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Block 4 - Cardio > Congenital Heart Disease > Flashcards

Congenital Heart Disease Flashcards

0
Q

What is the most common cause of congenital heart disease?

A

Multifactorial

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1
Q

What is the frequency of congenital heart disease?

A 
4-9/1000 live births not including bicuspid aortic valve 
32,000 new cases per year in US
1.5 million new case per year worldwide
20,000 surgeries per year in US
>85% survive to adulthood
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2
Q

What is the normal fetal circulation?

A

Gas exchange occur in placenta
Blood leaves RV to pulm artery then through ductus arteriosus to descending aorta and placenta for oxygenation
Some blood returning to RA will flow through foremen ovale to LA

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3
Q

What changes happen to the fetal circulation at birth?

A

Placenta gone - doubles systemic resistance
Lungs expand and lower pulmonary resistance
Flow across DA reverses and it closes within 18-24 hrs
Volume of blood through lungs and back through pulm veins to LA increases and raises pressure in LA - pushes septum primum against septum secundum to close foramen ovale

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4
Q

What does a probe patent foramen ovale allow the potential for?

A

Right to left shunt if right atrial pressure ever more than left due to pulm HT

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5
Q

What are the three general groups of congenital heart disease?

A

Left to right shunts - acyanotic - ASD, VSD, PDA
Right to left shunts - cyanotic - with decreased pulmonary flow (tetralogy of ferot) or increased pulmonary flow (transposition, truncated arteriosus)
Obstruction - aortic or pulmonic stenosis, coarctation of aorta, atrioventricular canal

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6
Q

What is the pathophysiology of an ASD?

A

Septum primum normally acts as valve to allow flow from R to L across foramen ovale but not L to R
RV is thinner and more compliant than LV allowing less resistance to filling during diastole
Increased volume flow through lungs - can usually tolerate and patients asymptomatic until adulthood - HPVD uncommon
RHF may develop because of increased work for RV

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7
Q

What are the different kinds of ASD?

A

Secundum type - no involvement of AV valves
Primum type - more problematic - abut AV valves and cause AV valve insufficiency
Sinus venosus type - faulty incorporation of pulm veins into posterior wall of developing atrium

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8
Q

What are the different types of VSD?

A

Peri membranous - involve membranous septum
Muscular
Inlet (endocardial cushion/AV canal)
Outlet (conal, infundibular)

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9
Q

What is the pathophysiology of a VSD?

A

Up to half close spontaneously
Shunting during both systole and diastole
Lungs have excessive volume and pressure - pulmonary vascular resistance increases which can lead to fixed pulm HT which can cause RHF

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10
Q

What are common complications of a VSD?

A

HPVD
CHF
Subacute bacterial endocarditis

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11
Q

What is eisenmenger syndrome?

A

In VSD
Pulm vascular resistance can become greater than systemic vascular resistance and flow across VSD reverses producing R to L shunt and tardive cyanosis
Can expose patient to thromboemboli and stroke from thrombi bypassing filtration in lungs

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12
Q

What is hypertensive pulmonary vascular resistance?

A

A VSD may not be apparent in the newborn because pulm resistance is still higher than systemic
As this changes, the shunt will increase and lead to pulm HT
grade 1 - arteriolar smooth muscle hypertrophies
Grade 2 - endothelial cells proliferate in pulmonary vessels
Grade 3 - intima becomes thick and fibrotic, narrowing lumen
Grade 4 - arterial lesions of tortuous endothelial channels develop, irreversible

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13
Q

What are predisposing factors to a PDA?

A

Prematurity
High altitude
Congenital rubella
Because normally closing is facilitated by increased oxygen content of blood flowing through

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14
Q

What is the pathophysiology of a PDA?

A

Flow during systole and diastole
Similar to VSD
Infants can develop CHF and adults at risk for subacute bacterial endocarditis and pulm HT

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15
Q

What are the four components of tetralogy of fallot?

A

VSD
Pulmonic stenosis
Overriding aorta
RV hypertrophy

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16
Q

What is the pathophysiology of ToF?

A

Narrowing of pulmonary outflow tract due to misalignment of conal septum during embryogenesis leads to pulm stenosis
If resistance caused by RVOT obstruction exceeds systemic resistance a R to L shunt across VSD and cyanosis results
Cyanosis increases with age and irreversible by 2-3 months

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17
Q

What are complications of uncorrected ToF?

A 
Acute cyanotic spells
Polycythemia
Strokes from paradoxical emboli
Cerebral abscess
Hypertrophic osteoarthropathy
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18
Q

What is the cause of transposition of the great arteries?

A

Failure of aortico-pulmonary and truncal septa to form in normal spiral manner

19
Q

When is TGA lethal in the neonate unless there is immediate intervention?

A

If there is not an accompanying ASD or VSD - then circulations running in series rather than parallel so venous blood recirculates without being oxygenated

20
Q

What can help survival in TGA until surgery can be performed? When must this surgery be performed by?

A

Ductus arteriosus and foramen ovale
ASD or VSD - allow SOME oxygenated blood to flow in both circuits
By 4-6 weeks, usually in first week before LV becomes too thin

21
Q

What is the pathophysiology of truncus arteriosus?

A

Upper aspect of conal septum is also absent creating large VSD in continuity with truncal defect at base of the heart
Single truncal valve usually quadricuspid
Large bidirectional shunt
Lungs at high pressure and flow - risk for HPVD

22
Q

What are the consequences of aortic stenosis from a congenital aspect?

