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Block 9 Week 1 > Congenital Heart Disease-Fung > Flashcards

Congenital Heart Disease-Fung Flashcards

1
Q

General term used to describe abnormalities of the heart and great vessels that are present from birth
Arise due to faulty embryogenesis during week (blank) of gestation

A

Congenital heart disease

3 and 8

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2
Q

1% of births have a (blank) defect and are the most common form of cardiovascular disease among children

A

congenital cardiovascular

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3
Q

Describe fetal circulation?

A

oxygenated blood from placenta goes into the ductus venosus where it goes into the liver and enters the IVC. deoxygenated blood enters the SVC and joins the oxygenated blood of the IVC. This passes into the right ventricles and goes into the pulmonary artery. oxygenated blood returns to the left atrium into the left ventricle where it passes into the aorta and mixes with the deoxygenated blood from the ductus arteriosus and goes into the descending aorta where it will go into the umbilical arteries into the placenta

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4
Q

What are the sporadic genetic abnormalities that cause congenital heart disease?

A 
sporadic genetic abnormalities
-single gene mutations
-small chromosomal deletions
-trisomies/monosomies
(Turner syndrome (XO), Trisomy 13, Trisomy 18 (Edward syndrome), Trisomy 21 (Down's syndrome)
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5
Q

What are environmental factors that causes congenital heart diseases?

A
  • congenital rubella
  • gestational diabetes
  • teratogens
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6
Q

What is this:
abnormal communication between chambers or blood vessles
What is a complete obstruction?

A

Shunt

Atresia

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7
Q

What are the three primary categories of a congenital heart disease?

A

left to right shunt
right to left shunt
obstruction

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8
Q

In right to left shunts what happens to the blood?

How do patients present?

A
  • poorly oxygenated venous blood mixes with systemic arterial blood
  • hypoxemia and cyanosis (cyanotic congenital heart disease)
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9
Q

What are the associated clinical situations with a right to left shunt?

A
  • tetralogy of fallot
  • transposition of the GVs
  • Persistent truncus arteriosus
  • Tricuspid atresia
  • Total anomalous pulmonary venous connection
  • paradoxical embolism
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10
Q

What are the clinical features of right to left shunts?

A

Clubbing of toes and fingers (hypertrophic osteoarthropathy)

Polycythemia

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11
Q

What are the cardinal features of tetralogy of fallot?

Why does it happen?

A
  • VSD
  • Obstruction of the right ventricular outflow tract (subpulmonary stenosis)
  • Overriding aorta
  • Right ventricular hypertrophy

-results from the anterosuperior displacement of the infundibular septum

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12
Q

Can patients survive with tetrology of fallot?

What are the clinical consequences dependent on?

A

yes into adulthood even untreated

-degree of subpulmonic stenosis

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13
Q

What does a mild tetralogy of fallot look like?

A

resembles an isolated VSD and more like a left to right shunt without cyanosis (pink TOF)

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14
Q

What does a severe tetralogy of fallot look like?

A
  • greater resistance to RV outflow and a right to left shunt (classic TOF)
  • pulmonary arteries become hypoplastic and aortic dilation
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15
Q

What do most patients with TOF look like at birth? what should you do?

A

cyanotic

surgical repair

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16
Q

What is this:

aorta arises from the RV and the pulmonary artery arises from the LV

A

Transposition of the great vessels

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17
Q

Why does transposition of the great vessels develop?

A

due to abnormal formation of the truncal and aortopulmonary septa

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18
Q

What does the degree of transposition of the great vessels depend on?

A
  • Degree of mixing of blood (via shunt)
  • Magnitude of tissue hypoxia
  • The ability of the RV to maintain the systemic circulation
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19
Q

What is a stable shunt associated with 35% of cases of transposition of great vessels?
What are unstable shunts?
What do you do with patients with an unstable shunt?

A
  • VSD
  • Patent foramen ovale, ductus arteriosus
  • give them a balloon atrial septostomy
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20
Q

What are the clinical features of transposition of the great vessels?
Do most survive?

A

-Right ventricular hypertrophy
-Atrophic left ventricle
Without surgery most die within first months of life
With surgery can survive into adulthood

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21
Q

Why does persistent truncus arteriosus arise?

A

due to failure of separation of the embryological truncus arteriosus into the aorta and the pulmonary artery

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22
Q

What is this:
single great vessel that receives blood from both ventricles and coronary circulation.
What is associated with this and what does this cause?

