Chd And L->R Shunts; Ped Cards: Obstruc, Regurg

Front 1

Which is more common: L to R or R to L shunts?

Back 1

L to R

Front 2

By the end of the ____ gestational week, the heart develops into 4 chambers

Back 2

7th

Front 3

Peak vulnerable period for exposure to environmental influences is thought to be the first _____ gestational weeks

Back 3

2-4 weeks (day 20)

Front 4

What CV manifestations is Marfan syndrome associated with?

Back 4

Aortic root dilations, aortic dissection with mitral valve prolapse and insufficiency.

Front 5

What CV manifestations is William syndrome associated with?

Back 5

Supravalvular aortic stenosis, peripheral pulmonary stenosis, systemic arterial stenosis

Front 6

What CV manifestations is Ehlers-Danlos IV syndrome associated with?

Back 6

Aortic dilations and aneurysms

Front 7

approximately _% of CHD have an etiologic basis solely on environmental factors

Back 7

2

Front 8

What environmental factors contribute to CHD?

Back 8

Drugs (lithium, ETOH); Viruses; Radiation; CT disease in mother; Metabolic disease (infant of diabetic mother)

Front 9

The ____ is the major route of gas
exchange, excretion, and acquisition of
essential fetal chemicals, providing a low resistance circuit.

Back 9

placenta

Front 10

The ___ is the first fetal organ to receive maternal substances like oxygen, glucose, amino acids, etc

Back 10

liver

Front 11

In the fetus, the ___ ventricle pumps against higher pressure than the ____ ventricle

Back 11

right; left

Front 12

What type of shunt allows for increased pulmonary blood flow?

Back 12

Left to right shunts are communications between the systemic and pulmonary circuits which allow already oxygenated blood to recirculate through the pulmonary circuit.

Front 13

Flow (Q) =

Back 13

Pressure (P) / Resistance (R)

Front 14

How does the size of the VSD affect the pressure of the RV?

Back 14

The larger the ventricular septal defect, the more closely the RV systolic pressure approximates that of the LV.

Front 15

What are restrictive defects?

Back 15

Smaller VSDs associated with significantly sub-systemic RV pressures

Front 16

What are non-restrictive defects?

Back 16

Larger VSDs associated with systemic RV pressures. Volume of blood flow will depend on where the combined resistance to flow produced by septal defect and pulmonary vascular resistance vs. the resistance in systemic circulation.

Front 17

Flow goes where ________

Back 17

resistance is least

Front 18

Pulmonary artery pressure is directly determined by the radius of what?

Back 18

* The RADIUS of the ductus arteriosus
* The INVERSE of the LENGTH of the ductus.

Front 19

The volume of the shunt blood flow of a PDA depends upon the combined resistance of the ductus and pulmonary arterioles versus the resistance offered by_____________

Back 19

the systemic circulation.

Front 20

Critical obstructive CHD: defn

Back 20

maintenance of fetal circulation is required to maintain adequate CO

Front 21

the pressure difference across stenotic  artery or valve

Back 21

gradient

Front 22

narrowing of valve or artery resulting in a  pressure difference across it.

Back 22

stenosis

Front 23

What is the most common etiology of congenital aortic stenosis?

Back 23

valvular - usually result of bicuspid aortic valve

Front 24

Other than valvular, what 2 other types of aortic stenosis are there?

Back 24

1) Supravalvular - rare, associated with Williams syndrome

2) Subvalcular - discrete or tunnel like, rare

Front 25

With critical aortic stenosis, what happens when the DA closes?

Back 25

LV fails and there are signs of congestive heart failure

Front 26

How does typical (non critical) aortic stenosis present?

Back 26

as a murmur in asymptomatic patient.

With a gradual increasing stenosis/enlarging gradient, the LV compensates with hypertrophy which may eventually cause symptoms with exercise, particularly

Front 27

What type of murmur characterizes aortic stenosis?

Back 27

Systolic Ejection Murmur (SEM)

Front 28

How will the pulses change in critical Aortic stenosis?

Back 28

When the DA closes in critical aortic stenosis, there will be a huge decrease in pulses (comcomitant with decreased total body perfusion).

Front 29

Signs of CHF in an infant

Back 29

poor feeding, tachypnea, hepatomegaly, failure to thrive

Front 30

With typical aortic stenosis, which of S1 or S2 will be affected?

