TY  - CHAP
M1  - Book, Section
TI  - Vascular Rings and Pulmonary Artery Sling
A1  - Backer, Carl Lewis
A1  - Mongé, Michael C.
A1  - Russell, Hyde M.
A2  - Yuh, David D.
A2  - Vricella, Luca A.
A2  - Yang, Stephen C.
A2  - Doty, John R.
Y1  - 2014
N1  - 
T2  - Johns Hopkins Textbook of Cardiothoracic Surgery
AB  - EpidemiologyVascular  rings  and  pulmonary  artery  sling  (PAS)  represent  1  to  3  percent  of  all  congenital  cardiac  malformations.  The  most  frequent  type  of  vascular  ring  is  aberrant  left  subclavian  artery,  whereas  the  most  rare  condition  is  the  PAS.MorphologyOf  the  over  100  variations  in  morphology  that  have  been  described,  6  are  most  commonly  observed:  (1)  anomalous  left  subclavian  artery  with  left  aortic  arch,  (2)  double  aortic  arch  (DAA),  (3)  right  aortic  arch  with  left  ligamentum,  (4)  right  aortic  arch  with  aberrant  left  subclavian  artery,  (5)  tracheal  compression  by  the  innominate  artery,  and  (6)  PAS.  Cardiac  malformations  (tetralogy  of  Fallot,  ventricular  septal  defect,  atrial  septal  defect,  and  aortic  coarctation)  are  seen  in  15  to  20  percent  of  cases.PathophysiologyStructural  obstruction  of  the  aerodigestive  tractClinical  featuresMost  vascular  rings  are  symptomatic.  Compression  by  the  complete  or  partial  ring  of  the  esophagus,  trachea,  or  both  dictates  the  mode  of  presentation.  Patients  with  an  aberrant  left  subclavian  artery  are  often  asymptomatic,  whereas  pulmonary  artery  sling  and  complete  rings  are  almost  uniformly  clinically  significant.DiagnosisChest  x-ray  is  rarely  diagnostic.  Barium  esophagography  is  usually  able  to  point  to  the  presence  of  a  vascular  ring  with  reasonable  specificity.  Computed  tomography  (CT)  and  magnetic  resonance  imaging  (MRI)  are  useful  in  defining  the  anatomy  and  are  currently  more  routinely  utilized.  Bronchoscopy  is  important  in  the  diagnosis  of  both  innominate  artery  compression  and  PAS  and  in  assessing  the  degree  of  tracheobronchomalacia.TreatmentTreatment  is  indicated  in  all  symptomatic  patients.  With  the  exception  of  DAA  with  dominant  left  component,  almost  all  cases  (left  aortic  arch  with  right  ligamentum,  PAS,  and  innominate  artery  compression)  are  approached  through  a  left  thoracotomy  with  division  of  the  vascular  ring.OutcomesFor  the  vast  majority  of  vascular  rings,  morbidity  and  mortality  are  the  same  as  seen  in  any  simple  thoracic  procedure  involving  a  thoracotomy.  Mortality  is  much  higher  for  patients  with  PAS.  The  degree  of  tracheobronchomalacia  has  a  significant  impact  on  long-term  prognosis.  
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/16
UR  - accesssurgery.mhmedical.com/content.aspx?aid=1104598823
ER  -