Congenital Heart Dzs And Valve Stuff

Front 1

Congenital heart diseases can be genetic (association with chromosomal abnormalities like trisomies ___,___,___,____; or what syndrome) or can also be environmental such as a ______________ infection or exposure to things like ____________ and ___________.

Back 1

Genetic: Trisomies 13, 15, 18, 21: Turner syndrome

Environmental: Congenital Rubella infection, thalidomide and alcohol

Front 2

Congenital heart dz can be divided into two major categories: _________, or abnormal communications between vessels or chambers, and ______________, or valve malformations or vascular narrowings.

Back 2

Shunts
Obstructions (hypoplasoa or atresia = valve malformations; coarctation of the aorta = vascular narrowings)

Another way to look at congenital heart dzs is clinical categorizations of cyanotic or acyanotic. Cyanotic is when poorly oxygenated blood enters systemic arterial circuit. Ex. R to L shunt through septal defect.

Front 3

What is the most common congenital heart dz defect?
A. Atrial septal defect
B. Ventricular septal defect
C. Patent ductus arteriosus
D. Tetralogy of Fallot

Back 3

B. Ventricular septal defect

90% involve a small membranous portion just underneath the aortic valve. That small membranous portion is called the membranous septum.

Signs and symptoms depend on the SIZE of the ventricular septal defect.

Front 4

To discern whether the individual will be cyanotic or acyanotic, remember the golden rule that blood flows in direction of.....

Back 4

BLOOD FLOWS IN THE DIRECTION OF LEAST RESISTANCE!!

So, for example, if defect is in intraventricular septum, which direction will blood flow? L --> R b/c pressure is loewr on the R therefore resistance is lower on R so blood goes there.

Front 5

True or False:
Small ventricular septal defects might close spontaneously.

Back 5

TRUE (50%) do close spontaneously. If they remain patent and are truly small, then they might be tolerated well.

Large VSDs though usually remain patent due to sheer size. Are acyanotic. But over time can lead to shunt reversal (Eisenmenger) and then become a cyanotic process.

Front 6

What are the four components of Tetralogy of Fallot?

Back 6

Ventricular septal defect, obstruction of RV outflow tract (subpulmonary stenosis), overriding aorta (positioned over the VSD),R ventricular hypertrophy (consequence of the obstruction of RV outflow tract).

Front 7

What is the most common form of cyanotic dz?

A. Atrial septal defect
B. Ventricle septal defect
C. Tertalogy of Fallot

Back 7

C. Tetralogy of Fallot

Four components of a tetralogy of fallot: Ventricular septal defect, obstruction of RV outflow tract (subpulmonary stenosis), overriding aorta (positioned over the VSD),R ventricular hypertrophy (consequence of the obstruction of RV outflow tract).

Direction of flow and severity of symptoms depends n the degree of obstruction to RV outflow.

Front 8

When transposition of the great vessels occurs (pulm artery arises from the L ventricle, aorta arises from the R ventricle), survival depends on ......

Back 8

patent ductus arteriosis or septal defect

Front 9

Prevalence of congenital malformation of the heart is ___% of live births if family history is negative (8/1000), _____% if Trisomy 21, ______ if mom has a congenital malformation of the heart, ______ if mom is a diabetic.

Back 9

1% worldwide if negative fam history
40-50% if Trisomy 21
1 in 20 if mom has congenital heart defect
1 in 39 if mom is a diabetic

Front 10

What are the top two most frequent congenital heart malformations?
A. Atrial septal defect
B. Venricular septal defect
C. Tetralogy of Fallot
D. Patent ductus arteriosus
E. Pulmonary stenosis
F. Aortic stenosis

Back 10

B. Venricular septal defect (25%)
C. Tetralogy of Fallot (15%)

All rest is 10%

Front 11

Which syndrome has cardiovascular manifestations of aortic root dilations, aortic dissection with mitral prolapse and insufficiency? (Hint: Gene defect is fibrillin-1)
A. Ehlers-Danlos IV
B. Marfan's syndrome
C. Rendu-Osler Weber
D. William's syndrome

Back 11

B. Marfan's syndrome

Front 12

Which syndrome has cardiovascular manifestations of supravalvar aortic stenosis, peripheral pulmonary stenosis, systemic arterial stenoses? (Hint:Elastin is genetic defect)
A. Ehlers-Danlos IV
B. Marfan's syndrome
C. Rendu-Osler Weber
D. William's syndrome

Back 12

D. William's syndrome

Front 13

Which syndrome has cardiovascular manifestations of telangiectasia and pulmonary arteriovenous fistulas? (Hint: unknown genetic defect)
A. Ehlers-Danlos IV
B. Marfan's syndrome
C. Rendu-Osler Weber
D. William's syndrome

Back 13

C. Rendu-Osler Weber

Front 14

Which syndrome has cardiovascular manifestations of aortic dilation and aneurysms? (Hint: type III collagen is the genetic defect)
A. Ehlers-Danlos IV
B. Marfan's syndrome
C. Rendu-Osler Weber
D. William's syndrome

Back 14

A. Ehlers-Danlos IV

Front 15

In fetal circulation, the placenta provides a [ high / low ] resistance circuit.

