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Flashcards in Congenital Heart Disease Deck (21)
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1

What type of shunts do the following have?
-Acyanotic CHD "pink babies"
-Cyanotic CHD "blue babies"
-Obstructive CHD

-Acyanotic CHD "pink babies"
--left to right shunt

-Cyanotic CHD "blue babies"
--right to left shunt

-Obstructive CHD
--narrowing structures (not necessarily a shunt)

2

What is Acyanotic CHD?
What are the types?

What is an Acyanotic CHD?
-left to right cardiac shunt; blood from the systemic arterial circulation mixes with systemic venous blood

What are the types?
-Atrial septal defect
-ventricular septal defect
-patent ductus arteriosus

3

What is Cyanotic CHD?
What are the types?

What is Cyanotic CHD?
-right to left shunt; shunt allows blood to flow from the right to left side of the heart

What are the types?
-tetralogy of fallot
-transposition of the great arteries

4

What is Obstructive CHD?
What are the types/examples?

What is Obstructive CHD?
-obstructive...

What are the types/examples?
-obstruction to right side of heart
--pulmonic valve stenosis
-obstruction to left side of the heart
--coarctation of the aorta
--congenital aortic stenosis

5

In fetal circulation, how many arteries and veins enter/exit through the umbilicus?

2 arteries and one vein

6

Explain fetal circulation

Blood from umbilical vein (through the placenta) feeds into the right atrium which supplies the right ventricle and the left atrium and ventricle through the foramen ovale. Blood is pushed from the right ventricle to the pulmonary trunk which feeds the lungs and the ductus arteriosus. Blood from the left ventricle is pushed into the aorta where the blood from the ductus arteriosus mixes with it and supplies the rest of the body.

7

Why/how do the foramen ovale and ductus arteriosus close?

When breathing begins, there is a decrease in the pulmonary arterioles constriction and decrease pulmonary vascular resistance.

This causes pulmonary resistance to fall below systemic circulation which changes the blood flow across the ductus arteriosus to a left right shunt. the change in pressure closes the FO and DA.

8

What is an Atrial Septal Defect (ASD)?
What are the types?

What is an Atrial Septal Defect (ASD)?
-opening in the atrial septum permitting blood between the two atrias

What are the types?
-ostium secundum (most common) (right in the middle of the septum)
-sinus venus
-ostium primum

9

ASD
clinical presentation
dx
tx

clinical presentation
-most often have CV sx
-rarely CHF
-heart is usually hyperactive with a right ventricular heave felt best at lower left sternal border
-S2 is widely split and fixed at a pulmonary area

dx
-Initial testing is usually CXR and EKG. Echo is the test of choice

tx
-surgical
-percutaneous transcatheter closure

10

Ventricular Septal Defect (VSD)
what is it?
what are the types?
clinical presentation
dx
tx

what is it?
-an abnormal opening in the ventriclar septum which allows blood to flow across the right and left ventricles

what are the types?
-membranous defect
-subpulmonic or outlet defect
-AV or Inlet defect
-muscular defect

clinical presentation
-small to moderate shunt usually have no CV sx
-small to moderate shunts have a loud, harsh holosystolic murmur
-Large shunts: frequent resp infections, poor weight gain, dyspnea and fatigue, pansystolic murmur, CHF develops between 1-6 months

dx
-initial CXR and EKG
-echo confirms dx, location of the lesion, and size of shunt (right atrium will be big from the back up)

tx
-small to moderate shunts in asymptomatic patients do not require surgery or other intervention
-medical management
-transcatheter closure
-moderate to large shunts need surgical repair

11

What is the most common reason for an infant to see a cardiologist?

VSDs

12

Patent Ductus Arteriosus
what is it?
who is it common in?
clinical presentation
dx
tx

what is it?
-a normal fetal vessel that connects the pulmonary artery to the aorta, and its patent

who is it common in?
-preterm infants

Clinical presentation
-usually asymptomatic unless large, LV failure or pulmonary htn can develop
-continuous rough "machinery" murmur accentuated in late systole at time of S2, heard at left sternal border at the first and second interspaces
-fast breathing, poor growth

Dx
-CXR, EKG, echo confirms dx

Tx
-in preterm infants, Indomethacin (a prostaglandin inhibitor) is used to close the shunt
-cardiac cath with coils or occluder devices
-large shunts require surgical ligation

13

In cyanotic disease, would you want to keep the abnormality open or close it? Acyanotic disease?

