TY  - CHAP
M1  - Book, Section
TI  - Coarctation of the Aorta and Interrupted Aortic Arch
A1  - Mark, D. Rodefeld
A2  - Yuh, David D.
A2  - Vricella, Luca A.
A2  - Yang, Stephen C.
A2  - Doty, John R.
PY  - 2014
T2  - Johns Hopkins Textbook of Cardiothoracic Surgery
AB  - Coarctation  of  the  aorta  (CoA)IntroductionCoA  is  defined  as  a  hemodynamically  significant  narrowing  of  the  aorta.  It  occurs  in  about  4  in  10,000  births  and  accounts  for  more  than  5  percent  of  congenital  heart  defects.Clinical  featuresCoA  presents  as  a  spectrum  of  disease,  ranging  from  neonatal  ductal  dependence  to  newly  diagnosed,  previously  unrecognized,  long-standing  hypertension  in  an  adult.  One-third  of  neonates  have  an  isolated  CoA,  one-third  a  ventricular  septal  defect  (VSD),  and  one-third  have  complex  congenital  heart  disease.  A  bicuspid  aortic  valve  is  present  in  50  percent.  Up  to  80  percent  of  neonates  with  hypoplastic  left  heart  syndrome  have  a  CoA.DiagnosisEchocardiography  is  the  diagnostic  modality  of  choice  in  neonates.  In  older  patients,  computed  tomography,  magnetic  resonance  imaging  and  angiography  are  employed.TreatmentSurgical  treatment  is  preferred,  with  several  techniques  available.  The  choice  of  procedure  depends  on  the  anatomy  of  the  aorta  and  associated  anomalies.  Native  CoA  balloon  angioplasty  is  possible  but  has  decreased  long-term  success  in  neonates;  its  use  is  perhaps  of  better  application  in  desperately  ill  neonates  and  older  children  and  recoarctation.  Endovascular  stents  have  emerged  as  a  possible  alternative  to  surgery,  but  long-term  data  are  not  available.ResultsSurgical  repair  of  isolated  CoA  in  the  neonatal  period  can  be  accomplished  with  minimal  morbidity  and  mortality,  whereas  the  results  of  repair  of  CoA  in  association  with  complex  congenital  heart  disease  vary  according  to  the  dominant  cardiac  pathology  and  patient-related  variables.  Recoarctation  occurs  in  up  to  30  percent  of  patients  corrected  in  the  neonatal  period,  with  a  similar  incidence  despite  the  different  techniques  utilized.  Late  hypertension  is  common  in  patients  operated  later  in  life  and  is  partly  responsible  for  the  slightly  decreased  long-term  survival  of  this  patient  population.Interrupted  aortic  arch  (IAA)MorphologyIn  this  condition  there  is  anatomic  lack  of  continuity  in  the  aortic  arch,  classified  according  to  the  site  of  occurrence  into  type  A  (distal  to  left  subclavian),  type  B  (between  the  left  common  carotid  and  subclavian),  and  type  C  (just  distal  to  the  innominate  artery).  Type  B  is  most  common  and  is  associated  with  thymic  agenesis  and  22q11  microdeletion.  The  prevalence  of  IAA  is  0.003  per  1000  live  births.  A  VSD  is  nearly  always  present.  Bicuspid  aortic  valve  is  found  in  50  percent  of  infants,  with  left  ventricular  outflow  tract  obstruction  (LVOTO)  often  seen  because  of  hypoplasia  of  the  aortic  root  or  posterior  malalignment  of  the  infundibular  septum.Clinical  featuresIn  newborns  not  prenatally  diagnosed,  presentation  is  rapid  because  of  ductal  closure  and  resulting  visceral  hypoperfusion  and  shock.  Peripheral  pulses  vary  according  to  the  site  of  interruption  and  there  is  variable  pulmonary  overcirculation.  Median  time  of  death  if  untreated  is  between  4  and  10  days  from  birth,  with  75  percent  mortality  within  1  year.DiagnosisEchocardiography  is  the  diagnostic  modality  of  choice  in  neonates.  In  more  complex  anomalies,  computed  tomography,  magnetic  resonance  imaging,  and  angiography  are  employed.TreatmentSurgical  treatment  follows  a  brief  period  of  stabilization  with  prostaglandin  (PGE1)  infusion  and  restoration  of  ductal  patency.  Single-stage  repair  of  aortic  arch  interruption  and  coexisting  cardiac  anomalies  is  then  undertaken.  Alternatively,  a  staged  approach  with  initial  arch  repair  and  banding  of  the  pulmonary  artery  followed  by  ...
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/16
UR  - accesssurgery.mhmedical.com/content.aspx?aid=1104597896
ER  -