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CV Final Material > Congenital Heart Disease (Wednesday) > Flashcards

Congenital Heart Disease (Wednesday) Flashcards

1
Q

What is congenital heart disease?

A

abnormalities of the heart and/or great vessels present from birth

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2
Q

What is the incidence of CHD?

A

6-8 per 1,000 liveborn term infants

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3
Q

In general, what is the outcome of CHD?

A

Can result in stillbirth, present at birth, or remain undetected until later life

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4
Q

What about detection and treatment of CHD?

A

In the 21st century, these problems are detected earlier (fetal ultrasound) and treated better (surgical advances), but cause more chronic disease (patients live longer).

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5
Q

When does most CHD develop?

A

Most heart anomalies arise during weeks 3-8 of embryogenesis

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6
Q

What are the causes of CHD?

A 
– Genetic 
   • Trisomies 13, 15, 18, 21
   • Turner syndrome (45,XO)
   • Monogenic disorders (next slide)
– Environmental
– Idiopathic (90%)
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7
Q

What is a shunt?

A

an abnormal communication between chambers or blood vessels can be structural or functional and in the context of CHD, classified as right to left or left to right

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8
Q

Which specific CHD cause left to right shunting (late cyanosis)?

A

ASD
VSD
AVSD
PDA

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9
Q

Which specific CHD cause obstruction (acyanotic)?

A

Pulmonary stenosis
Aortic stenosis
Coarctation

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10
Q

Which specific CHD cause right to left shunting (early cyanosis)?

A 
TOF
Transposition
Truncus
TV Atresia
TAPVR
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11
Q

Which specific CHD causes valvular regurgitation?

A

Ebstein

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12
Q

What is a left to right shunt and does it stay that direction?

A

Initially, L-R shunts result in oxygenated blood flowing into right-sided circulation (no cyanosis). However, this increases pulmonary blood flow
beyond its designed capacity results in pulmonary hypertension and right
ventricular hypertrophy. Increased right-sided pressure reverses the blood flow and it becomes a R-L shunt causing late cyanosis.

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13
Q

Is increased pulmonary blood flow well tolerated?

A

Well-tolerated by pulmonary vessels

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14
Q

Is increased pulmonary blood pressure well tolerated?

A

Not well-tolerated by pulmonary vessels

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15
Q

What is Plexogenic pulmonary hypertension?

A

Medial hypertrophy, Intimal proliferation, Plexiform lesions (irreversible damage) occurs with VSD > PDA&raquo_space; ASD

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16
Q

What anomalies cause L-R shunts?

A

– Atrial septal defect (ASD)
– Ventricular septal defect (VSD)
– Patent ductus arteriosus (PDA)
– Atrioventricular septal defect (AVSD)

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17
Q

What is an atrial septal defect (ASD)?

A

Interatrial opening, present throughout cardiac cycle

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18
Q

Where do ASD occur?

A

– Secundum: at fossa ovalis (90%)
– Primum: adjacent to AV valves (5%)
– Sinus venosus: near SVC entrance (5%)

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19
Q

What are some other features of ASD?

A

– May be asymptomatic until adulthood
– Can allow paradoxical embolism
– <10% lead to pulmonary hypertension

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20
Q

ASD vs patent foramen ovale (PFO)

A

ASD: rare, deficient fossa ovale, L-R shunt, potential pulmonary HTN and right sided failure
PFO: 1/3 of people have it, small remnant opening, no shunt, potential paradoxical emboli, decompression sickness and migraines

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21
Q

ASD long term effects include?

A

pulmonary hypertension, reversal of shunt, right heart failure, right ventricle hypertrophy, right atria/ventricle dilation

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22
Q

What is ventricular septal defect (VSD)?

A

-Interventricular opening between LV and RV
-Most common congenital
heart anomaly

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23
Q

Where does VSD occur?

A

90% at septum (membranous VSD)

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24
Q

Characteristics of perimembranous VSD?

A

Defect is usually large
– Spontaneous closure by septal TV leaflet occurs in <10% of cases
– Requires surgical closure, usually around 1 year of age

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25
Q

Characteristics of muscular VSD?

A

– Defect is usually small
– Spontaneous closure by fibrous adhesions occurs in >60% of cases by 1 year of age
– Most do not need surgery
– Multiple muscular VSDs = “swiss cheese septum”

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26
Q

Normal closure of patent ductus arteriosus?

A

– Functional (~12 h)
– Structural (~3 mo)
– Delayed by prostaglandin E
– Closes later in preemies and at high altitude

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27
Q

Classic exam findings of PDA?

A

Harsh, continuous, “machinery-like” murmur

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28
Q

Other features of PDA?

A

– Usually seen in isolation (90%)

– Necessary for survival in AV or PV atresia, others

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29
Q

Direction of PDA shunting normal and with pulmonary HTN?

A

normal is left to right

pulmonary HTN is right to left

30
Q

What is atrioventricular septal defect?

A

– Deficient AV septum, associated with MV and TV anomalies

31
Q

What are the two types of AVSD?

A

– Partial: primum ASD & cleft MV with MR

– Complete: AVSD & common AV valve (5 leaflets)

32
Q

What are other features of AVSD?

A

– Down syndrome (40%) with complete AVS

– Needs early surgical correction

33
Q

How do right to left shunts present?

A

R-L shunts dump deoxygenated blood into the systemic (left-sided) circulation, decreasing pulmonary blood flow and causing early cyanosis, digital clubbing, and polycythemia.

34
Q

Anomalies causing R-L shunting?

A 
– Tetralogy of Fallot
– Transposition of the great arteries
– Truncus arteriosus
– Tricuspid atresia
– Total anomalous pulmonary venous connection
35
Q

What is the most common form of cyanotic congenital

heart disease?