Back 15

LOW

(the baby's pulmonary circuit is a HIGH RESISTANCE circuit and thus blood flow to the lungs is only a fraction of the combined ventricular output . The R ventricle pumps against a higher resistance than the L ventricle. The two ventricles work in parallel rather than in series. And as a normal state, the intra and extra cardiac shunts are present.

Front 16

Describe the fetal circulation.

Back 16

In utero, the placenta is the oxygenation organ of the fetus. Oxygenated blood comes up thru the IVC. B/c of the eustacian valve, a valve of the inferior ven acava, the majority of the IVC flow (the most oxygenated blood in the fetus) is directed across the foramen ovale to the L atrium. That blood flow mixes with the very little pulmonary venous flow there is, goes thru L ventricle and out the aorta so on the aortic side, the brain gets the most oxygenated blood (makes sense). The remainder of the flow that does NOT go across the foramen ovale from the IVC mixes with the blood coming back from the superior vena cava, goes across the tricuspid valve to the R ventricle, then out pulmonary artery. Because the pulm circuit has a high resistance though, the vast majority of the blood traveling thru the pulm artery (mostly deoxygenated blood) will go thru the patent ductus arteriosus and eventually placenta where gas exchange takes place.

So, at the foramen level there is a R to L shunt and at the ductus arteriosus level there is a R to L shunt. Avg pO2 of a fetus is 18-25 mmHg (anaerobic state).

Front 17

Neonatal circulation occurs because there are two important events that take place immediately following birth (15-30 seconds afterwards): the infant takes his first breath of air and the placenta is separated from the infant. What happens to the pulmonary vascular resistance, pulmonary blood flow, and systemic vascular resistance?

Back 17

Pulmonary vascular resistance decreases, pulmonary blood flow increases, systemic vascular resistance increases.

Other important things that happen: blood flow thru ductus arteriosus becomes primarily L to R, and the foramen ovale closes functionally.

Front 18

Which of the following is the most common cause of death due to congenital heart dz in the first month of life? Which is the most common congenital heart defect presenting in the 2nd week of life?

A. Coarctation of the Aorta
B. Hypoplastic L heart syndrome
C. R sided obstructive lesions like pulmonary stenosis

Back 18

death in first month given congenital heart dz = B. Hypoplastic L heart syndrome

2nd week of life presentation = coarctation of the aorta

Front 19

Systolic clicks are often caused by ________. Systolic clicks are high pitched, usually followed by a late systolic crescendo murmur. Findings can vary with time and body position. Squatting delays click and murmur ; standing moves click and murmur closer to S1.

Back 19

Mitral valve prolapse

Front 20

What two types of valve disorders are described as pansystolic mumurs?

Back 20

mitral regurgitation and tricuspid regurgitation.

for mitral: S1 might be diminished. pitch is medium to high, quality harsh.
for tricuspid: pitch is medium

Front 21

How would one describe a classic aortic stenosis?

(hint: systolic/diastolic , early/middle/pan, crescendo/decrescendo/crescendo-decrescendo)

Back 21

aortic stenosis is a MIDSYSTOLIC MURMUR that has a crescendo-decrescendo quality to it.

Other: pitch is medium, harsh. Heard best with patient sitting leaning forward.

Front 22

How would one describe a classic pulmonic stenosis?

(hint: systolic/diastolic , early/middle/pan, crescendo/decrescendo/crescendo-decrescendo)

Back 22

Pulmonic stenosis is a MIDSYSTOLIC MURMUR. There is a cresc-decresc quality.

Other: pitch medium, quality harsh. A splitting of the S2 (A2 then P2) can occur.

Front 23

How would one describe a classic aortic regurgitation?

(hint: systolic/diastolic , early/middle/pan, crescendo/decrescendo/crescendo-decrescendo)

Back 23

Diastolic murmur, early, decrescendo.

high pitched, blowing. If S3 or S4 is present, suggests severe regurgitaiton.

Front 24

How would one described a classic mitral stenosis?

(hint: systolic/diastolic , early/middle/pan, crescendo/decrescendo/crescendo-decrescendo)

Back 24

Diastolic murmur, opening snap , low pitched rumble. Is more of a decrescendo-crescendo pattern!

