Adult Congenital Heart Diseases Flashcards

1
Q

ASD

A
  • open communication b/w atria via a defect in the intra-atrial septum
  • second most common adult congenital disease after bicuspid AV, usually asymp. until adulthood
  • left to right shunt (originally)
  • complications: atrial arrhythmias, paradoxical embolus, cerebral abcess, right heart failure, pulmonary HTN –> Eisenmenger syndrome
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2
Q

patent foramen ovale

A

often of little significance

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

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3
Q

which echo is needed for operative closure and ddx of ASD?

A

trans-esophageal echo

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4
Q

two most common in adult cardiology?

A
  • Bicuspid aortic valve

- myxomatous mitral valve (MVP)

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5
Q

Eisenmenger sydrome

A
  • irreversible pulmonary HTN
  • results in reversal from left to right, to right to left shunting due to noncompliance of the lungs
  • this can happen with any kind of shunting, i.e. ASD, VSD, etc.
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6
Q

most common type of ASD?

A
  • secundum ASD, occurs in the middle of atria
  • 70% of ASD’s
  • due to defects in foramen ovale, not usually assoc. w/ other cardiac defects
  • usually closed with catheterization and plug
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7
Q

second most common ASD?

A

= primum defect

  • at bottom of atrial septa
  • 15-20% of patients
  • much more serious
  • sometimes involves portions of top of ventricular septum and mitral/tricuspid valve= endocardial cushion defect (or AV canal defect- could be in any chamber through putting probe in)
  • Almost always associated with defects in the AV valves or Ventricular septum (almost always assoc. w/ other defects)
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8
Q

Sinus Venosus ASD

A

third most common, about 5% of ASD’s

  • located high in the right atrium
  • 90% are assoc. w/ anomalous pulmonary vein insertion (one or more of four pulmonary veins enters into right atria rather than left)
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9
Q

Two types of sinus venosus ASDs?

A

Superior Sinus Venosus - SVC defect

Inferior Sinus Venosus- IVC defect

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10
Q

Scimitar Syndrome

A

Triad:

  1. Partial anomalous venous return
  2. Hypoplasia of a lobe of the right lung
  3. Thoracic aorta>Pulmonary artery collaterals
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11
Q

Pathophys of shunting in ASD?

A
  • shunt depends on the size of the defect, the compliance of the right and left ventricles, and the phases of contraction (systole/diastole, atrial ventricular, early or late in phase)
  • Most shunts start L>R, but all large shunts have some R>L *(even small shunts will have a small amount of R>L)
  • Shunt flow leads to a “useless circuit” of blood through the defect
  • This leads to right heart volume overload, well tolerated for many years, but can cause pulmonary HTN
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12
Q

sizes of ASDs?

A

lesions 2:1 will become symptomatic and req. surgical repair by age 40

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13
Q

clinical manifestations of ASD?

A
  • atrial arrhythmia: see increased HR, due to scarred/irritated SA node
  • see 20% due to atrial fibrillation/flutter that increases with advancing age, A fib in older pt. is more imp.
  • see risk of embolic events, including stroke. paradoxical stroke, systemic emboli
  • migraine cephalgia: might be assoc. though no evidence to back this up
  • pulmonary HTN and Eisenmenger syndrome can result, which requires >2.5:1 shunt
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14
Q

cyanosis?

A

know that shut is occuring in right to left - usually have Eisenmenger sydnrome or pulmonary valve stenosis

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15
Q

PE of ASD?

A
  • Physical findings are related to three things:
  • Size and location of defect
  • Size of the shunt
  • Pulmonary Artery Pressure (resistance)

** feel heaves at left sternal border, or retrosternal heaves (over the pulmonic valve) **

Shock = when you can feel a sound

Heart sounds: hear wide fixed split S2 (ASD makes splitting stay equal due to shunting), increased P2 with pulmonary HTN-
- usually heart S1 split w/ increase in tricuspid component

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16
Q

heart murmurs hear w/ ASD?

A

Heart Murmurs:

  • SEM Upper LSB from increased flow
  • Early DM, upper LSB from PI secondary to pulmonary hypertension
17
Q

Heart sound heard with ASD?

A

Heart sounds: hear wide fixed split S2 (ASD makes splitting stay equal due to shunting), increased P2 with pulmonary HTN-
- usually heart S1 split w/ increase in tricuspid component

18
Q

where do you feel heaves of ASD?

A

left sternal border, or retrosternal heaves (over the pulmonic valve) ***

19
Q

VSD

A
  • Most common congenital heart disease at birth, only accounts for 10% of adult congenital due to spontaneous closure
20
Q

small VSD

A

Small or Restrictive VSD have orifice diameter
R shunt with no LV volume overload

No pulmonary Hypertension

21
Q

moderate sized VSD?

A

Moderate sized defect, >25%-<75%

Mild-moderate volume overload of PA, LA,LV

No pulmonary hypertension

22
Q

large VSD?

A

Large VSD, >=75% of annulus

Moderate to large L>R shunts with LV volume overload, if uncorrected leads to PHTN, develops with pulmonary arterial obstructive disease

23
Q

VSD path?

A

Progressive pulmonary hypertension leads to RV pressures approaching systemic

Leads to reversal of shunt with R>L flow

Hypoxemia and cyanosis develop

This is Eisenmenger syndrome, and when coupled with VSD is called Eisenmenger Complex

24
Q

VSD clinical?

A

Small defect = small L>R shunt, asymptomatic adults

Moderate sized defect = either asymptomatic or mild CHF in children, usually gets smaller with growth and may have Aortic regurgitation (b/c membranous defects are directly below aortic valve, and can cause the prolapse of the aortic valves)

Large VSD = Usually early presentation with CHF in infancy or Eisenmenger’s in late childhood, early adulthood.

25
Q

PE of VSD?

A
  • hear long, loud holosystolic murmur at LSB, 2nd or 3rd ICS (usually w/ associated thrill)
  • 2/3 of EKG will be normal
  • Echo will show it directly
  • MRI/CT is excellent tool for looking at this
  • Cardiac Catheterization is less important for ddx now
  • defects are not usually closed percutaneously, must go in and close with surgery
26
Q

4 features of Tetralogy of Fallot

A
  1. RVOT (right ventricular outflow) obstruction
  2. VSD
  3. Aorta overrides IVS (aorta serves right and left ventricle)
  4. Concentric RVH

–> results in cyanosis

  • 10% of CHD, one of most common cyanotic congenital heart disease
  • this defect affects many things that are happening at the same time during embryonal development
  • The need for medical intervention is dependent on the degree of RVOT obstruction