A

Tetralogy of fallot

36
Q

What is Tetralogy of fallot?

A 
Anteriosuperior displacement of the infundibular septum leads to
– Ventricular septal defect
– Subpulmonary stenosis
– Overriding aorta
– Right ventricular hypertrophy
37
Q

Clinical outcome of TOF depends on what?

A

depends on severity of subpulmonary stenosis

38
Q

How does TOF appear on xray?

A

Heart may appear boot-shaped due to right ventricular hypertrophy

39
Q

What kind of shunting occurs with TOF?

A

R-L shunt does not damage lungs because subpulmonary stenosis restricts pulmonary blood flow

40
Q

Does TOF need surgery?

A

Pulmonary outlet does not grow with child, so effects are worse with age = Surgery required

41
Q

What is transposition of the great arteries?

A

Aorta arises from RV & Pulmonary artery arises from LV

42
Q

What does transposition of the great arteries mean for circulation?

A

– Aorta lies anterior and to the right of the pulmonary artery
– Separate pulmonary and systemic circulations
– Right ventricular hypertrophy develops
– Pulmonary hypertension develops unless pulmonary stenosis is present

43
Q

There are two types of transposition of the great arteries what are they?

A

– Intact ventricular septum (65%): unstable, needs prompt surgical intervention
– With VSD (35%): stable

44
Q

What is truncus arteriosus?

A

– Origin of aorta & pulmonary artery from truncal artery

– Most have large VSD

45
Q

What is the etiology of truncus arteriosus?

A

Developmental failure of separation of the embryologic truncus into the aorta and pulmonary artery.

46
Q

What does truncus arteriosus do to the circulation?

A

Mixing of blood with Increased pulmonary blood flow & pulmonary HTN

47
Q

Truncus arteriosus can be associated with what syndrome?

A

DiGeorge syndrome

48
Q

What is tricuspid atresia?

A

Complete occlusion of the tricuspid valve orifice that results from unequal division of the AV canal – mitral valve is enlarged

49
Q

What other defect is needed in tricuspid atresia for survival?

A

Needs coexisting ASD/PFO and VSD

50
Q

What is Total anomalous pulmonary venous return?

A

Pulmonary veins do not directly drain into left atrium; left atrial hypoplasia. Instead, they connect via left innominate vein or coronary sinus with ASD/PFO allows oxygenated blood to enter systemic circulation

51
Q

How does Total anomalous pulmonary venous return happen?

A

Occurs when common pulmonary vein fails to develop or regresses

52
Q

What are the specific obstructive forms of CHD?

A
  • Aortic coarctation
  • Pulmonary stenosis / atresia
  • Aortic stenosis / atresia
53
Q

What is Aortic coarctation?

A

Constriction/narrowing of aorta

54
Q

What are the two types of aortic coarctation?

A

– Preductal/infantile: Tubular hypoplasia with PDA

– Postductal/adult: Ridgelike infolding at ligament without PDA

55
Q

What is the clinical presentation of aortic coarctation?

A

– Bicuspid AV (50%)
– Preductal:Lower body cyanosis & Requires surgery in neonatal period
– Postductal: Symptoms depend on degree of narrowing, Surgically treatable HTN, upper > lower & Rib notching (CXR)

56
Q

Can blood get around an aortic coarctation?

A

Some gets through and anastamoses function to help try to bring enough blood past

57
Q

What is pulmonary stenosis?

A

Pulmonary valve obstruction due to hypoplasia, dysplasia, or abnormal number of cusps

58
Q

What if there is isolated pulmonary stenosis?

A

RV dilatation & hypertrophy, Post-stenotic injury to PA & May be asymptomatic until adulthood

59
Q

What if there is pulmonary valve atresia with intact VS?

A

– Hypoplastic RV and TV

– PDA needed to get blood to lungs

60
Q

What long term changes occur with pulmonary stenosis?

A

Pulmonary artery dilatation, RV hypertrophy and RA dilatation

61
Q

What is aortic stenosis?

A

Aortic valve obstruction due to hypoplasia, dysplasia, or abnormal number of cusps

62
Q

What points to isolated AV stenosis?

A

occurs 80% of the time
– LV hypertrophy and LA dilatation
– AS may range from mild to critical
– Systolic murmur

63
Q

What is AS – hypoplastic left heart syndrome?

A

Aortic valve atresia with intact VS
– Hypoplastic mitral valve and left ventricle
– Dependent on PDA for survival
– Requires staged surgical correction

64
Q

What is ebstein anomaly of tricuspid valve?

A

– Inferiorly displaced and adherent septal and posterior leaflets
– Redundant anterior leaflet
– Dilated annulus with TR

65
Q

What are the secondary effects of ebstein anomaly of the tricuspid valve?

A
  • Secondary effects – RV and RA dilatation

* Other features– Arrhythmias, including WPW syndrome or may be asymptomatic until adulthood

66
Q

The treatment of CHD is usually what?

A

primarily by surgical repair and/or transplantation

67
Q

What are the risks of surgical repair of CHD?

A

bleeding, infection, death, stroke, long term arteriosclerosis and fibrosis

68
Q

What are the risks of transplant repair of CHD?

A

same as surgery, plus rejection, infection, post-transplant lymphoproliferative disease, other malignancies

69
Q

What are some late effects of CHD?

A
  • Endocarditis
  • Hyperviscosity
  • Pulmonary hypertension and shunt reversal (Eisenmeiger complex)
  • Childbearing risk
  • Residual post-surgical pathology
70
Q

What is the most common CHD?

A

VSD

71
Q

What is the least worrisome CHD?

A

ASD

CV Final Material (16 decks)

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