In cyanotic disease, you would want to keep it open because that is keeping them alive.
In acyanotic disease, you would need to close it.

14

Tetralogy of Fallot
What is this?
what is it associated with?
clinical presentation
Dx
Tx

What is this?
-condition of several congenital defects that occur: VSD, pulmonary obstruction (narrowing of the pulmonary valve), overriding aorta (displacement of the aorta over the VSD), RVH

what is it associated with?
-cleft palate, hypospadias, skeletal malformations

clinical presentation
-cyanotic
-fatigue and dyspnea
-tet spell (Sometimes, babies who have tetralogy of Fallot will suddenly develop deep blue skin, nails and lips after crying or feeding, or when agitated. These episodes are called tet spells and are caused by a rapid drop in the amount of oxygen in the blood.)
-harsh systolic ejection murmur

Dx
-CXR, may have boot shaped heart
-EKG
-Echo
-cardiac cath

Tx
-immediate: prostaglandins
-surgical: temporary placement of blalock-taussig shunt or total repair

15

Transposition of the Great arteries
what is this?
who is it common in?
clinical presentation
Dx
Tx

what is this?
-the aorta comes off the right ventricle and pulmonary artery comes off the left ventricle

who is it common in?
-males 3:1

clinical presentation
-cyanotic
-poor feeders
-fatigue

Dx
-CXR, "egg on a string" appreance
-EKG: little help
-Echo: usually diagnostic
-cardiac cath: rashkind balloon

Tx
-prostaglandins to keep ductus open
-atrial septostomy: rashkind balloon (A procedure in which the formamen ovale is expanded by a balloon to allow free mixing of blood. )
-arterial switch operation at 4-7 after birth

16

Pulmonic Valve Stenosis
What is this?

obstruction of blood flow from the right ventricle to the pulmonary artery
-obstruction usually caused by narrowing

Areas of narrowing:
-thickened muscle below pulmonary valve
-stenosis of valve itself (most common)
-stenosis of pulmonary artery above the valve

17

Pulmonic Valve Stenosis
Clinical Presentation
Dx
Tx

Clinical Presentation
-usually asymptomatic
-newborn: cyanotic
-older child: rough ejection systolic murmur and ejection click

Dx
-echo
-cardiac cath

Tx
-mild stenosis rarely need intervention
-moderate/severe: require tx with balloon valvuloplasty or surgery

18

Coarctation of the Aorta
what is this
who is this common in?
clinical presentation
Dx
Tx

what is this
-narrowing at the aorta most commonly at the juxtaductal aorta

who is this common in?
-males, associated with turners syndrome

clinical presentation
-usually presents at days 4-10
-cardinal finding is decrease or absence of femoral pulses
-can have a "blowing systolic murmur" heard at left axilla and left back that is pathognomonic

Dx
-CXR
-notching of ribs (not usualy visible until age 5)
-EKG (not much help)
-Echo (confirm dx)

Tx
-prostaglandins
-primary balloon aortoplasty
-surgery to repair defect

19

What is the difference between coarctation and stenosis?

coarctation is narrowing in the blood vessel
stenosis is narrowing of valves

20

Congenital Aortic Stenosis
What is this
what are the types
clinical presentation
Dx
Tx

What is this
-obstruction of blood outflow between LV and aorta

what are the types
-valvular aortic stenosis (most common)
-subvalvular aortic stenosis
-supravalvular aortic stenosis

clinical presentation
-usually asymptomatic until severe
-systolic ejection murmur
-rapid breathing, chest pain, lightheadedness

Dx
-CXR and EKG: may show LVH
-Echo: most diagnostic

Tx
-cardiac cath
-surgical repair or replacement
--ross procedure

21

What is the ross procedure?

a cardiac surgery operation where a diseased aortic valve is replaced with the person's own pulmonary valve. A pulmonary allograft (valve taken from a cadaver) is then used to replace the patient's own pulmonary valve.