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CPR Exam 1 > Congenital heart dz > Flashcards

Congenital heart dz Flashcards

1
Q

What are the major causes of congenital heart disease?

A

Sporadic genetic abnormalities

Ex: Turner syndrome (issues w/ blood vessels), & trisomies 13, 18, 21

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2
Q

What is the single most common genetic cause of congenital heart disease?

A

Trisomy 21 (Down’s)

40% of Down’s pts have at least 1 heart defect

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3
Q

In Down’s, where is the heart defect usually derived from?

A

The second heart field (arterioventricular septae)

Most commonly defects of the endocardial cushion:

  • VSDs
  • ASDs
  • ostium primum
  • AV valve malformations
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4
Q

What genes are associated with congenital heart defects?

A

Notch pathway associated with variety of congenital heart defects:

  • NOTCH1 (bicuspid aortic valve)
  • JAG1 & NOTCH2 (tetralogy of Fallot)

Fibrillin mutations underlie Marfan syndrome = associated with valvular defects & aortic aneurysms

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5
Q

Name some congenital heart diseases. Include the most frequent first.

A

Ventricular septal defects (VSD)
Atrial septal defects (ASD)
Patent Ductus Arteriosus (PDA)

Left-to-right shunts
Right-to-left shunts
Obstructive lesions

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6
Q

What is the most common congenital heart dz?

A

Left-to-right shunts :

  • ASD
  • VSD (most common)
  • PDA
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7
Q

During ASD, VSD, & PDA, what happens to pulmonary volume and pressure?

A

ASD increases only RV & pulm outflow volumes

VSD & PDA increase pulm blood flow & pressure

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8
Q

What is a secundum ASD?

A

90% of all ASDs

Center of atrial septum; may be multiple or fenestrated

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9
Q

What is a primum anomalie?

A

5% of all ASDs

Adjacent to AV valves; often assoc w/ AV valve abnormalities &/or VSD

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10
Q

What are sinus venosa defects?

A

5% of all ASDs

Near entrance of SVD; can be assoc w/ anamalous pulmonary venous return to the R atrium

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11
Q

What is the result of left-to-right shunts? How can it be resolved?

A

Cause volume overload on right side; may lead to:

  • Pulmonary HTN
  • Right heart failure
  • Paradoxical embolism (where it travels to brain & causes stroke)

May be closed surgically with normal survival

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12
Q

Describe what happens with a patent foramen ovate (PFO). Include Sx.

A

80% closes permanently by 2 yo
Remaining 20% - flap can open if there is increase in right side pressure

Even temporary increase in pressure produces brief periods of R-L shunting causing:

  • Pulmonary HTN
  • Bowel movement
  • Coughing
  • Sneezing

Can lead to a paradoxical embolus

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13
Q

Describe the types of VSDs

A

90% = membranous VSD = in membranous interventricular septum

Infundibular VSD = below pulmonary value, or within muscular septum

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14
Q

How do VSD effects differ from pt to Pt?

A

Effects depend on size, & presence of other heart defects

Those that manifest w/ Sx as children, often assoc w/ other cardiac anamolies

Many (50%) small VSDs close spontaneously

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15
Q

Large VSDs may cause significant shunting. What does this lead to?

A

Right ventricular hypertrophy

Pulmonary HTN

Unclosed large VSD can ultimately result in shunt reversal, leading to cyanosis & death

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16
Q

When may a patent ductus arteriosus (PDA) fail to close?

A

Infants are hypoxic, and/or have defects assoc w/ increased pulmonary vascular pressure (VSD)

17
Q

Describe how a PDA appears clinically.

A

Produces a harsh, machinery-like murmur

  • usually asymptomatic at birth
  • initially left-to-right, so no cyanosis
18
Q

How is the effect of a PDA determined?

A

Shunt’s diameter

Large shunts can increase pulmonary prisolated PDA should be closed as early as possible

Isolated PDA should be closed as early as possible

19
Q

When can a PDA be beneficial? How can it be preserved?

A

Preservation of patency can be lifesaving in infants w/ obstruction of pulmonary or systemic outflow

Can be preserved w/ prostaglandin E

20
Q

What is the main Sx you see with right-to-left Shunts? What is the most common right-to-left shunt? What are some others?

A

Sx: cyanosis early in postnatal life (cyanotic congenital heart dz)

Tetralogy of Fallot (TOF) = most common

Transposition of great arteries
persistent truncus arteriosus
Tricuspid atresia
Total anomalous pulmonary venous connection

21
Q

What are the 4 cardinal features of Tetralogy of Fallot?

A

VSD
Obstruction of RV outflow tract
Aorta overrides the VSD
RV hypertrophy

22
Q

How does tetralogy of Fallot appear on imaging?

A

Heart is enlarged a “boot-shaped” b/c of the right ventricular hypertrophy

23
Q

What does clinical severity of Tetralogy of Fallot depend on?

A

Degree of subpulmonary stenosis

Mild stenosis = L-to-R shunt

Classic TOF = R-L shunt with cyanosis

Most infants cyanotic form birth, or soon thereafter

24
Q

Describe the transpostion of the great vessels (TGA). What other conditions do you see with this? What does survival look like?

A

Results in 2 separate systemic and pulmonary circulations

Incompatible with life after birth unless a shunt is present for mixing of blood b/w 2 circulations

  • approx 1/3 have VSD
  • 2/3 have patent foramen ovale or PDA
  • Right ventricle becomes hypertrophic (supports systemic circulation) and the left ventricle atrophies

Without surgery, pts will die within a few months

25
Q

What are some obstructive lesions of the heart?

A

Coarctation of the Aorta
Pulmonary stenosis or atresia
Aortic stenosis or atresia

26
Q

Describe coarctation of the aorta.

A

Narrowing of the aorta, generally seen WITH a PDA (infantile form), or WITHOUT PDA (adult form)

27
Q

In who are you more likely to see coarctation of the aorta?

A

Males more likely than female (2x)

Assoc with Turner Syndrome

28
Q

How does affect of coarctation differ?

A

Degree of narrowing is variable, with variable clinical effect

Clinical severity depends on degree of stenosis and patency of the ductus arteriosus

29
Q

What Sx are seen with coarctation of the aorta (with/without PDA)?

A

Coarctation with PDA = manifest at birth:
-may produce cyanosis in lower half of body

Coarctation without PDA = usually asymptomatic; may see:

  • HTN in upper extremities
  • Hypotension in lower extremities
  • Claudication and cold lower extremities
  • May eventually see concentric LV hypertrophy
  • Enlarged intercostal and internal mammary arteries “notching” on undersurface of ribs
30
Q

What would you see on a PE that would tell you it is an aortic coarctation?

A

Murmurs throughout systole +/- vibratory thrill

Concentric LVH

31
Q

Describe Eisenmenger syndrome.

A

Complication of having hole (shunt) b/w 2 chambers

Increased blood flow to pulmonary arteries

Shunt irreparable damages the walls of your lung’s arteries (pulm a.)

Syndrome occurs when pressure in pulm a. becomes so high that it causes oxygen-poor blood to flow from R-to-LV and then to body = cyanosis

Pressure also causes right ventricle to hypertrophy

CPR Exam 1 (11 decks)

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