TY  - CHAP
M1  - Book, Section
TI  - Total Anomalous Pulmonary Venous Connection
A1  - Kocyildirim, Ergin
A1  - Morell, Victor O.
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  - EpidemiologyTotal  anomalous  pulmonary  venous  connection  (TAPVC)  accounts  for  1  to  3  percent  of  all  cases  of  congenital  heart  defects.  Obstructed  TAPVC  is  one  of  the  few  true  emergencies  in  neonatal  surgery.TAPVC  is  either  an  isolated  lesion  or  associated  with  complex  atrial  isomerism.MorphologyAll  the  pulmonary  veins  drain  into  one  or  several  systemic  veins  instead  of  the  left  atrium  (LA).The  presence  of  an  inter-atrial  communication  is  crucial  to  sustain  life  and  an  atrial  septal  defect  (ASD)  or  a  patent  foramen  ovale  (PFO)  is  considered  as  a  part  of  TAPVC  complex.Four  anatomic  subtypes  of  TAPVC  are  recognized  based  on  the  location  of  the  anomalous  pulmonary  venous  drainage:  supracardiac  (40–50  percent),  cardiac  (20–25  percent),  infracardiac  (10–15  percent),  and  miscellaneous  or  mixed  (10  percent).PathophysiologyTAPVC  is  a  mixing  lesion  in  which  pulmonary  venous  blood  completely  mixes  with  systemic  venous  blood  and  returns  to  the  right  atrium  through  a  systemic  vein,  with  consequent  left-to-right  shunt.In  neonates  and  small  infants  with  obstructed  pulmonary  venous  return,  pulmonary  hypertension  and  pulmonary  edema  are  always  present.Clinical  featuresInfants  with  obstructed  TAPVC  present  with  cyanosis,  respiratory  distress  soon  after  birth.  Infants  with  unobstructed  TAPVC  present  later  than  infants  with  obstruction.DiagnosisEchocardiography  is  diagnostic.  Angiography  is  not  indicated.  Magnetic  resonance  imaging  (MRI)  may  be  useful  as  a  supplement  to  echocardiography.TreatmentSurgical  repair  involves  creation  of  a  connection  between  the  pulmonary  venous  confluence  and  the  LA.  Stabilization  with  preoperative  extracorporeal  membrane  oxygenation  (ECMO)  is  an  option  for  desperately  ill  neonates.OutcomesThe  long-term  outcome  for  patients  with  repaired  TAPVC  is  very  good,  with  an  operative  mortality  of  10  percent.  Pulmonary  venous  stenosis  is  a  relatively  uncommon  complication  but,  when  it  occurs,  the  impact  on  prognosis  is  profound.  
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/16
UR  - accesssurgery.mhmedical.com/content.aspx?aid=1104597038
ER  -