Front 25

Match the movement to its effect on the intensity of a murmur: hand grip, leg raise, squat, squat to stand, valsalva maneuver

1. Preload increases, murmur decreases
2. Preload decreases, murmur increases
3. Afterload increases, murmur decreases

Back 25

1. Preload increases, murmur decreases : leg raise, squat
2. Preload decreases, murmur increases : squat to stand, valsalva maneuver
3. Afterload increases, murmur decreases: hand grip

Front 26

The click heard in mitral valve prolapse is dynamic. If patient is squatting, would hear it in ______; supine in _______ and standing in __________.

Back 26

squatting would hear in late systole; supine would hear in mid systole, standing would hear in early systole

Front 27

Regarding the pathophysiology of cardiac causes of cyanosis, cyanosis due to cardiac lesions [ MAY / MUST ] involve right to left shunting and [ MAY / MUST ] involve obstruction to pulmonary blood flow.

Back 27

MUST involve L to R shunting;
MAY involve obstruction to pulmonary blood flow

Front 28

What are the four components of a tetralogy of fallot physiology?

Back 28

1. ventricular septal defect
2. pulmonary stenosis
3. aortic override
4. right ventricular hypertrophy

The VSD is NON restrictive. The direction and magnitude of flow through the VSD depends on the severity of the pulmonary stenosis. The greater the obstruction, the greater the R to L shunting. R to L shunting will occur with severe obstruction to right ventricular outflow.

Front 29

With transposition of the great arteries, the aorta arises entirely from the R ventricle and the pulmonary artery arises from the L ventricle. The dominant physiologic abnormality in transposition of the great arteries is a deficiency of oxygen supply to the tissues. The systemic and pulmonary circulations function in parallel rather than in series as in a normal infant. A small proportion of blood is exchanged by intercirculatory shunts between the two circulations. The two circulations are dependent on at least one of the following for proper viability...

Back 29

- an intracardiac path like a patent foramen ovale, ASD, VSD
- an extracardiac (patent ductus arteriosus)

Front 30

True or False:
Creating an atrial septal defect and maintaining ductal patency in transposition of the great arteries decreases both equally the effective pulmonary and systemic blood flow.

Back 30

FALSE.

It actually INCREASES both effective pulmonary and systemic blood flow. Thinkaboutit.... normally you have an atrial septal defect and you get a L to R shunt. BUT, with TGA, the aorta blood is the deoxygenated blood so you're increasing effective pulmonary blood flow with a patent ductus arteriosus in TGA. When you have an atrial septal defect too, that's also increasing the effective systemic blood flow!

Front 31

Nowadays corrective surgery for transposition of the great arteries involves...
A. anatomic repair
B. physiologic repair

Back 31

A. anatomic repair

done immediately after newborn period, the baby goes on bypass, the heart is stopped, the baby is frozen and the great vessels are switched and the coronary arteries are too

Front 32

What is the best example of a venous admixture lesion?

Back 32

The univentricular heart. Both atria drian into a common ventricle (mixing chamber) and the saturations of the blood leaving the heart via the pulmonary artery and aorta are nearly identical. Many heart defects that are more complicated anatomically have some admixture physiology. The degree of cyanosis or degree to which the blood is desaturated is directly related to the amount of obstruction to pulmonary blood flow.

Front 33

In venous admixture physiology (ex. where both atria drain into a common ventricle and the saturations of the blood leaving the heart via the pulmonary artery and aorta are nearly identical) have anatomic defects of tricuspid atresia, mitral atresia, double outlet right ventricle, and total anomalous pulmonary venous return. So, the degree of cyanosis or degree to which the blood is desaturated is related to pulmonary blood flow in what way?

Back 33

INVERSELY related to the amount of PULMONARY BLOOD FLOW

in other words, decreased pulmonary blood flow = increase cyanosis

Front 34

What is able to dilate a closing ductus arteriousus and maintain its patency?

Back 34

Prostaglandin E!

Front 35

True or False:
Regarding bare minimums, to survive in utero you just need one ventricle, one artery and a patent ductus arteriosus.

Back 35

TRUE!

Front 36

In which of the following cases is maintenance of a patent ductus arteriosus NOT necessary for adequate pulmonary blood flow?
A. severe Tetralogy of Fallot
B. Pulmonary Atresia with intact ventricular septum
C. Critical valvar pulmonary stenosis
D. Hypoplastic Left Heart
E. Tricuspid atresia

Back 36

D. Hypoplastic Left Heart (no L ventricle, no mitral valve, no aortic valve, no ascending aorta b/c it looks more like a coronary) - maintenance of PDA is essential in this case, but not for Pulmonary blood flow but instead for Systemic blood flow!

