1.2.3 Congenital Heart Disease (CHD)

Question 1

The acyanotic L-R shunt associated with VSD puts patients at increased risk for?

Answer 1

Pulmonary hypertension

Question 2

What is a patent ductus arterosis?

Answer 2

Failure of ductus arteriosus to close. This results in left to right shunt between aorta and the pulmonary artery

Question 3

What are the three main examples of left to right shunt congenital heart diseases?

Answer 3

ASD, VSD and PDA

Question 4

What are the main clinical features of Tetrology of Fallot?

Answer 4

P - Pulmonary Stenosis

R - Right ventricular hypertrophy

O - Overriding of the aorta

V - Ventricular Septal defect

Question 5

What is the most common type of ASD?

Answer 5

The septum secundum is a subsequent membranous ingrowth located to the right and anterior of the septum primum.

Question 6

What can induce closure of a PDA?

Answer 6

Indomethacin

Question 7

This “boot shaped” image of the heart is due to what disease?

Answer 7

Tetralogy of fallot - he heart is typically enlarged and is classically “boot-shaped” due to marked right ventricular hypertrophy. The VSD is usually large with the aortic valve at the superior border, thereby overriding the defect and both ventricular chambers. The obstruction to right ventricular outflow is most often due to narrowing of the infundibulum (sub-pulmonic stenosis) but can be accompanied by pulmonary valvular stenosis. Sometimes there is complete atresia of the pulmonary valve and variable portions of the pulmonary arteries, such that blood flow through a PDA, dilated bronchial arteries, or both, is necessary for survival. Aortic valve insufficiency or an ASD may also be present; a right aortic arch is present in about 25% of cases.

Question 8

What is a tetralogy spell?

Answer 8

A sudden increased constriction of the out flow tract to the lungs

Question 9

What disease is this an image of?

Answer 9

Transposition of the Great Vessels

Question 10

What common genetic syndrome tends to be associated with CHD?

Answer 10

Down syndrome

Question 11

When can a diagnosis of Transposition of the Great Vessels be made?

Answer 11

Within the first hours or days of life. This patient will have severe cyanosis due to circulation running in parallel.

Question 12

How will a patient with Tetrology of Fallot present in clinic?

Answer 12

This patient will have a loud murmur in the first few weeks of life or this patient will have cyanosis. These patients may also have rapid breathing in response to low O2 levels.

Question 13

With the normal on the left, explain what the disease is and what is occuring in the image.

Answer 13

This is VSD. Blood from the LV is under higher pressure, so some moves to the RV, which overloads the pulmonary circulation, and may eventually lead to pulmonary hypertension. RV hypertrophy from volume overload, and LV hypertrophy (because it’s pumping more blood than normal, because of the defect)

Question 14

How will coarctation of the Aorta present clinically?

Answer 14

These patients will have hypertension in the upper parts of the body and hypotension in lower parts. Anastomoses will develop between subclavian artery and aorta through the intercostal arteries. Notching of the ribs.

Question 15

What will occur in patients with a Patent Ductus Arteriosus?

Answer 15

These patients will present with clubbing of toes not fingers. This is due to shunting of hypoxemic blood from PA to descending aorta.

Question 16

What is a VSD?

Answer 16

An incomplete closure of the ventricular septum. This accounts for roughly 40% of CHDs.

Question 17

What is this an image of?

Answer 17

Ventricular septal defect, muscular wall of the septum

Question 18

What is this an image of?

Answer 18

A ventricular septal defect (membranous type)

Question 19

When is the most likely age of presentation of ASD?

Answer 19

Patients usually do not present until age 30

Question 20

What is the special murmur associate with PDA?

Answer 20

Machinery murmur

Question 21

What rare syndrome is associated with CHD?

Answer 21

DiGeorge syndrome

Question 22

What might you want to do in regards to the ductus arteriosus in a patient with TOF?

Answer 22

You may not want their ductus arteriosus to close. May want to give these patients prostaglandin to keep it open.

Question 23

In a patient with TOF what will happen if they are given extra oxygen?

Answer 23

This will have little change.

Question 24

When does a PDA tend to close?

Answer 24

It closes functionally within 10-15 hours of birth. Anatomically it closes 2-3 days after (fusion)

Question 25

This image is associated with which CHD?

Answer 25

Tetralogy of fallot

Question 26

Eisenmengers syndrome is a result of?

Answer 26

Pulmonary vascular disease that develops in patients with large unclosed VSDs. This can lead to shunt reversal then cyanosis and eventually death.

Question 27

What provides blood to the lungs and reduces cyanosis in patients with TOF?

Answer 27

PDA

Question 28

What determines the severity of TOF?

Answer 28

Clinical severity of the disease depends on the extent of pulmonary stenosis.

Question 29

With the normal being on the left what is the disease occurring in the right image?

Answer 29

This is an image of a Patent Ductus Arteriosus

Question 30

Patients with a VSD are at increased risk for?

Answer 30

Pulmonary hypertension

Question 31

What is this an image of ?

Answer 31

Notching of the ribs associated with Coarctation of the Aorta.

Question 32

What aortic stenosis caused by?

Answer 32

This tends to be caused by a bicuspid aortic valve instead of a tricuspid valve. This leads to microtrauma to the valve.

Question 33

What is a paradoxical emboli?

Answer 33

This is a emboli that has crossed an ASD

Question 34

What are the main examples of right to left shunt congenital heart diseases?

Answer 34

Tetralogy of fallot and transposition of the great arteries

Question 35

What is coarctation of the Aorta?

Answer 35

This is a narrowing of the aorta. Infantile forms present as lower extremity cyanosis. Adult forms have hypertension in upper extremeties and hypotension in lower.