Cardiology 47, 48 Flashcards

1
Q

When to suspect CHD? congenital heart defect

A
  • Positive family history
  • chromosome abnormalities
  • heart murmur
  • failure to thrive
  • abnormal skin color
  • tachypnea/dyspnea
  • easily exhausted
  • pronounces sweating during feeding
  • active precordial pulsations
  • abnormal peripheral pulses
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2
Q

Prenatal diagnosis of congenital heart defect

A
  • General screening by obstetrician in every trimester
  • Fetal echocardiography by pediatric cardiologist
    (if suspicious finding,
    developmental abnormality,
    multiple pregnancies,
    family history of CHD,
    >40 maternal age, maternal IDDM)
    Should be done week 18-22 so that termination of pregnancy is still an option
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3
Q

when should fetal ECHO be performed and why

A

Should be done week 18-22 so that termination of pregnancy is still an option

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4
Q

newborn diagnosis in congenital heart defect

A

Pulse oximetry screening

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5
Q

Classification of CHD:

A

Cyanotic heart defects

Non-cyanotic heart defect

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6
Q

Cyanotic congenital heart defects
types

A
  • Right-to-left shunt:
    *Tetralogy of Fallot
  • Separate circulations:
    *Transposition of the great arteries
  • Complete mixing:
    *Tricuspid atresia
    *Truncus arteriosus
    *Hypoplastic left heart syndrome
    *TAPVR
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7
Q

right to left shunt cyanotic heart defect

A

Tetralogy of Fallot

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8
Q

Complete mixing type of cyanotic heart defect

A

Tricuspid atresia
Truncus arteriosus
Hypoplastic left heart syndrome
TAPVR

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9
Q

Tetralogy of fallot characterized by

A

1) pulmonary stenosis
2) VSD
3) misplaced aorta
4) right ventricular hypertrophy

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10
Q

what disease have seperate circulations in cyanotic heart defect

A

transposition of great vessel

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11
Q

Tetralogy of fallot results in which type of shunt

A

Right to left shunt

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12
Q

Tetralogy of fallot what determine extent of shunting

A

Degree of stenosis determines extent of shunting + cyanosis

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13
Q

cyanosis

A

bluish color of the skin and mucus membranes, arterial hypoxemia (arterial sat of < 95%)

Perioral (around mouth) cyanosis or acrocyanosis (hands/feet) —> NOT a sign of cyanotic heart defect, but a common physiological sign of cold/decreased circulation

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14
Q

clinical features of tetralogy of fallot

A
  • Boot-shaped heart on x-ray
  • Patients learn to squat in response to cyanosis —> increase systemic resistance —> more blood flow through stenotic pulmonary arteries to lungs
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15
Q

tetralogy of fallot treatment

A

surgery

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16
Q

Transposition of the great vessels characterized by

A

Characterized by
pulmonary a. arising from LV
+ aorta arising from RV

Associated with maternal diabetes

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17
Q

Clinical features of transposition of the great vessels

A

Early cyanosis
—> pulmonary + systemic circuits do not mix

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18
Q

treatment of transposition of the great vessel

A

Creation of shunt (ASD) —> required for survival

PGE administered to maintain PDA until surgery

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19
Q

cyanotic heart defects

A
  • Tetralogy of fallot
  • Transposition of the great vessels
  • Tricuspid atresia
  • Truncus arteriosus
  • Hypoplastic left heart syndrome
  • Total anomalous pulmonary venous return (TAPVR)
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20
Q

Non-cyanotic congenital heart defects

A

Left-to-right- shunt: ASD, VSD, AVSD, PDA

Obstructive lesions: AS, PS, CoA

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21
Q

Tricuspid atresia

A
  • Absent or rudimentary tricuspid valve resulting in no blood flow between RA and RV
  • Patient survival is only possible if there are interatrial or interventricular communications (ASD, VSD)
  • Accompanied by RV hypoplasia and RA dilation due to volume overload
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22
Q

clinical features of tricuspid atresia

A

central cyanosis
holosystolic murmur at lower left sternal border

diminished peripheral pulses

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23
Q

treatment of tricuspid atresia

A

surgery

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24
Q

Truncus arteriosus

A
  • Underdevelopment of aorticopulmonary septum
    —> failure of truncus arteriosus to divide into the aorta and pulmonary trunk
    —> instead a single trunk that receives output from both ventricles

*Truncus arteriosus:
single arterial trunk that originates from both ventricles of the embryonic heart and give rise to ascending aorta and pulmonary trunk

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25
Q

*Truncus arteriosus:

A

single arterial trunk that originates from both ventricles of the embryonic heart and give rise to ascending aorta and pulmonary trunk

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26
Q

truncus arteriosus is associated with what disease

A

DiGeorge syndrome

27
Q

Truncus arteriosus
effect of oxygenated blood

A

Results in the mixture of oxygenated and deoxygenated blood

28
Q

clinical features of truncus arteriosus

A

cyanosis
respiratory distress
failure to thrive,
harsh systolic murmur
bounding peripheral pulses

