TY  - CHAP
M1  - Book, Section
TI  - Pulmonary Atresia
A1  - Doherty, Gerard M.
PY  - 2010
T2  - Quick Answers Surgery
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,  can  cause  hypoxic  spells•  Squatting  increases  systemic  resistance,  causing  increased  pulmonary  flow  and  oxygen•  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:  -Blalock-Taussig  shunt:  Subclavian  artery  to  ipsilateral  pulmonary  artery  (PA)  end  to  side  fashion-Modified  Blalock-Taussig  shunt:  Subclavian  to  PA  using  PTFE-Glenn:  Superior  vena  cava  (SVC)  to  PA  shunt  -Fontan:  SVC  and  inferior  vena  cava  (IVC)  rerouted  to  PA-Excision  of  obstructive  muscle,  patch  enlargement  of  infundibulum,  and  valve  replacement•  Pulmonary  valve  replaced  by  diaphragm  of  tissue,  causing  obstruction•  PA  normal  size•  Atrial  septal  defect  (ASD)  or  patent  ductus  arteriosus  (PDA)  necessary  for  survival  after  first  few  hours  of  birth•  RV  and  tricuspid  annulus  typically  small•  RV:  Coronary  artery  fistulas  and  coronary  artery  stenoses  are  common•  Some  coronary  flow  dependent  on  increased  pressures  in  RV  
SN  - 
PB  - The McGraw-Hill Companies
CY  - New York, NY
Y2  - 2024/04/19
UR  - accesssurgery.mhmedical.com/content.aspx?aid=58098908
ER  -