Path: Chd; Myocardial Dis; Pulm Htn And Cor Pulm

Front 1

There is a hole within the membranous or muscular portions of the intraventricular septum that produces a left-to-right shunt, more severe with larger defects

Back 1

ventricular septal defect

Front 2

In the developing heart, the cavity of the primitive atrium becomes subdivided into right and left chambers by a septum, the______, which grows downward into the cavity. The _______, semilunar in shape, grows downward from the upper wall of the atrium immediately to the right of the primary septum and ostium secundum.

Back 2

septum primum; septum secundum

Front 3

The foramen ovale is aka ___________

Back 3

ostium secundum

Front 4

foramen ovale allows blood to enter the _____ from the ______

Back 4

Left atrium; Right atrium

This direction of flow is favorable only until birth because pulmonary pressures are so high (right side is higher pressure than left side, so it flows R --> L)

Front 5

The ductus arteriosis allows blood to enter the _______ from the _______

Back 5

aorta; Pulmonary artery

It allows most of the blood from the right ventricle to bypass the fetus's fluid-filled non-functioning lungs

Front 6

A hole from a septum secundum or septum primum defect in the interatrial septum produces a modest left-to-right shunt

Back 6

Atrial septal defect

Front 7

The ductus arteriosus, which normally closes soon after birth, remains open, and a left-to-right shunt develops

Back 7

Patent ductus arteriosus.


After birth, the flow through the ductus arteriosus is reversed because pulmonary pressures drop when the lungs inflate.

Front 8

Tetralogy of Fallot: what is it?

Back 8

a congenital heart defect which is classically understood to involve four anatomical abnormalities (although only three of them are always present).

Pulmonic stenosis results in right ventricular hypertrophy and a right-to-left shunt across a VSD, which also has an overriding aorta

Front 9

What are the 4 anatomical abnormalities in Tetralogy of Fallot?

Back 9

1) Pulmonary Artery stenosis: A narrowing of the right ventricular outflow tract

2) RV hypertrophy due to increased pressure that it must generate because of 1.)

3) Ventricular septal defect: results in R --> shunt because of high pressure to get blood into pulmonary circulation due to 1.)

4) Overriding aorta: aorta is positioned in such a way that it's receiving blood RIGHT OVER the VSD and/or RV.

Front 10

Most common cyanotic congenital HD

Back 10

Tetralogy of Fallot

Front 11

What happens in transposition of the great vessels?

Back 11

The aorta arises from the right ventricle and the pulmonic trunk from the left ventricle. A VSD, or ASD with PDA, is needed for extrauterine survival. There is right-to-left shunting.

Front 12

What happens in truncus arteriosus?

Back 12

There is incomplete separation of the aortic and pulmonary outflows, along with VSD, which allows mixing of oxygenated and deoxygenated blood and right-to-left shunting

Front 13

What is hypoplastic left heart syndrome?

Back 13

There are varying degrees of hypoplasia or atresia of the aortic and mitral valves, along with a small to absent left ventricular chamber

Front 14

What is coarctation of the aorta?

Back 14

Either just proximal (infantile form) or just distal (adult form) to the ductus is a narrowing of the aortic lumen, leading to outflow obstruction

Front 15

Why is probe patent foramen ovale in an adult not that big of a deal in normal healthy people?

Back 15

Normally the higher LA pressure keeps blood from going L->R

Front 16

How can probe patent foramen ovale become a problem?

Back 16

If pulmonary pressures rise (ie from pulmonary hypertension (as with pulmonary embolus), there can be a R->L shunt

Front 17

paradoxical embolus: defn

Back 17

Embolus from venous circulation ends up on the L side of heart (or even in arteries!) because it crossed over a patent foramen ovale.

Front 18

Congenital heart disease is usually due to faulty embroygenesis during gestational weeks _____ to ____

Back 18

3 - 8

Front 19

What are some environmental causes of congenital heart disease?

Back 19

Congenital rubella infection; thalidomide and alcohol exposure

Front 20

abnormal communications between vessels or chambers

Back 20

shunts

Front 21

failure of something to form

Back 21

atresia

Front 22

what are the two major categories of congenital heart disease?

