Cardiac Embryology and congenital heart disease III Flashcards
Define
tetralogy of Fallot
cyanotic heart disease
1) RV outflow tract obstruction
2) RVH
3) dextraposition of aorta (aorta overrides VSD)
4) VSD
monology of Fallot
anterior and superior deviation of infundibular portion of ventricular septum
most common cyanotic defect
tetralogy of fallot
physiology of Tetralogy of fallot
1) VSD is large so RV and LV pressures equal)
magnitude of pulmonary blood flow in tetralogy of fallot determined by (4) things
1) source of pulm blood flow
2) severity of RV outflow obstruction
3) balanced RV and LV pressure
4) ductus arteriosus
source of pulm blood flow
1) antegrade RV output to pulm arteries
2) ducts arteriosus flow
if outflow obstruction is severe, most PBF derived from ___
ductus arteriosus
size of ducts primary determinant of PBF magnitude
What happens in RV outflow obstruction
1) narrowing of infundibular region
2) stenosis of pulm valve
what determines R–> L shunt with RV outflow obstruction
if RV outflow resistance HIGHER THAN SYSTEMIC VASCULAR RESISTANCE –> cyanosis
what determines L –> R shunt with RV outflow obstruction
if RV outflow resistance LESS THAN SYSTEMIC VASCULAR RESISTANCE –> no cyanosis
which shunt has cyanosis
R–> L shunt
which shunt occurs if RV outflow resistance greater than systemic vascular resistance
R–> L shunt
what are tet spells
hypoxic or hypercyanotic spells
when do tet spells occur
2-6 y/o
Different mechanisms of Tet spells
1) infundibular spasm –> decr PBF
2) decr venous return to heart –> decr RV pressure –> decr PBF
3) decr SV –> incr R to L shunt –> decr PBF
what can precipitate tet spells
1) prolonged crying
2 anemia
3) dehydration
exam of tet spells
1) BLUE
2) decr murmur intensity
3) altered consciousness/seizures
treatment of tet spells
INCREASE PULM BLOOD FLOW
1) knee chest position
2) phenylephrine
3) morphine for sedation
4) volume expansion with IV fluids
prevention of tet spells
beta blockers (propranolol) –> decr infundibular obstruction
what does clinical presentation of tetralogy of fallot depend on (3)
1) size of VSD
2) severity of RV outflow obstruction
3) SVR level
how does tetralogy of fallot present
BLUE BABY WITH LOUD MURMUR
HOW CAN CYANOSIS IN tetralogy of fallot worsen?
as ductus arteriosus closes
if a patient with tetralogy of fallot had severe RV outflow tract obstruction how do you treat
pt may have ductal dependent pulm blood flow
need prostaglandins or early surgical repair
how to diagnosis tetralogy of fallot
1) tachycardic and cyanotic if blue tet
2) diaphoretic and tachypneic if pink tet
3) precordial impulse displaced to left lower sternal border –> RV dominance
murmur of tetralogy of fallot
2-3/6 short systolic murmur of pulmonary stenosis
ecg of tetralogy of fallot
Right axis deviation
RVH
prevention of tetralogy of fallot
elective surgical repair at 2-4 month before tet spell risk inca