Thursday, May 16, 2013

Congenital cyanotic heart disease

congenital cyanotic heart disease

DDx - five "Ts"



90% of all  congenital heart defects are ToF (75%) or TGA (15%)









Treatment
TGA
PGE1 - keep PDA open
Fluids - needs volume for continued operation of atrial septal defect
Do not use oxygen -  want to keep pulmonary vascular resistance high
(+/- BAS)
Surgery: Switch

ToF

PGE1
+/- Oxygen
Fluids
Chromosomes - deGeorge syndrome
*Irradiate & wash blood*  -  kill white cells to avoid graph vs. host reaction

Surgery - Shunt or Repair

Total Anomolous Pulmonary Vascular Connection (TAPVC)


When should you NOT start with PGE1?  NEVER
Concerns re: PGE1
  • Apnea
  • Fever
  • PVR
  • O2 effect

PGE1 Dosing & PVR
PGE1 in obstructed TAPVC
PGE1 in TGA…& lasix. NEEDS FLUIDS


PDA



PDA Notes
#1 Determining factor – intervene or not – is…
Clinical trend line

“Severe” PAH ≠≠ elevated PVR  (REMEMBER P = Q x R)   inc.  P due to increased Q

Indomethacin - opposite of PGE1 = closes PDA (but side effects - close renal flow)
Indometacin (INN) or indomethacin (USAN and former BAN) is a non-steroidal anti-inflammatory drug(NSAID) commonly used as a prescription medication to reduce feverpain, stiffness, and swelling. It works by inhibiting the production of prostaglandins, molecules known to cause these symptoms




CHF = congestive heart failure: clinical 
manifestations of inadequate cardiac output.




Differential Diagnosis
Primary




Myocardial
Rhythm
Blood 



Secondary



Shunt*
Obstruction
Both
A-V valve regurgitation










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