Back 22

Cyanotic (venous blood gets into arterial circulation)

Noncyanotic (no venous blood getting into arterial blood)

Front 23

90% of the ventricular septal defects involve what portion of the septum?

Back 23

The membranous (as opposed to muscular) portion. This is because it's thinner.

Front 24

Most common form of CHD

Back 24

VSD

Front 25

T/F Signs, symptoms, and severity of VSDs depend on size

Back 25

T

Front 26

Small VSDs even though they might be tolerated well, place patient at risk for __________

Back 26

infectious endocarditis

Front 27

T/F Small VSDs may close spontaneously

Back 27

T

Front 28

Large VSDs present with a __ to ___ shunt

Back 28

L to R shunt (This is path of least resistance)

Front 29

Over time, what will happen in a large VSD?

Back 29

There will be L to R flow at first, which will increase pulmonary flow ---> this will increase pulm vascular resistance over time --> finally, the shunt will be reversed, R --L, which will cause--> CYANOSIS

Front 30

Over time, what can happen with all large L to R shunts?

Back 30

Same as last card.

There can be reversal of the shunt when the pulmonary resistance increases due to increased flow.

Reversal of the shunt (now a R->L shunt) will cause cyanosis.

Front 31

Large VSD may be associated with ____ valve insufficiency

Back 31

aortic.

Front 32

3 major types of atrial septal defect

Back 32

1) Secundum
2) Primum
3) Sinus venosus

Front 33

most common type of atrial septal defect

Back 33

ostium Secundum

Front 34

Eisenmenger's complex: defn

Back 34

pulmonary hypertension with increased pulmonary arterial pressures that eventually led to reversal and right-to-left shunt, resulting in marked right ventricular hypertrophy

Front 35

What causes the closing of the ductus arteriosus after birth?

Back 35

Usually closes in first couple of days due to increased O2 levels and decreased prostaglandin levels (prostaglandins keep it open)

Front 36

Signs of patent DA

Back 36

Harsh "machinery" murmur

Front 37

patent DA causes a __ to ___ shunt

Back 37

L->R

Front 38

In tetralogy of Fallot, what is crucial in determining clinical outcome?

Back 38

The degree of obstruction of RV outflow.

Front 39

There is hypertrophy of which heart chamber in tetralogy of Fallot?

Back 39

RV

Front 40

Aortic stenosis can be caused by a cusp abnormality where normally they're supposed to be ___ valves, but instead there are __ or ___

Back 40

3; 1 (unicuspid) or 2 (bicuspid)

Front 41

What is the problem with a bicuspid aortic valve?

Back 41

Most bicuspid valves are prone to calcification. Patients can remain relatively asymptomatic until the stenosis reaches a critical point when congestive heart failure rapidly ensues.

Front 42

What is Hypoplastic left heart syndrome?

Back 42

rare congenital heart defect in which the left ventricle of the heart is severely underdeveloped.

Front 43

Where is coarctation of the aorta usually present?

Back 43

Usually located at the level of the ductus or slightly proximal

Front 44

What happens if there is coarctation of the aorta proximal to the ductus AND a patent ductus?

Back 44

Normal arterial blood enters upper body (subclavian and brachiocephalic trunk has already branched off) but there will be cyanosis of lower trunk and extremities due to there being a R->L shunt (thru the patent DA) into the descending aorta

Front 45

When there is a coarctation of the aorta and the ductus is closed, what will be seen?

Back 45

prominent collateral arterial circulation (especially intercostal arteries which can cause notching of ribs due to increased prominence of these vessels).

Front 46

In transposition of the great vessels, what happens? What does survival depend on?

Back 46

The aorta is connected to the RV and the pulmonary artery is connected to the LV.

Survival depends on the presence of a PDA or a septal defect

Front 47

Congenitally corrected transposition of the great vessels: what happens?

