Cyanotic heart defects Flashcards

1
Q

what are the respiratory causes of desaturation?

A

V/Q mismatch
obstruction
weakness of respiratory muscles

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2
Q

how do you augment pulmonary blood flow?

A

keep the PDA open

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3
Q

what is the use of the hyperoxitest?

A

to determine if cause of cyanosis is due to the heart or lungs

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4
Q

what is the role of PGE-1?

A

maintain patency of ductus arteriosus

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5
Q

what are the five Ts of cyanotic heart disease?

A
truncus arteriosus (1) 
transposition of the great arteries (2) 
tricuspid atresia (3) 
tetralogy of fallot (4) 
total anomalous pulmonary venous return (5)
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6
Q

what are the PE findings (2) in D-transposition?

A
  1. cyanotic full term male in no apparent distress

2. S2 is single and loud because aorta is anterior to PA

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7
Q

what test confirms the anatomy in D-transposition?

A

echo

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8
Q

what is the medical management for D-transposition (5)?

A
  1. PG
  2. correct acidosis
  3. oxygen
  4. balloon atrial septostomy
  5. anti-congestive
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9
Q

what is the rashkind procedure used for? how does it work?

A

D-transposition

special catheter advanced into heart via umbilical or femoral vein and advanced across restrictive patent foramen ovale - balloon is ripped back into RA

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10
Q

what are the four anomalies of tetralogy of fallot?

A
  1. large VSD
  2. overriding aorta
  3. RV outflow obstruction (sub-pulmonary / pulmonary stenosis)
  4. RV hypertrophy
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11
Q

what is the pathophysiology of tetralogy of fallot (3)?

A
  1. RV outflow obstruction limits pulmonary blood flow
  2. right to left shunt across VSD into aorta
  3. degree of cyanosis depends a lot on the degree of RV outflow obstruction
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12
Q

what is present at birth in children with tetralogy of fallot?

A
cyanosis 
murmur (RV outflow obstruction - not from VSD)
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13
Q

what are the EKG findings in tetralogy of fallot?

A

RAD

RVH

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14
Q

what is the classic morphology of the heart on CXR in tetralogy of fallot?

A

boot shaped

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15
Q

what is the pathophysiology of tet spells?

A

spasm of RV outflow tract increases right to left shunt across VSD, leading to worsening cyanosis and acidosis

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16
Q

what is the anatomy (4) of tricuspid atresia?

A
  1. absent tricuspid valve
  2. RV is hypoplastic
  3. majority of cases have either pulmonary atresia or pulmonary stenosis
  4. small VSD is often present
17
Q

what is the pathophysiology of tricuspid atresia?

A
  1. RA blood must cross atrial septum (complete mixing in LA)
  2. pulmonary blood flow is usually limited
18
Q

what test is significant for tricuspid atresia? why?

A

EKG - shows left superior axis

19
Q

what is main source of pulmonary blood flow in tricuspid atresia?

A

ductus arteriosus

20
Q

what is the ultimate surgical goal for tricuspid atresia?

A

fontan procedure

21
Q

what is fontan circulation? what drives blood to lungs?

A

IVC and SVC directly to pulmonary arteries

systemic venous pressure

22
Q

what is the anatomy in truncus arteriosus (2)?

A
  1. only a single arterial trunk leaves the heart, giving rise to systemic, pulmonary, and coronary circulations
  2. large VSD is present directly below the arterial trunk
23
Q

what is the pathophysiology of truncus arteriosus (3)?

A
  1. both ventricles empty into great artery
  2. pulmonary over-circulation (PVR less than SVR)
  3. pressure overload to lungs
24
Q

what is the surgical management for truncus arteriosus?

A
  1. direct LV flow out the truncal valve via patch closure of VSD
  2. disconnect and attach to RV-PA conduit
25
Q

what is the pathophysiology (2) of total anomalous pulmonary venous return?

A
  1. complete mixing in RA

2. veins can be obstructed

26
Q

which test is helpful in evaluating for obstruction in total anomalous pulmonary venous return?

A

CXR