29
Q

truncus arteriosus treatment

A

surgery

30
Q

Hypoplastic left heart syndrome

A

Spectrum of disease consisting of severe hypoplasia of left ventricle with possible

and/or atresia of the mitral valve, aortic valve, or aortic arch

31
Q

Hypoplastic left heart syndrome associated with which diseases

A

Associated with
Patau syndrome,
Edward’s syndrome,
Turner syndrome

32
Q

prognosis and treatment of hypoplastic left heart syndrome

A

bad prognosis
treatment surgery

33
Q

Total anomalous pulmonary venous return (TAPVR)

A

All four pulmonary veins drain into systemic venous circulation instead of left ventricle
—> oxygenated blood returns back to right atrium —> pulmonary edema

34
Q

clinical features of Total anomalous pulmonary venous return (TAPVR)

A

cyanosis,
respiratory failure,
failure to thrive,
hepatomegaly

35
Q

Total anomalous pulmonary venous return (TAPVR) treatment

A

surgery

36
Q

Non-cyanotic congenital heart defects
Types* :

A

Left-to-right- shunt: ASD, VSD, AVSD, PDA

Obstructive lesions: AS, PS, CoA

37
Q

Atrial septal defect (ASD)

A

Defect in septum btw atria

38
Q

ASD associated with

A

Down syndrome, fetal alcohol syndrome

39
Q

ASD results in what shunt

A

left to right shunt

40
Q

auscultation of ASD

A

split S2 (increased blood in right heart —> delays closure of pulmonary valve
—> 2 sounds

41
Q

Paradoxical emboli:

A

emboli produced in systemic circulation normally go to lungs but can now travel to left heart —> brain)

associated with ASD

42
Q

Most common congenital defect of heart

A

Ventricular septal defect (VSD)

43
Q

VSD associated with

A

Down syndrome,
intrauterine infections (TORCH),
maternal diabetes

44
Q

Ventricular septal defect (VSD) what shunt

A

Results in left-to-right shunt
—> dilation of LV since increased preload (blood goes through shunt instead of the periphery —> RV —> lungs —> back to LV)

45
Q

Size of shunt determines

A

extent of shunting
+ age of presentation

46
Q

Small VSD defect

A

—> asymptomatic

47
Q

Large VSD defect —>

A

can lead to Eisenmenger syndrome (reversal of shunt)

48
Q

treatment of VSD

A

loop diuretics
ACEi,
surgery

49
Q

Atrioventricular septal defect (AVSD) associated with

A

down syndrome

50
Q

AVSD shunt type

A

Defect in septum btw atria and ventricles of right and left heart

Results in left-to-right shunt —> dilation of heart —> HF + pulmonary HTN

treatment: surgery

51
Q

Patent ductus arteriosus (PDA)

A

ure of ductus arteriosus (connects aorta + pulmonary a.) to close

52
Q

Patent ductus arteriosus (PDA) associated with

A

Associated with congenital rubella

53
Q

PDA shunt type

A

Results in left-to-right shunt *

  • In utero DA shunts blood in other direction (right-to-left) to bypass lungs
  • Systemic circulation increases after birth
  • If DA stays open —> blood shunt the other direction (left-to-right)
54
Q

If DA stays open —>

A

blood shunt the other direction (left-to-right)

55
Q

in utero Ductus arteriosus function

A

In utero DA shunts blood in other direction (right-to-left) to bypass lungs

56
Q

PDA has hemodynamically similar effects as

A

vsd

57
Q

complication of PDA

A

Eisenmenger syndrome with increasing pulmonary pressure

58
Q

treatment of PDA

A

indomethacin —> decrease PGE —> close PDA (kept open in utero by placental PGE)

59
Q

Coarctation of aorta (CoA)

A

Narrowing of aorta
* infantile form
* adult form

60
Q

Coarctation of aorta (CoA)- infantile form

A

Associated with PDA
* Coarctation lies distal to aortic arch but before PDA

  • Presents as lower extremity cyanosis
61
Q

Adult form CoA

A
  • Coarctation lies distal to aortic arch ‣
  • Presents as hypertension in upper extremities + hypotension in lower extremities
  • Not associated with PDA
62
Q

which form of CoA is associated with PDA

A

infantile form
Coarctation lies distal to aortic arch but before PDA
Presents as lower extremity cyanosis

63
Q

coarctation position in infantile vs adult form of CoA

A

Coarctation lies distal to aortic arch but before PDA –> lower extremity cyanosis

Coarctation lies distal to aortic arch ‣
Presents as hypertension in upper extremities + hypotension in lower extremities

64
Q

what is used for diagnosis of CoA

A

4 limb pulsoximetry:
crucial to check both upper (brachial) and lower (femoral) extremity pulses!
—> if decreased in legs: possible coarctation that need to be double checked with saturation and blood pressure