Back 47

the primary arteries (the aorta and the pulmonary artery) are transposed, with the aorta anterior and to the left of the pulmonary artery; the morphological left and right ventricles are also transposed.

With l-TGA, blue blood is pumped from the right atrium into the morphological left ventricle (which lies on the right side of the heart), then through the pulmonary artery to the lungs. The red blood then returns, via the pulmonary veins, to the left atrium from which it is pumped into the morphological right ventricle, then through the aorta.

Front 48

Prevalence of Congenital Malformations of heart

Back 48

1% of live births

(40-50% if trisomy 21, 1 in 20 if mother has CHD; 1 in 39 if mother is diabetic; increased risk if father has CHD)

Front 49

Most common congenital malformation of heart

Back 49

Ventricular Septal Defects

Front 50

What type of cardiomyopathy:

All four chambers are dilated, and there is also hypertrophy

Back 50

Dilated

Front 51

What is Arrhythmogenic Right Ventricular Cardiomyopathy?

Back 51

Fat and fibrous tissue replace parts of RV wall, causing arrhythmias

Front 52

What are three morphologic/functional patterns of cardiomyopathy?

Back 52

1) Dilated
2) Hypertrophic
3) Restrictive

Front 53

What is most common cardiomyopathy?

Back 53

dilated

Front 54

Dilated cardiomyopathy is associated with (systolic, diastolic) dysfunction.

Back 54

systolic - problem contracting because it's big and floppy.

Front 55

Which chambers are affected in dilated cardiomyopathy

Back 55

All 4 are dilated and hypertrophied.

Front 56

What is the etiology of dilated cardiomyopathy?

Back 56

Idiopathic in ~50%

In others, there are genetic factors, including mutations in cytoskeletal proteins

Also alcohol, other drugs

Immunologic abnormalities: Ab against cardiac proteins

Front 57

What is peripartum cardiomyopathy?

Back 57

Dilated cardiomyopathy that occurs in last month of pregnancy or within 5 months postpartum. Cause unknown.

Front 58

What is the cause of patchy white areas seen when the ventricles are opened up in dilated cardiomyopathy?

Back 58

Reflects myocardium fibrosis.

Front 59

What's seen histologically in dilated cardiomyopathy?

Back 59

See myocyte hypertrophy with non-specific areas of interstitial fibrosis.

MUST exclude ischemic heart disease.

Front 60

Hypertrophic cardiomyopathy is associated with (systolic, diastolic) dysfunction.

Back 60

Diastolic.

It's a problem with less area available to be filled.

Front 61

What is the etiology of Hypertrophic cardiomyopathy ?

Back 61

Autosomal dominant disease of cardiac sarcomere (50%)

Mutations of proteins encoding myosin heavy chain, alpha-tropomyosin, and troponins have been ID'd.

Front 62

What's seen histologically in hypertrophic cardiomyopathy?

Back 62

See myocytes in thick, disorganized pattern with some interstitial fibrosis.

See thickened coronary arteries.

Front 63

hypertrophic cardiomyopathy: complications

Back 63

1) Atrial fib with thrombus/embolization

2) Infective endocarditis of MV

3) Cardiac failure

4) Sudden death with severe outflow obstructions

Front 64

What is restrictive cardiomyopathy?

Back 64

Decrease in ventricular compliance through a variety of reasons.

Front 65

Restrictive cardiomyopathy is associated with (systolic, diastolic) dysfunction.

Back 65

Diastolic. The problem is decrease in ventricular compliance, making filling harder. This decreases CO.

Front 66

Etiology of restrictive cardiomyopathy

Back 66

Primary form is rare. Dx of exclusion.

Front 67

What's seen histologically in restrictive cardiomyopathy?

Back 67

Interstitial fibrosis

Front 68

What is Loeffler endomyocarditis?

Back 68

Restrictive cardiomyopathy that is due to eosinophilic damage to heart.

Front 69

disease of the heart muscle which results in impaired filling of the heart ventricles during diastole.

Back 69

restrictive cardiomyopathy

Front 70

Complications of Loeffler endomyocarditis

Back 70

Mural thrombi/emboli.