Front 37

In what congenital heart conditions (3 general categories and then name a few conditions) would prostaglandin E-1 (PGE-1) be utilized?

Back 37

"Cyanotic" CHD where PBF is dependent on PDA - as in severe Tetralogy of Fallot, Pulmonary atresia with intact ventricular septum, critical valvar pulmonary stenosis, etc.
"Acyanotic" CHD where SBF is dependent on PDA - as in hypoplastic L heart, crticial valvar aortic stenosis, critical coarctation of the aorta, interrupted aortic arch, etc.

PGE-1 maintains the patency of the ductus AND is a pulmonary and systemic vasodilator
Transposition of the great arteries

Front 38

Inhaled nitric oxide [ increases / lowers ] PVR without effecting SVR.

Back 38

lowers! remember, NO is a potent vasodilator!

Alternatively, CO2 increases PVR without effecting SVR.

Front 39

The combination of :
- signs and symptoms of Heart Failure
- normal systolic function
- atrial enlargement
are all hallmarks of what primary myocardial dz/ cardiomyopathy?

Back 39

There are three subdivs of cardiomyopathy/ primary myocardial dz:
- dilated (aka congestive)
- hypertophic
- restrictive

Described is what would be a hallmark of restrictive cardiomyopathy. Remember that restrictive cardiomyopathy is a diastolic dysfunction problem. Consistent elevated ventricular end diastolic pressures lead to BILATERAL atrial enlargement, and thus increased risk of atrial fibrilation.

Front 40

Why does restrictive cardiomyopathy yield normal ejection fractions but different cardiac outputs as compared to its regular heart equivalent?

Back 40

Restrictive cardiomyopathy is a diastolic dysfunction problem. There is decreased compliance of the ventricles thus less blood volume per cycle will pass through (thus lowered CO); but, remember as we said it is a diastolic dysfunction problem so the systolic side is just fine. Since ejection fraction is, just a fraction of the diastolic end volume, it doesn't matter that the EDV is less, the fraction ejected is still the same. Thus, restrictive cardiomyopathy yields normal EF but lowered CO.

Front 41

There are subsets of restrictive cardiomyopathy. For example, the rare dz _________ is accumulation of a protein mycopolysaccharide complex in the heart and elsewhere. Secondary types of this is associated with TB, RA, multiple myeloma and lymphoma.

Back 41

amyloidosis

Front 42

What restrictive cardiomyopathy does a patient probably have if their EKG doesn't match with their ECHO, or in other words their ECG voltages are much lower than expected given the L ventricle thickness?
A. Amyloidosis
B. Hypersensitivity Myocarditis
C. Loeffler's endomyocarditis
D. Sarcoidosis

Back 42

A. Amyloidosis

Front 43

What primary myocardial dz/ cardiomyopathy is characterized by decreased ejection fraction and decreased cardiac output?
A. Dilated CM
B. Hypertrophic CM
C. Restrictive CM

Back 43

A. Dilated CM

Remember, dilated CM is a systolic dysfunction issue. So it'd make sense that both EF and CO is decreased.

Front 44

With both dilated and restrictive cardiomyopathies, the physical exam shows low systolic BP and narrow PP and diminished pulses due to low CO. So what is a good way to clinically differeniate dilated from restricted?

Back 44

Dilated will have apex laterally displaced, reflecting L ventricular enlargement. Pulmonary component of S2 may be accentuated with pulmonary HTN. S3 and S4 might be present.

Front 45

Which are subsets of dilated cardiomyopathy. (4)
A. Adriamycin
B. Amyloidosis
C. Coxsackie virus
D. Heavy alcohol use over many years
E. Peripartum cardiomyopathy
F. Sarcoidosis

Back 45

A. Adriamycin (chemo-drug)
C. coxsackie virus (esp in young adults in US with DCM)
D. Heavy alcohol use over many years (primary myocardial dz in US seen in middle aged adults).
E. peripartum cardiomyopathy

Treatment for DCM is vasodilators and beta blockers, ACEi.

Front 46

In aortic regurgitation physical signs, the pulse pressure [ decreases / increases /stabilizes ] as the aortic regurgitation gets worse.

Back 46

pulse pressure INCREASES as aortic regurgitation gets worse. (systolic - diastolic pressure gets increasingly larger) p.334

Front 47

A newly occurring mitral stenosis (early on) yields a fibrosed valve (versus calcified immobile valve). Which of the following is characteristic (2)?
A. Absent opening snap
B. Opening snap present
C. Loud S1
D. Soft S1
E. Loud S2
F. Soft S2

Back 47

B. Opening snap present
C. Loud S1

Only after a long time (2+ decades of mitral stenosis) do you get calcified immobile mitral valve which yields the Soft S1 and absent opening snap.

Same applies for tricuspid valve.