Back 30

S1 is normal (mitral/tricuspid valve); There will be a systolic murmur present that radiates to neck. Systolic ejection click heard in 60-80% of cases.

Front 31

What will be seen on CXR in critical aortic stenosis?

Back 31

cardiomegaly, pulmonary venous congestion,

Front 32

What imaging is diagnostic for critical aortic stenosis?

Back 32

Echo - will show aortic valve turbulence.

Front 33

What will become apparent on EKG as stenosis progresses (typical aortic stenosis)?

Back 33

LV hypertrophy

Front 34

What is the treatment of critical aortic stenosis?

Back 34

MUST give prostaglandin E1 to stabilize/maintain ductal patency.

Needs intervention in infancy - options include balloon valvuloplasty or surgical valvotomy.

Front 35

What is the treatment of typical aortic stenosis?

Back 35

these patients will remain well generally. Treatment consists of observing the progression of pressure difference across valve and looking for regurgitation. When it gets to a certain point, must do surgery.

Front 36

, the most common cause of death due to heart disease in the first month of life

Back 36

Hypoplastic Left Heart Syndrome

Front 37

Describes a group of cardiac malformations where there is aortic valve stenosis or atresia with hypoplasia or absence of LV and as a consequence, hypoplasia of ascending aorta

Back 37

Hypoplastic Left Heart Syndrome

Front 38

Hypoplastic Left Heart Syndrome: PE findings at birth

Back 38

NORMAL until the DA closes.

Then see tachypnea, tachycardia, hepatomegaly and poor perfusion/pulses resulting.

Murmurs are variable and non-specific.

Front 39

What is seen on
A) CXR
B) EKG in
Hypoplastic Left Heart Syndrome?

Back 39

A) enlarged heart with increased vascular markings


B) Right axis deviation and ventricular hypertrophy - dominant RV forces due to absence of LV.

Front 40

Options for tx of Hypoplastic Left Heart Syndrome

Back 40

Initially: Give PGE1 to maintain DA patency;

Then:

Palliative surgery

Transplant (hard to get normal infant hearts)

Do nothing

Front 41

T/F Hypoplastic Left Heart Syndrome can be dx'ed in utero

Back 41

T. This gives parents chance to end pregnancy.

Front 42

Coarctation of the aorta: defn

Back 42

constriction somewhere in the aorta

Front 43

Where do most Coarctations of the aorta occur?

Why?

Back 43

98% present around the insertion of the DA (juxtaductal.)

This is because the ductus arteriosus is a muscle vessel and when there is an increase in oxygen it constricts. The muscular band can sometimes cause a constriction in the aorta.

Front 44

PE findings of a typical (ie, non-critical) Coarctation of the aorta

Back 44

asymptomatic, normal cardiac exam

Pulse: classic sign is <b> diminished distal pulse which is delayed from right arm to leg.</b>

BP may be preserved in older patients with efficient  collateral network.

Front 45

What are the most frequent collateral blood vessels to develop, allowing blood to bypass the coarcted area and reach lower part of body?

Back 45

Intercostal arteries.

Front 46

What is seen on
A) CXR
B) EKG
C) Echo

in critical coarctation of aorta?

Back 46

A) Cardiomegaly with increased Pulmonary Vascular Markings (PVM)

B) Normal neonatal patterns (right axis deviation and ventricular hypertrophy)

C) Diagnostic

Front 47

What is seen on
A) CXR
B) EKG
C) Echo

in typical coarctation of aorta?

Back 47

A) Normal heart, see rib notching due to overdevelopment of collateral vessels.

B) Normal or LV hypertrophy and axis deviation, but depends on severity

C) Echo is diagnostic

Front 48

Treatment of coarctation of aorta

Back 48

Mostly surgical.

Use PGE1 to maintain PDA if it's critical.

May be able to use balloon dilation in some types.

Front 49

Prognosis for coarctation of aorta

Back 49

Usually good, less than 2% mortality. Older patients at higher risk for persistent systemic htn. These patients will have higher risk for circle of willis aneursyms and stroke related to HTN.

Front 50

T/F incidence of htn is increased in coarctation of aorta later in life despite adequate tx

Back 50

t

Front 51

Pulmonary stenosis is associated with what cyanotic-causing disease?

Back 51

tetralogy of fallot

Front 52

What is the most common type of pulmonary stenosis

Back 52

valvular

Front 53

What is the causes of valvular pulmonary stenosis?