A

persistent truncus arteriosus

VSD, produces systemic cyanosis and increased pulmonary blood flow (danger of irreversible pulmonary HTN)

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23
Q 
What is tricupsid atresia?
What does this result from?
How can the heart function with this?
What does the patient look like at birth?
Is this fatal?
A
  • complete obstruction of tricuspid valve orifice
  • unequal division of the AV canal resulting in an enlarged mitral valve, underdeveloped right ventricle
  • right to left shunt through the atria (ASD, patent foramen ovale) and a VSD allows communication b/w the left ventricle and pulmonary artery that arises from a hypoplastic right ventricle
  • cyanosis is present at birth
  • very high mortality rate
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24
Q

What is this:
pulmonary veins fail to directly join the left atrium due to the failure of the development (atresia) of the common pulmonary vein.
How does the heart compensate for this?

A

total anomalous pulmonary venous circulation

-a patent foramen ovale or ASD allows pulmonary venous blood to enter the atrium

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25
Q

What are the clinical features of total anomalous pulmonary venous circulation?

A
  • volume and pressure overload leads to hypertrophy and dilation of the right heart
  • dilation of the pulmonary trunk
  • hypoplastic left atrium
  • possible cyanosis due to the right to left shunt of the ASD
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26
Q

What do left to right shunts do?

A

increase pulmonary blood flow and volume

pulmonary circuit is normally low flow, low resistance

27
Q

In a left to right shunt you have increased pulmonary blood flow and volume, what is the first response to this? THen what happens?

A

increased blood flow from the shunt is medial hypertrophy and vasoconstriction to maintain distal pressures and prevent pulmonary edema.

  • > prolonged increased pulmonary vasoconstriction induces obstructive intimal lesions (hyaline and hyperplastic arteriolosclerosis)
  • > pulmonary vascular resistance reaches systemic levels and converts the left to right shunt to a right-to-left shunt
28
Q

What is the late reversal of a left to right shunt becoming a right to left shunt called? What happens after this?

A

late cyanotic congenital heart disease

Once irreversible pulmonary HTN develops the structural defects of Coronary heart disease are irreparable.

29
Q

What are some examples of Left to right shunts?

A
  • ASD
  • VSD
  • PDA
30
Q

What is this:
Abnormal fixed opening in the atrial septum caused by incomplete tissue formation that allows communication of blood between the left and right atria

A

ASD

31
Q

What are the three types of ASD?

A
  • Secundum (90%)
  • Primum (5%)
  • Sinus venosus (5%)
32
Q

What is this:

An ASD that results from deficient or fenestrated oval fossa near the center of the atrial septum

A

Secundum (90%)

33
Q

What is this:

An ASD that occurs adjacent to the AV valves

A

Primum (5%)

34
Q

What is this:

An ASD that occurs near the entrance of the SVC and may be associated with anomalous pulmonary venous return to the RA

A

Sinus venosus (5%)

35
Q

What are the clinical features of ASD?

When do you get symptoms?

A
  • left to right shunt due to lower pulmonary vascular resistance and greater distensibility of the RV
  • increased pulmonary blood flow produces a murmur
  • do not become symptomatic before age 30 and irreversible pulmonary HTN is unusual
36
Q

Surgical or catheter based repair of ASD is possible and prevents complications such as…?

A
  • Heart failure
  • Paradoxical embolization
  • Irreversible pulmonary vascular disease
37
Q

During fetal life the (blank) allows oxygen rich blood from the placenta to bypass the underinflated lungs. After birth, what happens to this and why?
There is a small possibility of what occurring?

A

foramen ovale

  • it is forced shut due to increased BP on the left heart (in 80% of people)
  • In 20% of people you can get increased pressure on the right, the flap can open and can create a right to left shunt (bowel movement, coughing, sneezing)
  • paradoxical embolism
38
Q

What is the most common form of congenital cardiac anomaly?

How do they commonly occur?

A

VSD

-associated with another congenital anomaly (only 20-30% occur in isolation)

39
Q

How are VSD’s classified?
What is a membranous VSD and are they common?
What is an infundibular VSD?
What kind of VSD is within the muscular septum and may have multiple?

A

by size and location

  • VSD in a membranous interventricular septum (90% of all VSDs)
  • VSD occurs below the pulmonary valve
  • swiss cheese septum
40
Q

What is this:

  • difficulties from birth
  • usually membranous or infundibular
  • cause significant left to right shunting
  • must have early correction
A

Large VSD

41
Q

What is this:

a VSD that is well tolerated and may close spontaneously

A

small VSD

42
Q

In fetal circulation the (blank) shunts blood from the pulmonary artery to the aorta. In PDA, what happens?
-What will a PDA sound like?
(blank) percent of PDA occur as an isolated anomaly
10% are associated with what?

