TY  - CHAP
M1  - Book, Section
TI  - Cardiac Surgical Intervention in Pediatric Patients with Connective Tissue Disorders
A1  - Zanotti, Giorgio
A1  - Cameron, Duke E.
A1  - Vricella, Luca A.
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  - Thoracic  aortic  aneurysm  syndromes  are  due  to  genetic  disorders  inherited  as  autosomal  dominant  traits  with  reduced  penetrance  and  incomplete  expression.  Hence,  clinical  phenotype  is  complex  and  highly  variable  with  often  no  genotype-phenotype  correlation.Altered  TGF-β  signaling  and  proteolytic  metabolism  involving  the  entire  aortic  wall  are  now  challenging  old  pathophysiologic  models  by  which  aortic  aneurysms  were  solely  caused  by  extracellular  matrix  structural  protein  deficiency  in  the  media.Indications  for  surgery  are  still  mainly  based  on  aortic  diameter  as  well  as  aortic  growth  rate  and  family  history  of  early  rupture/dissection.  However,  efforts  are  made  to  identify  circulating  markers  associated  with  early  complications  to  guide  risk  stratification.Surgery  is  the  only  treatment  that  can  modify  the  natural  history  of  pediatric  patients  with  thoracic  aortic  aneurysm  syndromes.  Although  promising,  medical  treatment  has  not  yet  been  validated.Aortic  valve-sparing  root  replacement  (AVSRR)  is  currently  the  favored  surgical  approach,  whereas  the  Bentall  operation  should  be  reserved  for  patients  with  acute  aortic  dissection,  unrepairable  leaflet  damage,  or  failed  AVSRR.  Early  and  midterm  results  of  AVSRR  in  pediatric  patients  are  excellent  and  comparable  to  those  obtained  with  composite  valve  graft  root  replacement.  Long-term  event-free  survival  in  this  particular  group  of  patients  has  yet  to  be  assessed.  
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/18
UR  - accesssurgery.mhmedical.com/content.aspx?aid=1104598462
ER  -