Back 53

Complete or partial fusion of one or more of the commissures, resulting in systolic obstruction.

There is post-stenotic dilation of the proximal main pulmonary artery.

Front 54

Symptoms of pulmonary stenosis?

Back 54

most patients are asymptomatic. (mild-moderate).

Front 55

Physiologic consequences of mild-moderate pulmonary stenosis?

Back 55

Resultant RV hypertrophy. Longstanding or severe RVH may result in RV failure or decrease in ventricular compliance.

Front 56

What is definition of severe pulmonary stenosis?

Back 56

Those with presence of atrial communication like foramen ovale or ASD where cyanosis may result bc of R to L atrial shunt.

Generally systemic level RV pressures and/or pulmonary valve gradients > than 80 mmHg.

Front 57

Physical exam findings in mild-moderate pulmonary stenosis?

Back 57

rarely RV impulse, nl S1 and S2 (usually)
Systolic ejection click in 80%, varies with respiratory cycle

Front 58

Physical exam findings in severe valvular pulmonary stenosis?

Back 58

Jugular venous distension

Cardiomegaly

RV impulse

Long harsh systolic murmur, may mask S2 and the ejection click

Palpable thrill

Front 59

Where are murmurs from pulmonary stenosis best heard?

Back 59

Left sternal border.

They radiate with pattern of flow.

Front 60

What are CXR findings in pulmonary stenosis?

Back 60

See enlarged main pulmonary artery segment corresponding to a post-stenotic dilation.

Late finding of cardiac enlargement.

Front 61

What are EKG findings in pulmonary stenosis?

Back 61

See a spectrum from normal to severe cases with Right Axis Deviation due to RV hypertrophy.

Front 62

What is tx for severe pulmonary stenosis?

Back 62

Balloon angioplasty of valve to reduce transvalvular gradients by tearing open fused commissures.

Front 63

Regurgitation is better tolatered on the _ side as opposed to the __ side

Back 63

R as opposed to L. This is because the pulmonary pressure isn't too high, unlike the aortic pressure. Not that much will come back in thru regurgitant pulmonary valve.

Front 64

T/F Isolated regurgitant congenital heart disease is common

Back 64

F. Rare.

Front 65

What is Ebstein anomaly?

Back 65

congenital heart defect in which the opening of the tricuspid valve is displaced towards the apex of the right ventricle of the heart.

Almost always has tricuspid regurgitation as part of its pathophysiology.

Front 66

What is the primary physiologic consequence of valvular regurgitation?

Back 66

Increased volume load on ventricle responsible for forward flow on that side.

Front 67

A usually benign condition where there's redundant MV tissue without associated lengthening of the MV chordae

Back 67

MV prolapse

Front 68

What happens in MV prolapse during systolic closure?

Back 68

The prolapsing leaflets undergo a sudden snapping tension.

Front 69

PE findings for MV prolapse

Back 69

Maybe hyperdynamic precordium with diminished S1.

Hear a high-frequency blowing pansystolic murmur at left apex radiating to axilla and back. Classic midsystolic click is heard variably followed by a short regurgitant murmur.

Front 70

What does mitral valve prolapse carry as an additional risk?

Back 70

bacterial endocarditis

Front 71

usually a result of RV volume overload, hypertrophy, or Ebstein's anomaly

Back 71

tricuspid regurgitation

Front 72

(Acute, Chronic) regurgitation is better tolerated than (acute, chronic).

Back 72

Chronic; Acute

Front 73

Rheumatic Heart Disease
Bacterial Endocarditis
Aortic root abnormalities (Marfan’s)

All associated with what ?

Back 73

Aortic regurgitation

Front 74

Most common cause of aortic regurgitation

Back 74

Congenital bicuspid or unicuspid aortic valve.

Front 75

What is a common cardiac problem in marfan disease?

Back 75

aortic root dilation causing aortic regurgitation

Front 76

Symptoms of acute aortic regurgitation

Back 76

Decompensation is usually dramatic as the LV has no time to compensate for acute changes in volume work.

Front 77

Pulmonary Regurgitation: Clinical consequences

Back 77

Not as many as aortic regurgitation.

Not of physiologic consequence as the regurgitant volume is small due to low downstream resistance
Well tolerated whether acute or chronic
Treatment generally not necessary