RT Book, Section
A1 Doherty, Gerard M.
SR Print(0)
ID 58109147
T1 Double-Outlet Right Ventricle
T2 Quick Answers Surgery
YR 2010
FD 2010
PB The McGraw-Hill Companies
PP New York, NY
SN 
LK accesssurgery.mhmedical.com/content.aspx?aid=58109147
RD 2024/04/25
AB •  A  congenital  heart  lesion  that  decreases  pulmonary  arterial  blood  flow  resulting  in  a  right  to  left  shunt•  Cyanosis  and  decreased  oxygen  delivery  causes  compensatory  polycythemia  (Hct  >  70%)  and  spontaneous  thrombosis•  Exercise,  acidosis,  pain  worsens  cyanosis  and  can  cause  hypoxic  spells•  Squatting  increases  systemic  resistance,  causing  increased  pulmonary  flow  and  blood  oxygenation•  β-Blockers  (decreases  spasm),  fluid  intake,  HCO3  administration,  norepinephrine  (increases  systemic  resistance)  may  help  decrease  hypoxia•  Clubbing  due  to  proliferation  of  capillaries  and  AV  fistulas  in  extremities•  Bronchial  and  mediastinal  arteries  enlarge•  Ductus  arteriosus  maintains  flow  to  lungs  during  fetal  development•  Alprostadil  early  can  allow  time  for  optimization  before  definitive  treatment•  Operative  options  to  increase  pulmonary  flow:  -Fontan  procedure:  Superior  vena  cava  and  inferior  vena  cava  rerouted  to  pulmonary  artery