A 
Worsens with age - severe by age 20
LVH
LHF
Bacterial endocarditis
Ischemic myocardial injury 
Outflow obstruction above or below valve - sub valvar stenosis by restrictive fibrous ring in tract below valve, supravalvar by mutation of elastin gene
23
Q

What is hypoplastic left heart syndrome?

A

Severe aortic stenosis or atresia is a component
LV and mitral valve small or absent
Preductal coarctation of aorta because low flow means no grow
Oxygenated blood from LA goes through foramen ovale and rescues systemic circulation only through DA
Can be lethal once DA closes - present like septic shock when it happens

24
Q

What is the pathophysiology of congenital pulmonic stenosis?

A

Main pulmonary artery dilated distal to valve
RV is hypertrophic
Doesn’t progress over time and endocarditis is rare
Mild usually asymptomatic
Moderate to severe disease treated by balloon

25
Q

What can severe stenosis or atresia of pulmonic valve cause?

A

Cyanotic congenital heart disease in neonate - results from R to L shunt across atrial septum and diminished flow to lungs
RV may also be hypoplastic

26
Q

What is infantile (preductal) coarctation of the aorta?

A

Tubular narrowing of entire aortic segment between subclavian artery and ductus
Large PDA always present - as it closes, symptoms increase
Often associated with other cardiac malformations reducing LV output
Surgical intervention required for survival

27
Q

What is adult (postductal) coarctation?

A

More sharply define ridge of tissue narrowing aortic lumen in area of closed ductus (ligamentum arteriosum)
Ridge contains aortic media and a thickened intima
Vascular collaterals develop and can cause notching of ribs
Hypertension, murmur, and weak pulses are clinical findings
Bicuspid aortic valve in half - risk for aortic stenosis and IE
Some have berry aneurysms
Die by mean age of 30 unless fixed

28
Q

How can cardiac cath help in congenital heart disease?

A

Can detect increased oxygen content of blood in right sided chamber in L to R shunts

29
Q

When is a L to R shunt considered hemodynamically significant?

A

If pulmonary blood flow is 1.5-2x greater than systemic blood flow

30
Q

What is the difference between central and peripheral cyanosis?

A

Central - from decreased arterial oxygen saturation (alveoli not adequately ventilated or shunting)
Peripheral - from increased oxygen extraction in peripheral tissues (shock, hypovolemia, vasoconstriction)

31
Q

What are the initial steps in evaluation of a patient with congenital heart disease?

A

Careful physical, measurement of oxygen saturation, EKG, and chest x ray
Classify as cyanotic or acyanotic
Further eval using echo or cath

32
Q

What are the five most common cardiac causes of cyanotic children?

A 
ToF
Tricuspid atresia 
TGA 
Truncus arteriosus
Total anomalous pulm venous return
33
Q

What are the two most common cardiac causes of cyanotic adults?

A

Eisenmenger syndrome

ToF

34
Q

What is revealed on the exam of ASDs?

A

Enlargement of RA and RV, and pulm vasculature
No pressure gradient between atria
Systolic ejection murmurs over pulm valve due to increased flow
RAD
Incomplete RBBB

35
Q

What are exam findings with VSDs?

A

Holosystolic murmur with a thrill
EKG may be normal or may have LV or biventricular hypertrophy
X ray may be normal or may have enlarged LA, LV, or RV and pulm vessels

36
Q

What do ALL patients with any kind of VSD large or small need?

A

Prophylaxis against bacterial endocarditis

37
Q

What are clinical or exam findings with a PDA?

A

Large one can compress laryngeal nerve resulting in hoarseness
Systolic murmur until 2-6 weeks, then continuous
EKG normal or LVH
X ray normal, or dilated pulm artery, pulm vessels and aorta, LA or LV may be enlarged
Pressures elevated everywhere but RA and RV

38
Q

What are the clinical and exam findings in coarctation of aorta?

A

Elevated BP in upper extremities
Radial femoral pulse delay
Systolic murmur over coarctation or murmur from bicuspid valve
EKG normal or LVH
X ray can show enlarged LV, post stenotic dilatation of aorta, rib notching
Pressure elevated in LV and aorta only

39
Q

When is intervention used in coarctation of the aorta?

A

When pressure gradient is greater than 30-40

40
Q

What are the clinical findings of ToF?

A

Cyanosis in first year due to R to L shunt across septal defect as well as decreased pulm blood flow
Squatting in childhood
Spells characterized by worsening cyanosis, decreased intensity or disappearance of murmur, can result in loss of consciousness

41
Q

What are the physical exam findings in ToF?

A

Systolic ejection murmur, single S2, RV lift, cyanosis and clubbing
RV hypertrophy
Enlarged RV, small pulm artery, decreased lung markings, boot shaped heart

42
Q

What is important about eisenmenger reaction?

A

It is permanent and irreversible

Want to correct a L to R shunt before this can happen

43
Q

What kinds of echo are used to assess congenital heart disease?

A

Two dimensional TTE with Doppler color flow mapping provide real time imaging
Contrast involves IV injection of saline and can detect small R to L shunts
Doppler evaluates obstructive lesions and pressure gradients
TEE can visualize structure not seen on TTE

44
Q

What is MRI particularly useful for?

A

Assessment of extra cardiac anatomy

Contrast can help look at valves

45
Q

What are the main differences between echo and cath?

A

Both visualize anatomy
Echo assess function, cath measures pressure
Echo identifies shunts, Caths quantify size
Echo use TEE for posterior structures, cath can identify anomalous coronary arteries

Block 4 - Cardio (15 decks)

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