Pathoma - Congenital Cardiac Defects - Fundamentals Of Pathology

Front 1

When do congenital defects usually arise?

Back 1

Arise during embryogenesis. Usually weeks 3-8.

Front 2

Congenital defects are seen in about _________ of live births.

Back 2

1%

Front 3

Most defects that are formed in embryogenesis are ______.

Back 3

sporadic

Front 4

A congenital heart defect often results in ______________.

Back 4

shunting between left and right circulations

Front 5

What is a VSD?

Back 5

Defect in the septum that divides the right and left ventricles.

Front 6

This is the most common congenital heart defect.

Back 6

VSD

Front 7

What heart defect is associated with this?

Back 7

VSD

Front 8

What can a VSD lead to, if it's severe enough? Consequences?

Back 8

Eisenmenger's syndrome:

Blood flows into right ventricle (L-->R shunt) and into the pulmonary vasculature. In time, pulmonary hypertension develops. This reverses the shunt. Blue blood enters the left ventricle producing cyanosis.

Right ventricular hypertrophy
Cyanosis may cause polycythemia.
Clubbing due to cyanosis

Front 9

What determines when a VSD presents?

Back 9

(1) Size of defect determines extent of shunting
(2) Age at presentation

Front 10

VSD defects that are small often are ___________.

Back 10

asymptomatic

Front 11

Treatment of VSD?

Back 11

(1) Surgical closure
(2) Small defects may close spontaneously

Front 12

What is the MC type of ASD?

Back 12

Most common type is ostium secundum

Front 13

Bonus: Triple marker for Down syndrome?

Back 13

(1) AFP decreased
(2) hCG increased
(3) Urine estriol decreased

Front 14

A person with Down syndrome would have what heart defect?

Back 14

ASD; Ostium primum type.

Front 15

You auscultate a person with Down syndrome. You hear a split S2. What is going on and why a split S2?

Back 15

ASD.

The extra blood volume coming from the RA will cause a delayed closure of the pulmonic valve.

Front 16

A lazy down syndrome patient is known to have DVT. All of a sudden he develops a hemiparesis and complains of loss of sensation in the face. What might be going on?

Back 16

Paradoxical embolus via ASD to lodge in MCA.

Front 17

What is PDA?

Back 17

Patent ductus arteriosus. Failure of it to close.

Front 18

PDA is associated with?

Back 18

Congenital rubella

Front 19

Consequences of PDA?

Back 19

Like in ASD, blood will flood the pulmonary circuit causing pulmonary HTN. It will reverse the shunt. Deoxygenated blood is now in the systemic circuit and goes to the LOWER extremities (later in life).

Front 20

A baby is born. On auscultation you hear a holosystolic 'machine-like' murmur. What might be going on?

Back 20

PDA

Front 21

PDA is ___________ (symptomatic/asymptomatic) at birth.

Back 21

asymptomatic

Front 22

Treatment of PDA?

Back 22

Indomethacin. Decreases PGE2, resulting in PDA closure.

("Prostaglandin E is necessary to kEEEEEEEEEP it open")

Front 23

How many problems do we have in tetralogy of fallot?

Back 23

Four

Front 24

What defects are there in tetralogy of fallot?

Back 24

(1) Stenosis of RV outflow tract
(2) RV hypertrophy
(3) VSD
(4) Aorta that overrides the VSD

Front 25

What shunt are babies with tetralogy of fallot born with?

Back 25

R-->L

Early cyanosis is seen in these babies.

Front 26

The extremities of a child becomes blue during exercise. What do we call this and what could the child do to alleviate this?

Back 26

Cyanotic spell

Squat down to increase systemic bp and reverse the shunt temporarily.

Front 27

This is the MC cyanotic CHD.

Back 27

Tetralogy of fallot

Front 28

What is this?

Back 28

A typical heart shape seen on X-ray in babies with tetralogy of fallot.

Front 29

What is transposition of the great vessels?

Back 29

A CHD where the pulmonary artery arises from the LV and aorta arises from the RV.

Front 30

A mother has maternal diabetes. What is the child at risk for?

Back 30

Transposition of the great vessels

Front 31

What is the major problem in transposition of the great vessels?

Back 31

Two separate circuits

Front 32

You decide to close the PDA in a child with transposition of the great vessels. Why is this wrong?

Back 32

PDA is the only connection that allow mixing of blood between the two independent circuits.

Front 33

How does transposition of the great vessels present?

Back 33

Presents with early cyanosis (like tetralogy, however, tetralogy is the MCC).

Front 34

What is truncus arteriosus? What is it due to?

Back 34

When you have one large trunk (vessel) arising from both ventricles.

It is due to the truncus failing to divide.

Front 35

How does truncus arteriosus present?

Back 35

With early cyanosis.

Deoxygenated blood from the RV mixes with the oxygenated blood from the left ventricle before the independent circuits separate.

Front 36

What is tricuspid atresia?

Back 36

Tricuspid valve orifice fails to develop (atresia means failure to form the lumen of a tube)

Front 37

Features of tricuspid atresia?

Back 37

(1) Early cyanosis
(2) RV hypoplasia

Front 38

What is coarctation of the aorta?

Back 38

Narrowing of the aorta

Front 39

How is coarctation of the aorta divided?

Back 39

(1) Infantile form
(2) Adult form

Front 40

The infantile form of coarctation of the aorta is associated with what?

Back 40

PDA

Front 41

In the infantile form, where does the coarctation occur?

Back 41

Distal to the aortic arch but proximal to the PDA.

Front 42

Why would a baby with coarctation of the aorta present with lower extremity cyanosis?

Back 42

Because coarctation causes a distally lower blood pressure than normal. This coupled with an intact PDA which is often seen in this context, allows for some blood from the RV and pulmonary trunk to mix and go to the extremities.

Front 43

Bonus: How can you easily remember chromosomal number i turner and down syndrome?

Back 43

Front 44

What heart defect is associated with this patients syndrome?

Back 44

Infantile form of coarctation of the aorta

Front 45

True or false: The adult form of coarctation of the aorta is associated with PDA.

Back 45

False, it is not associated with a PDA.

Front 46

How does the adult form of coarctation of the aorta present?

Back 46

Presents as HTN in the upper extremities and hypotension with weak pulses in the lower extremities.

Front 47

When is adult coarctation of the aorta classically discovered?

Back 47

During adulthood.

Front 48

Adults with coarctation of aorta are at risk for what types of aneurysm?

Back 48

(1) Berry aneurysms

Front 49

A man with pain in buttocks and calves when walking is found to have coarctation of the aorta. What other heart defects may this man have? The man is at risk for what complication due to this?

Back 49

Bicuspid valve.

Bicuspid aortic valve increases risk and hastens disease onset of aortic stenosis (two valves has to do the work of three). AS is caused by age-related degenerative calcific changes of the valve ("wear and tear").

Front 50

What might develop in a patient with coarctation of the aorta?

Back 50

Collateral circulation can develop across intercostal arteries. Engorged arteries cause notching of ribs on x-ray.

Front 51

What congenital heart defect may this patient have?

Back 51

We see notching of the ribs, suggesting engorged vessels due to coarctation of the aorta.

Front 52

What may happen to the aorta in coarctation of the aorta?

Back 52

Valve ring and aorta may dilate. Risk for dissection.