Adult Congenital Heart Disease Flashcards

1
Q

Three most common in adults

A

Bicuspid aortic valve -> aortic stenosis or regurgitation
Myxomatous degeneration -> mitral valve prolapse
**ASD

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2
Q

ASD

A

Open communication between the atria via a defect in the intra-atrial septum

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3
Q

Effect of ASD on heart

A

Enlarged RA, RV and pulmonary arteries from increased blood volume due to shunting left to right

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4
Q

Patent Foramen Ovale

A

A foramen covered by the septum primum but is not sealed shut in 20% of normal subjects

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5
Q

Which direction does blood shunt in atrial defect

A

Left to right

Right to left is not common

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6
Q

When is shunting of blood noticeable to patient

A

Approximately 2.5:1

1.5:1 or 2:1 generally well tolerated

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7
Q

When do you notice PFO with imaging

A

Bubble study on echo

Can follow blood as it moves through heart

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8
Q

Diagnosis of atrial defect made with

A

Echo

Diagnose and characterize defect

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9
Q

Transient R to L shunt

A

Occurs at onset of ventricular contraction

Can lead to paradoxical stroke (neuro symptoms without cyanosis)

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10
Q

How well is right sided volume overload tolerated

A

Tolerated for years as long as less than 2.5:1 aka around 100% more volume

Right side of heart has more trouble compensating via hypertrophy to increased volume
Pulmonary vasculature also reacts –> constricts, atherothrombosis

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11
Q

Bicuspid aortic valve

A

Increased problems with aortic regurg and stenosis

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12
Q

Myxomatous Mitral Valve

A

Degeneration of collagen in leaflets -> prolapse

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13
Q

Other common defects

A

Tetrology of Fallot
Ebstein’s
Eisenmenger
PDA

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14
Q

Complications of ASD

A
Atrial Arrhythmias
Paradoxical Embolus
Cerebral Abcess
Right Heart Failure
Pulmonary Hypertension -> Eisenmenger Syndrome
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15
Q

Secundum ASD

A

Most common type - 70%
More common in females

Due to defects in the foramen ovalis
Usually not associated with other cardiac defects
Closed percutaneously

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16
Q

Primum ASD

A

Not as common - 10-15%
Large and more severe

Almost always associated with defects in the AV valves or Ventricular septum
AV Canal, or Endocardial Cushion Defect is the complete form

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17
Q

Sinus Venosus ASD

A
Not as common - 5%-10% of ASD
Often associated (>90%) with anomalous pulmonary vein insertion (empty in right atrium) -> L to R shunt

Superior Sinus Venosus-SVC Defect
Inferior Sinus Venosus Defect-IVC Defect

Can’t be closed percutaneously

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18
Q

Scimitar syndrome

A

Triad with ASD

  1. Partial anomalous venous return (to RA not LA)
  2. Hypoplasia of a lobe of the right lung (where vein comes from)
  3. Thoracic aorta -> Pulmonary artery collaterals

Seen on chest xray - curved shadow

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19
Q

ASD pathophysiology

A

Shunt depends on the size, compliance of the right and left ventricles, and phases of contraction (systole/diastole, atrial ventricular, early or late in phase)

Most shunts start L to R, but all large shunts have some R to L

Shunt flow leads to a “useless circuit” of blood through the defect back to RA. The flow may be trivial or as much as 8:1, but more likely 2:1-5:1

Right heart volume overload well tolerated for years, but can cause Pulmonary Hypertension and Eisenmengers

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20
Q

How to measure RV pressure

A

Measure velocity of echo - not very accurate

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21
Q

Higher systolic pressure with age

A

Stiffening of aorta

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22
Q

Size of ASD

A

Lesions <8mm often are without symptoms

Larger lesions tend to enlarge with age

23
Q

Clinical manifestations of ASD

A

Most patients with ASD and significant shunt flow will become symptomatic and require surgical repair by age 40

Atrial Arrhythmias – increase HR
20% atrial fibrillation or flutter, increases with advancing age

At risk for embolic events, including stroke, (paradoxical stroke and systemic emboli)

Migraine cephalgia may be associated -> PFO not really associated with it

Pulmonary Hypertension and Eisenmenger syndrome, requires >2.5:1 shunt

Cyanosis – R to L shunt
Usually associated with concomitant Pulmonic Valve Stenosis or Eisenmengers

24
Q

ASD associated with

A

Concomitant Pulmonic Valve Stenosis and Eisenmengers

25
Q

ASD arrythmias

A

Atrial -> increase HR

20% atrial fibrillation or flutter, increases with advancing age

26
Q

Does patient with ASD have risk of embolism?