A

ductus arteriosus

  • there is no spontaneous closure after birth
  • harsh, machine-like murmur
  • 90%
  • VSD, coarctation of the aorta or pulmonic or aortic valve stenosis
43
Q

What do the clinical features of PDA depend on?

A

diameter and CV status

44
Q

How does PDA present at birth?
How does narrow PDA present?
How do larger PDAs progress?

A
  • asymptomatic
  • does not affect child’s growth and development
  • larger PDA start out as a left to right shunt but can lead to Eisenmenger syndrome
45
Q

PDA closure should happen in early life, how do you keep one open?

A

prostaglandin E

46
Q

What is an AV septal defect (atrioventricular canal defect)

How does an AV result?

A
  • Patients have incomplete closure of the AV septum and malformation of the tricuspid and mitral valves
  • from the embryologic failure of the superior and inferior endocardial cushions of the AV canal to fuse adequately/
47
Q

What is a partial AV septal defect?

A

primum ASVD and cleft anterior mitral leaflet-> mitral insufficency

48
Q

What is a complete ASVD defect?

A

-Large combined AV septal defect and a large common AV valve (hole in center of the heart). -Resulting in communication of the four chambers-> volume hypertrophy

49
Q

1/3rd of AV septal defect patients have (blank)

A

down syndrome

50
Q

How to fix an ASVD?

A

Surgical repair

51
Q

What are the three types of obstructive congenital heart disease?

A
  • Coarctation of the aorta
  • Aortic valvular stenosis
  • Pulmonary valvular stenosis
52
Q

What is a common structural anomaly associated with Turner syndrome (XO)?

A

Coarctation of the aorta

53
Q

What is infantile coarctation of the aorta?

A
  • tubular hypoplasia of the aortic arch proximal to a patent ductus arteriosus
  • symptomatic in early childhood
54
Q

What is adult coarctation of the aorta?

A

Discrete ridgelike infolding of the aorta just opposite of the closed ductus arteriosus (ligamentum arteriosum) distal to the arch vessels

55
Q

The infantile form of coarctation of the aorta leads to manifestations (blank) in life. Does it have a high mortality rate?

A
  • early (right after birth)

- yes without surgical intervention

56
Q

Why does coarctation of the aorta result in lower extremity cyanosis?

A

because of delivery of unsaturated blood through the PDA

57
Q

How will the adult form of coarctation of the aorta present?

How do you repair this?

A
  • children may go unrecognized until adulthood unless severe
  • symptoms include: htn in the upper extremities, weak pulses and hypotension in the lower extremities, claudication and coldness of lower extremities, development of collateral circulation b/w pre and post coarctation arteries via enlarged intercostal and internal mammary arteries (notching seen on radiography)

-Surgical repair is possible

58
Q 
If you have pulmonary stenosis, what else will you get?
Mild cases may be (blank).
Severe cases require (blank).
Is it common?
Is it isolated or part of a syndrome?
A 
RVH
Asymptomatic
Surgical repair
Common malformation
Can be either
59
Q

What is this:
no communication b/w the right ventricle and lungs
What will this result in?
How do you get blood to the lungs?

A

Atresia
Hypoplastic RV and ASD
Patent Ductus Arteriosus

60
Q

What is this:

Cusps may be hypoplastic, thickened/nodular or abnormal in number

A

valvular aortic stenosis

61
Q

What is this:

  • Caused by thickened ring or collar of dense endocardial fibrous tissue below the cusps
  • Associated with a prominent systolic murmur
  • pressure hypertrophy of the LV
  • Mild stenosis managed conservatively and antibiotics (prevent endocarditis)
A

Subvalvuar aortic stenosis

62
Q

What is this:

  • An inherited form of aortic dysplasia
  • Ascending aorta is thickened causing luminal constriction
A

Supravalvular aortic stenosis

63
Q

(blank) causes obstruction to LV outflow and leads to hypoplasia of the left ventricle and ascending aorta (hypoplastic left heart syndrome)
How can you survive this?

A

Severe aortic stenosis/atresia

  • PDA is required for life
  • nearly fatal unless surgically repaired

Block 9 Week 1 (7 decks)

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