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

Congenital Heart Disease (Wednesday) Flashcards

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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
Characteristics of muscular VSD?
– 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”
26
Normal closure of patent ductus arteriosus?
– Functional (~12 h) – Structural (~3 mo) – Delayed by prostaglandin E – Closes later in preemies and at high altitude
27
Classic exam findings of PDA?
Harsh, continuous, “machinery-like” murmur
28
Other features of PDA?
– Usually seen in isolation (90%) | – Necessary for survival in AV or PV atresia, others
29
Direction of PDA shunting normal and with pulmonary HTN?
normal is left to right | pulmonary HTN is right to left
30
What is atrioventricular septal defect?
– Deficient AV septum, associated with MV and TV anomalies
31
What are the two types of AVSD?
– Partial: primum ASD & cleft MV with MR | – Complete: AVSD & common AV valve (5 leaflets)
32
What are other features of AVSD?
– Down syndrome (40%) with complete AVS | – Needs early surgical correction
33
How do right to left shunts present?
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
Anomalies causing R-L shunting?
``` – Tetralogy of Fallot – Transposition of the great arteries – Truncus arteriosus – Tricuspid atresia – Total anomalous pulmonary venous connection ```
35
What is the most common form of cyanotic congenital | heart disease?
Tetralogy of fallot
36
What is Tetralogy of fallot?
``` Anteriosuperior displacement of the infundibular septum leads to – Ventricular septal defect – Subpulmonary stenosis – Overriding aorta – Right ventricular hypertrophy ```
37
Clinical outcome of TOF depends on what?
depends on severity of subpulmonary stenosis
38
How does TOF appear on xray?
Heart may appear boot-shaped due to right ventricular hypertrophy
39
What kind of shunting occurs with TOF?
R-L shunt does not damage lungs because subpulmonary stenosis restricts pulmonary blood flow
40
Does TOF need surgery?
Pulmonary outlet does not grow with child, so effects are worse with age = Surgery required
41
What is transposition of the great arteries?
Aorta arises from RV & Pulmonary artery arises from LV
42
What does transposition of the great arteries mean for circulation?
– 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
There are two types of transposition of the great arteries what are they?
– Intact ventricular septum (65%): unstable, needs prompt surgical intervention – With VSD (35%): stable
44
What is truncus arteriosus?
– Origin of aorta & pulmonary artery from truncal artery | – Most have large VSD
45
What is the etiology of truncus arteriosus?
Developmental failure of separation of the embryologic truncus into the aorta and pulmonary artery.
46
What does truncus arteriosus do to the circulation?
Mixing of blood with Increased pulmonary blood flow & pulmonary HTN
47
Truncus arteriosus can be associated with what syndrome?
DiGeorge syndrome
48
What is tricuspid atresia?
Complete occlusion of the tricuspid valve orifice that results from unequal division of the AV canal – mitral valve is enlarged
49
What other defect is needed in tricuspid atresia for survival?
Needs coexisting ASD/PFO and VSD
50
What is Total anomalous pulmonary venous return?
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
How does Total anomalous pulmonary venous return happen?
Occurs when common pulmonary vein fails to develop or regresses
52
What are the specific obstructive forms of CHD?
* Aortic coarctation * Pulmonary stenosis / atresia * Aortic stenosis / atresia
53
What is Aortic coarctation?
Constriction/narrowing of aorta
54
What are the two types of aortic coarctation?
– Preductal/infantile: Tubular hypoplasia with PDA | – Postductal/adult: Ridgelike infolding at ligament without PDA
55
What is the clinical presentation of aortic coarctation?
– 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
Can blood get around an aortic coarctation?
Some gets through and anastamoses function to help try to bring enough blood past
57
What is pulmonary stenosis?
Pulmonary valve obstruction due to hypoplasia, dysplasia, or abnormal number of cusps
58
What if there is isolated pulmonary stenosis?
RV dilatation & hypertrophy, Post-stenotic injury to PA & May be asymptomatic until adulthood
59
What if there is pulmonary valve atresia with intact VS?
– Hypoplastic RV and TV | – PDA needed to get blood to lungs
60
What long term changes occur with pulmonary stenosis?
Pulmonary artery dilatation, RV hypertrophy and RA dilatation
61
What is aortic stenosis?
Aortic valve obstruction due to hypoplasia, dysplasia, or abnormal number of cusps
62
What points to isolated AV stenosis?
occurs 80% of the time – LV hypertrophy and LA dilatation – AS may range from mild to critical – Systolic murmur
63
What is AS – hypoplastic left heart syndrome?
Aortic valve atresia with intact VS – Hypoplastic mitral valve and left ventricle – Dependent on PDA for survival – Requires staged surgical correction
64
What is ebstein anomaly of tricuspid valve?
– Inferiorly displaced and adherent septal and posterior leaflets – Redundant anterior leaflet – Dilated annulus with TR
65
What are the secondary effects of ebstein anomaly of the tricuspid valve?
* Secondary effects – RV and RA dilatation | * Other features– Arrhythmias, including WPW syndrome or may be asymptomatic until adulthood
66
The treatment of CHD is usually what?
primarily by surgical repair and/or transplantation
67
What are the risks of surgical repair of CHD?
bleeding, infection, death, stroke, long term arteriosclerosis and fibrosis
68
What are the risks of transplant repair of CHD?
same as surgery, plus rejection, infection, post-transplant lymphoproliferative disease, other malignancies
69
What are some late effects of CHD?
* Endocarditis * Hyperviscosity * Pulmonary hypertension and shunt reversal (Eisenmeiger complex) * Childbearing risk * Residual post-surgical pathology
70
What is the most common CHD?
VSD
71
What is the least worrisome CHD?
ASD

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