Front 71

What is endomyocardial fibrosis?

Back 71

Fibrosis of subendocardium and endocardium. Possibly end-stage disease of Loeffler endomyocarditis

Front 72

What population is primary affected by endomyocardial fibrosis?

Back 72

Children and young adults in Africa

Front 73

What is Endocardial Fibroelastosis?

Back 73

thickening of the inmost lining of the heart chambers (the endocardium) due to an increase in the amount of supporting connective tissue and elastic fibers.

Front 74

Who gets Endocardial Fibroelastosis?

Back 74

children less than 2 yo

Front 75

What are changes seen grossly in Endocardial Fibroelastosis?

Back 75

endocardium was pearly white or opaque instead of normally thin and transparent and microscopically showed a systematic layering of collagenous and elastic fibers.

Front 76

Etiology of Endocardial Fibroelastosis

Back 76

A variety of causes of stress - infections, cardiomyopathies, immunologic diseases, congenital malformations, even elecrocution by lightning strike. EFE has two distinct genetic forms, each having a different mode of inheritance.

Front 77

Viral myocarditis is realted to what viruses?

Back 77

Coxsackie A and B, ECHO, polio, influenza A and B

Front 78

What is seen histologically in viral myocarditis?

Back 78

Infiltration of lymphocytes, necrosis of myocytes.

MUST seen damage done to myocytes

Will see edematous mononuclear cells.

Front 79

What is hypersensitivity myocarditis?

Back 79

Infiltration by mostly eosinophils due to reaction to drugs.
Necrosis of myocytes is usually minimal.

Front 80

Giant Cell Myocarditis: what is it?

Back 80

Idiopathic myocardial necrosis and infiltration by lymphocytes, plasma cells, eosinophils, and giant cells.

Most cases are rapidly fatal without heart transplant

Front 81

Giant Cell Myocarditis: who gets it

Back 81

Young to middle-aged adults

Front 82

What is a "giant cell?"

Back 82

A giant cell is a mass formed by the union of several distinct cells (usually macrophages). It can arise in response to an infection or foreign body.

Front 83

What heart problems can sarcoidosis cause?

Back 83

Dysrythmias, CHF, sudden death.

See restrictive OR dilated changes.

Front 84

What does cardiac sarcoidosis look like histologically?

Back 84

See granulomas, fibrous scarring, but usually no necrosis.

Front 85

amorphous deposits of pale pink material between myocardial fibers.

Back 85

cardiac amyloidosis

Front 86

What type of cardiomyopathy does amyloidosis cause?

Back 86

Restrictive

Front 87

Plasma cell dyscrasias: defn

Back 87

cancer of plasma cell.

Plasma cell dyscrasias are produced as a result of malignant proliferation of a monoclonal population of plasma cells that may or may not secrete detectable levels of a monoclonal immunoglobulin or paraprotein commonly referred to as M protein.

Front 88

What is the association of plasma cell dyscrasia with cardiac amyloidosis?

Back 88

one-third to one-half of patients with amyloidosis associated with plasma cell dyscrasia have cardiac symptoms

Front 89

Congo red stain: how does amyloid appear? With polarized light?

Back 89

The amyloid stains orange-red, but with polarized light, the amyloid has an "apple-green" birefringence.

Front 90

Decreases in the following cause what:

Prostacyclin, NO, Thrombomodulin, Heparin sulfate, Plasminogen activator

Back 90

Vasoconstriction (normally these cause vasodilation)

Front 91

Increases in the following cause what:

Endothelin; Plasminogen Activator Inhibitor

Back 91

Vasoconstriction

Front 92

What vessels are usually the most prominently altered in pulmonary HTN?

Back 92

Usually small arteries and arterioles

Front 93

T/F The presence of high grade lesions correlates with irreversibility in pulmonary HTN

Back 93

T

Front 94

Acute cor pulmonale (eg., from a saddle embolus): what happens?

Back 94

Dilation of right ventricle

Front 95

Chronic cor pulmonale: what happens?

Back 95

Hypertrophy of right ventricle