A

Yes

At risk for embolic events, including stroke, (paradoxical stroke and systemic emboli)

27
Q

Is migraine cephalgia associated with PFO?

A

No - associated with ASD

28
Q

Physical exam findings depend on

A

Size and location of defect
Size of the shunt
Pulmonary Artery Pressure (resistance)

29
Q

Precordium PE findings of ASD

A
RV Heave (volume overloaded)
Palpable PA at upper LSB or retrosternal border (feel it lift)
30
Q

Heart sounds of ASD

A

Wide fixed split S2
Increased P2 with Pulmonary Hypertension
S1 split with increase in Tricuspid component

31
Q

Heart murmurs of ASD

A

SEM Upper LSB from increased flow

Early DM, upper LSB from PI secondary to pulmonary hypertension

32
Q

VSD

A

Most common congenital heart disease at birth, only accounts for 10% of adult congenital due to spontaneous closure

33
Q

Decreased size of VSD and it’s effect on murmur

A

Smaller VSD, louder murmur

34
Q

Infundibular VSD

A

Below the aortic and pulmonic valves, leading to progressive aortic regurgitation (hallmark)

35
Q

Membranous VSD

A

Conoventricular

Deficency of the membranous septum

36
Q

Inlet VSD

A

AV Canal

Down’s

37
Q

Muscular VSD

A

Trabecular system

5-20%

38
Q

VSD shunt flow depends on

A

Functional size and the ratio of pulmonary to systemic vascular resistance

Direction and severity

39
Q

Small or Restrictive VSD

A

Orifice diameter less than or equal to 25% of aortic annulus diameter
Small L to R shunt with no LV volume overload
No pulmonary Hypertension

40
Q

Moderate VSD

A

25% - 75%
Mild-moderate volume overload of PA, LA, LV
No pulmonary hypertension

41
Q

Severe VSD

A

75% or greater
Moderate to large L to R shunts with LV volume overload, if uncorrected leads to PHTN -> pulmonary arterial obstructive disease

42
Q

Eisenmenger syndrome

A

Progressive PHTN leads to RV pressures approaching systemic pressure
Leads to reversal of shunt with R to L flow
Hypoxemia and cyanosis develop

With VSD = Eisenmenger complex

43
Q

Too many changes in lungs due to VSD

A

Irreversible

44
Q

Clinical findings of small VSD

A

Small L to R shunt

Asymptomatic adults

45
Q

Clinical findings of moderate VSD

A

Either asymptomatic or mild CHF in children Usually gets smaller with growth
May have aortic regurgitation

46
Q

Clinical findings of large VSD

A

Usually early presentation with CHF in infancy or Eisenmenger’s in late childhood/early adulthood

47
Q

Heart sounds of VSD

A

Loud holosystolic at LSB, 2nd or 3rd ICS

Thrill

48
Q

EKG with VSD

A

66% are normal

49
Q

Risk of VSD surgical repair

A

Disrupt conduction system requiring pacemaker

50
Q

Test to confirm VSD

A

Echo

CT/MRI more important for complex defects and congenital defects

51
Q

Features of tetralogy of fallot

A
  1. RVOT obstruction
  2. VSD
  3. Aorta overrides IVS
  4. Concentric RVH
52
Q

Conserved EF with TOF

A

Has everything to do with LV

53
Q

One of most common cyanotic CHD

A

Tetralogy of fallot

54
Q

Need for surgical intervention with TOF

A

Dependent on the degree of RVOT obstruction