Congenital Heart Conditions

Question 1

What type of shunt results in absent cyanosis?

Answer 1

Left to Right

Question 2

What are 3 Left to Right Shunts?

Answer 2

3D’s
- ASD
- VSD
- PDA

Question 3

Do Left to Right shunts typically present at birth?

Answer 3

NO – not causing cyanosis

Question 4

What is a potential late complication of Left to Right shunts?

Answer 4

Eisenmenger Syndrome
= Increased blood flow to the lungs results in pulmonary HTN and eventually causes reversal of the shunt to Right to Left shunt

Question 5

What is often heard with an ASD?

Answer 5

Wide and Fixed split S2

Question 6

With both ASD’s and VSD’s what type of diastolic sound can be heard?

Answer 6

Mid-diastolic rumbles

Question 7

What part of the heart may be enlarged with ASD’s?

Answer 7

Right atrial enlargement
Right ventricular hypertrophy

Question 8

What is the treatment for most ASD’s and VSD’s?

Answer 8

NOTHING
– often close spontaneously
– surgery if severe symptoms are occurring

Question 9

What is the most common type of congenital heart condition?

Answer 9

VSD

Question 10

What is unique about the sound of the VSD?

Answer 10

The size of the VSD is inversely proportional to the intensity of the murmur
– Smaller VSDs = LOUDER murmurs

Question 11

What is often heard with a VSD?

Answer 11

Harsh HOLOsystolic murmur at the lower left sternal border

Question 12

What part of the heart is often enlarged with VSD’s?

Answer 12

Left ventricular hypertrophy
+/- Right ventricular hypertrophy

Question 13

What does the PDA (patent ductus arteriosus) connect?

Answer 13

Aorta sends blood to Pulmonary A.

Question 14

When should the PDA close?

Answer 14

First few days of life

Question 15

If the PDA does not close, what type of murmur is often heard?

Answer 15

Continuous machinery like murmur at left 2nd ICS

Question 16

If the PDA does not close, what type of murmur is often heard?

Answer 16

Continuous machinery like murmur at the left 2nd ICS

Question 17

What are 2 other features that can be seen with PDA?

Answer 17

Wide pulse pressure
Bounding peripheral pulses

Question 18

What is the pneumonic to remember what the treatment is for a PDA?

Answer 18

Come IN and CLOSE the door

Question 19

What is the treatment in order to close the PDA?

Answer 19

Indomethacin (NSAID)

Question 20

If you want to keep the PDA open what should be given?

Answer 20

Prostaglandin E1

Question 21

Where is the aorta most often constricted with coarctation of the aorta?

Answer 21

Distal to the left subclavian artery

Question 22

What 2 things are often present with coarctation of the aorta?

Answer 22

Bicuspid aortic valve
Turner Syndrome

Question 23

With coarctation of the aorta and many other right to left shunt heart conditions, what is essential to survival?

Answer 23

PDA or a septal defect

Question 24

What is the main sign of Coarctation of the aorta?

Answer 24

UE HTN or uneven blood pressures between arms

Question 25

Besides UE HTN, what are 3 other signs of Coarctation of the aorta?

Answer 25

- Weak femoral pulses - LE claudication - Radiofemoral delay

Question 26

What are 2 items that can be seen on CXR with coarctation of the aorta? Why?

Answer 26

- Rib notching - 3 sign => Formation of collateral circulation

Question 27

If a patient is cyanotic with a heart condition, what type of shunt is present?

Answer 27

Right to Left

Question 28

How can you tell if a baby is cyanotic due to a heart condition or lung condition with supplemental oxygen?

Answer 28

Heart conditions hypoxemia will NOT correct with supplemental oxygen whereas lung conditions will

Question 29

What is a Breath Holding Spell?

Answer 29

Emotional trigger for a child causes loss of consciousness or cyanosis - Will resolve with time; benign condition

Question 30

List 5 Right to Left shunts

Answer 30

- Truncus Arteriosus - Transposition of great vessels - Tricuspid Atresia - Tetralogy of Fallot - Total anomalous pulmonary venous return

Question 31

What is the setup with Transposition of the great vessels?

Answer 31

Aorta connects to the RV and the Pulmonary A. connects to the LV -- BLOOD NEVER MIXES

Question 32

Transposition of the Great Vessels is not compatible with life unless?

Answer 32

A septal defect is present

Question 33

Will there be a murmur with Transposition of the Great Vessels?

Answer 33

Not unless there is a septal defect present

Question 34

What will the heart look like on CXR with Transposition of Great vessels?

Answer 34

Egg shaped heart with a narrow base

Question 35

What are 2 risk factors for development of Tetralogy of Fallot?

Answer 35

Maternal PKU DiGeorge Syndrome

Question 36

What are the 4 things present with Tetralogy of Fallot?

Answer 36

- Pulmonary stenosis - RV hypertrophy - Overriding aorta - VSD

Question 37

What are the 4 things present with Tetralogy of Fallot?

Answer 37

- Pulmonary stenosis - RV hypertrophy - Overriding aorta - VSD

Question 38

What defect with Tetralogy of Fallot is the Right to Left shunt

Answer 38

VSD

Question 39

The cyanosis will develop over the first 2 years of life with Tetralogy of Fallot along with?

Answer 39

Dyspnea and fatiguability

Question 40

Why may a child squat with Tet spells?

Answer 40

Squatting relieves the hypoxemia by increasing systemic vasculature pressure and forces blood into the lungs

Question 41

What will the heart look like on CXR with Tetralogy of Fallot?

Answer 41

Boot shaped heart

Question 42

What is Total Anomalous Pulmonary Return?

Answer 42

Right heart --> Lungs --> Right heart

Question 43

With a majority of the Right to Left shunt heart conditions what should be given before surgery can be done?

Answer 43

PGE1 to keep the PDA open

Question 44

What does uncontrolled maternal diabetes cause to the heart?

Answer 44

Transient Hypertrophic Cardiomyopathy

Question 45

If a child has a thickened interventricular septum due to maternal diabetes, what is the treatment?

Answer 45

Nothing -- will regress