (RAA) with an aberrant (retro-esophageal) left subclavian artery. These are the most common types of vascular rings.[1][2]. Incomplete VRs do not completely encircle the trachea and esophagus, although some compress either the trachea or esophagus. It usually includes aberrant innominate artery, aberrant right subclavian artery, and pulmonary artery sling.[2]  An arch sidedness is defined by the position of the aortic arch in relation to the trachea and the bronchi. A left aortic arch would be towards the left of the trachea and run over the left bronchus, whereas an RAA would be towards the right of the trachea and run over the right bronchus. A DAA is when there are two transverse aortic arches running over the trachea and both bronchi. Normally, the arch sidedness is towards the left, and there is no arterial duct behind the trachea or esophagus. In cases of the vascular ring, there is a patent vessel, an atretic vessel, or its remnants circling the trachea or esophagus.  DAA can be further divided into three main types. The most common is the dominant RAA with a smaller LAA (80%). The dominant LAA is found in 10% and equal aortic arches in the rest 10%.  Depending on the site of the regression of the fourth aortic arch, RAA can be divided into 2 parts.[3] If regression is proximal to the left subclavian artery, it is called RAA with an aberrant (retro esophageal) left subclavian artery.[3] If regression is distal, it is called RAA with mirror image branching.[3] Around 50% of cases of right-sided aortic arch are associated with an aberrant (retro-esophageal) left subclavian artery.[4] This often has a Kommerell diverticulum named after the radiologist Dr. Burckhard F. Komerell, who reported this finding in 1936.[5] Kommerell diverticulum is an outpouching of the distal aorta and usually originates from the left arch.[5] Pulmonary artery sling occurs when the left pulmonary artery originates from the right pulmonary artery, which crosses between the trachea and esophagus before entering the left lung and thus compressing the trachea. It is the only type of VR which has an indentation in the anterior esophagus on barium swallow. Anomalous innominate artery originates later from the transverse arch and then crosses the trachea causing anterior tracheal compression.  According to the International Congenital Heart Surgery Nomenclature and Database Committee, the classification system for vascular rings is as follows:[1] A. Complete vascular rings 1. Double aortic arch (DAA) I. Dominant right arch II. Dominant left arch III. Equal arches/balanced arches 2. Right aortic arch (RAA) I. RAA+ aberrant left subclavian artery (ALSA) II. RAA with mirror imaging B. Incomplete aortic arch 1. Innominate artery compression syndrome 2. Pulmonary artery sling 3. Aberrant right subclavian artery (ARSA)

Publisher

StatPearls Publishing
Treasure Island (FL)

Language

eng

PubMed ID

32644364

Citation

Sahni D, Franklin WH: Vascular Ring Double Aortic Arch. StatPearls. StatPearls Publishing, 2020, Treasure Island (FL).
Sahni D, Franklin WH. Vascular Ring Double Aortic Arch. StatPearls. StatPearls Publishing; 2020.
Sahni D & Franklin WH. (2020). Vascular Ring Double Aortic Arch. In StatPearls. Treasure Island (FL): StatPearls Publishing
Sahni D, Franklin WH. Vascular Ring Double Aortic Arch. StatPearls. Treasure Island (FL): StatPearls Publishing; 2020.
* Article titles in AMA citation format should be in sentence-case
TY - CHAP T1 - Vascular Ring Double Aortic Arch BT - StatPearls A1 - Sahni,Deepank, AU - Franklin,Wayne H., Y1 - 2020/01// PY - 2020/7/10/pubmed PY - 2020/7/10/medline PY - 2020/7/10/entrez N2 - The term "vascular ring" (VR) refers to the vascular structures that encircle and compress the esophagus and trachea, causing respiratory and gastrointestinal symptoms. VRs are further divided into two broad categories: complete or incomplete.  Complete VRs encircle the trachea and esophagus entirely. These include double aortic arch (DAA) and right aortic arch (RAA) with an aberrant (retro-esophageal) left subclavian artery. These are the most common types of vascular rings.[1][2]. Incomplete VRs do not completely encircle the trachea and esophagus, although some compress either the trachea or esophagus. It usually includes aberrant innominate artery, aberrant right subclavian artery, and pulmonary artery sling.[2]  An arch sidedness is defined by the position of the aortic arch in relation to the trachea and the bronchi. A left aortic arch would be towards the left of the trachea and run over the left bronchus, whereas an RAA would be towards the right of the trachea and run over the right bronchus. A DAA is when there are two transverse aortic arches running over the trachea and both bronchi. Normally, the arch sidedness is towards the left, and there is no arterial duct behind the trachea or esophagus. In cases of the vascular ring, there is a patent vessel, an atretic vessel, or its remnants circling the trachea or esophagus.  DAA can be further divided into three main types. The most common is the dominant RAA with a smaller LAA (80%). The dominant LAA is found in 10% and equal aortic arches in the rest 10%.  Depending on the site of the regression of the fourth aortic arch, RAA can be divided into 2 parts.[3] If regression is proximal to the left subclavian artery, it is called RAA with an aberrant (retro esophageal) left subclavian artery.[3] If regression is distal, it is called RAA with mirror image branching.[3] Around 50% of cases of right-sided aortic arch are associated with an aberrant (retro-esophageal) left subclavian artery.[4] This often has a Kommerell diverticulum named after the radiologist Dr. Burckhard F. Komerell, who reported this finding in 1936.[5] Kommerell diverticulum is an outpouching of the distal aorta and usually originates from the left arch.[5] Pulmonary artery sling occurs when the left pulmonary artery originates from the right pulmonary artery, which crosses between the trachea and esophagus before entering the left lung and thus compressing the trachea. It is the only type of VR which has an indentation in the anterior esophagus on barium swallow. Anomalous innominate artery originates later from the transverse arch and then crosses the trachea causing anterior tracheal compression.  According to the International Congenital Heart Surgery Nomenclature and Database Committee, the classification system for vascular rings is as follows:[1] A. Complete vascular rings 1. Double aortic arch (DAA) I. Dominant right arch II. Dominant left arch III. Equal arches/balanced arches 2. Right aortic arch (RAA) I. RAA+ aberrant left subclavian artery (ALSA) II. RAA with mirror imaging B. Incomplete aortic arch 1. Innominate artery compression syndrome 2. Pulmonary artery sling 3. Aberrant right subclavian artery (ARSA) PB - StatPearls Publishing CY - Treasure Island (FL) UR - https://www.unboundmedicine.com/medline/citation/32644364/StatPearls:_Vascular_Ring_Double_Aortic_Arch L2 - https://www.ncbi.nlm.nih.gov/books/NBK558938 DB - PRIME DP - Unbound Medicine ER -
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