TY  - CHAP
M1  - Book, Section
TI  - Double Outlet Right Ventricle
A1  - Thompson, Jess
A1  - Dearani, Joseph 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  - EpidemiologyRelatively  uncommon  congenital  anomaly  seen  in  0.09  in  1000  birthsPathophysiologyConsiderable  variability  in  pathophysiology  is  seen  with  double  outlet  right  ventricle  (DORV)  and  is  primarily  dependent  on  the  location  of  the  ventricular  septal  defect  (VSD),  the  relationship  of  the  great  arteries  and  the  presence  of  great  vessel  stenosisClinical  featuresPatients  usually  present  by  age  2  months  (range  1  day–4  years).  Presentation  is  variable  and  is  dependent  on  the  type  of  DORV  and  associated  cardiac  anomalies.  Heart  failure  is  seen  in  patients  with  unrestricted  subaortic,  doubly-committed  or  non-committed  VSDs  without  right  ventricular  outflow  tract  obstruction  (RVOTO).  Cyanosis  results  from  RVOTO  leading  to  restriction  of  pulmonary  blood  flow  or  by  preferential  streaming  of  blood  from  the  left  ventricle  (LV)  to  pulmonary  artery  (PA)  as  seen  in  the  Taussig–Bing  anomaly.DiagnosisEchocardiography  defines  the  relationship  of  the  great  arteries  to  the  right  ventricle  (RV)  and  to  each  other,  and  delineates  the  size  and  position  of  the  VSD.  MRI  may  provide  complementary  information  about  intracardiac  anatomy,  size  and  status  of  the  aortic  arch  and  PAs,  and  3-dimensional  relationship  of  chambers  and  great  arteries.  Catheterization  can  be  performed  to  determine  hemodynamics,  exclude  pulmonary  vascular  disease  and  assess  coronary  anatomy.TreatmentDefinitive  management  includes  operation,  preferably  complete  repair  during  infancy.  The  surgical  repair  is  tailored  to  address  the  variable  anatomical  abnormalities.  In  patients  with  a  subaortic  or  doubly-committed  VSD  without  RVOTO,  the  repair  is  performed  with  an  intraventricular  tunnel  from  the  VSD  to  the  aorta.  If  RVOTO  exists,  right  ventricular  outflow  tract  (RVOT)  augmentation  or  conduit  placement  is  performed.  The  Taussig–Bing  anomaly  is  treated  with  the  arterial  switch  operation  and  tunneling  of  the  VSD  to  the  PA.  Patients  with  a  remote  VSD,  other  complex  valve  abnormalities  or  unbalanced  ventricles  may  require  staged  palliative  single  ventricle  procedures.Outcomes/prognosisOutcomes  are  determined  by  the  anatomy  and  the  repair  performed.  Late  results  may  range  from  those  of  tetralogy  of  Fallot  to  those  of  complex  intraventricular  tunnels,  with  potential  use  of  conduit,  who  are  at  subsequent  risk  for  subaortic  obstruction,  conduit  failure,  and  need  for  reoperation(s).  Patients  undergoing  the  arterial  switch  operation  have  excellent  late  survival  but  may  have  a  greater  incidence  of  neoaortic  valve  regurgitation  requiring  subsequent  reoperation.  Complex  biventricular  intracardiac  repairs  are  associated  with  a  higher  operative  risk  and  the  potential  need  for  reoperations.  Fontan  procedures  for  complex  anatomy  are  associated  with  less  operative  mortality  and  lower  reoperation  rates.  Functional  benefits  of  complex  biventricular  repairs  compared  to  single-ventricle  palliation  have  not  been  defined.  
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/19
UR  - accesssurgery.mhmedical.com/content.aspx?aid=1104596711
ER  -