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NPS Kettering Workbook > Pathology: Congenital Heart Defects > Flashcards

Pathology: Congenital Heart Defects Flashcards

1
Q

Right to left shunts result in

A

Cyanosis (hypoxemia)

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2
Q

Left to right shunts result in

A

Acyanosis (pulmonary congestion)

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3
Q

4 specific defects in tetralogy of fallot

A

VSD
Overriding aorta
Pulmonary artery stenosis
R ventricular hypertrophy

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4
Q

How is blood shunted in tetralogy of fallot?

A

R ventricle -> large VSD -> overriding aorta

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5
Q

TOF: what causes profound cyanosis?

A

Closing of PDA

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6
Q

How does TOF appear on CXR?

A

Boot-shaped

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7
Q

What causes “tet” spells

A

Coughing, crying, BM

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8
Q

What confirms dx of TOF?

A

Echo

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9
Q

Treatment/management of TOF

A

Supportive care/O2 prn
Prostaglandin E1
Surgery

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10
Q

What do prostaglandins do

A

Maintain PDA

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11
Q

Anatomical changes in transposition of great vessels (TGV)

A

Reversal of the origin of the aorta and the pulmonary artery, R ventricle -> aorta and L ventricle -> pulmonary artery

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12
Q

TGV: venous and arterial blood mixes through

A

ASD
VSD
PDA

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13
Q

TGV: when does cyanosis and hypoxia occur?

A

During first week of life as PDA closes

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14
Q

TGV: CXR

A

Egg-shaped

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15
Q

TGV: confirmation

A

Echo

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16
Q

TGV: treatment

A

Prostaglandins
Supportive care, O2 prn (decrease FiO2 if baby gets worse)
Balloon arterial septostomy (rashkind procedure) to create shunt for mixing
Surgery

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17
Q

What is coarctation of the aorta?

A

Severe narrowing of the aorta, causing decreased blood flow through aorta

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18
Q

Preductal coarctation of aorta

A

Narrowing occurs before PDA, results in R to L shunt

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19
Q

Postductal coarctation of aorta

A

Narrowing occurs after PDA, results in L to R shunt

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20
Q

Why is measuring BP in all four extremities when coarctation of aorta is suspected?

A

BP is higher in upper extremities

21
Q

Coarctation of aorta: treatment

A

Supportive care with digitalis for heart failure
Prostaglandins
Surgery for resection of coarctation

22
Q

Anatomical changes in truncus arteriosus

A

Single common vessel for both the aorta and pulmonary artery over VSD

23
Q

Blood flow in truncus arteriosus

A

Systemic and pulmonary circulations delivered through same ventricle

24
Q

Truncus arteriosus treatment

A

Be conservative with oxygen admin to keep PVR high
Rule of fourties’
Pulmonary artery banding
VSD closure
Surgery

25
Q

What is the rules of 40’s?

A

PaCO2: 40
PaO2: 40
SaO2: 75%
Balances systemic and pulmonary flow

26
Q

Anatomical changes in hypoplastic left heart syndrome

A

Severely underdeveloped L ventricle with stenosis or atresia of mitral valve
Dependent upon right ventricle for blood supply to both circulations

27
Q

Techniques to balance pulmonary and systemic vascular resistance in hypoplastic left heart syndrome

A

Withhold oxygen therapy, subambient O2 (17-20%)
Target SpO2: 70-80%
Rule of 40’s

28
Q

Hypoplastic left heart syndrome: treatment

A

Prostaglandins
Subambient FiO2
Hypercarbic gas mixtures
Complex surgical repair

29
Q

Total anomalous pulmonary venous return (TAPVR) anatomical changes

A

Pulmonary veins fail to connect to left atria, may connect to superior or inferior vena cava

30
Q

TAPVR: what two things are crucial for survival?

A

ASD
PDA

31
Q

TAPVR: CXR

A

Snowman shaped heart

32
Q

TAPVR: treatment

A

Prostaglandins
Balloon septostomy (ASD)
Surgery

33
Q

atrial septal defect: anatomical changes

A

Failure of the foremen ovale to close

34
Q

Cause of R heart failure in ASD

A

Increased workload of R heart

35
Q

ASD: presentation in newborn

A

Initially healthy, failure to thrive
Difficulty feeding
Turns blue after crying, feeding, burping, etc (increased intrathoracic pressure)

36
Q

ASD: treatment

A

Surgery
Supportive oxygen prn

37
Q

ventricular septal defect: anatomical changes

A

Opening in septum between ventricles (most common)

38
Q

How are small VSDs treated?

A

Spontaneous
Oxygen
Diuretics
Digoxin

39
Q

How are large VSDs treated?

A

Surgery to patch
pulmonary artery banding

40
Q

Why does blood shunt from the aorta into the pulmonary circulation (PDA)

A

Left to right, if left heart pressures are higher than right heart pressures

41
Q

How is a pt evaluated for a PDA?

A

Pre- and post-ductal studies (PaO2 10-15 higher in right hand vs left) (15% if SpO2)

42
Q

How is a newborn with a PDA treated?

A

Indomethacin (indocin) or ibuprofen (prostaglandin inhibitors)
O2 therapy
Fluid restriction and diuretics
Surgery

43
Q

Atrioventricular septal defect (ASVD) other terms

A

Atrial-ventricular canal or endocardial cushion defect

44
Q

ASVD: anatomical changes

A

Absence of atrial and ventricular septum

45
Q

ASVD results in

A

Intra-atrial and intra-ventricular shunting, and atrio-ventricular regurgitation

46
Q

ASVD most common in

A

Trisomy 21 (down syndrome)

47
Q

ASVD: treatment

A

Children without CHF: diuretics and digoxin
SpO2 75-90%
Careful oxygen administration
Surgery

48
Q

ASVD: higher SpO2 and oxygen results in

A

Reduced PVR and increased pulmonary blood flow (bad)

NPS Kettering Workbook (8 decks)

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