Primer 9 - Congenital Heart Disease

Question 1

What is an Ebstein anomaly?

Answer 1

Congenital defect when tricuspid leaflets are displaced into right ventricle, hypoplastic right ventricle, tricupid regurg or stenosis. Dilated right atrium causing increased risk of SVT and WPW.

Around 80% have a patent foramen ovale with a Right to Left shunt.

Question 2

What is a left-to-right shunt?

Answer 2

Congenital anomaly that causes the blood flow from high pressure of the left (systemic) to go to the right low pressure (pulmonary system).

Question 3

What are the 3 main left-to-right shunts?

Answer 3

1. Atrial septal defect (ASD)
2. Ventricular spetal defect (VSD)
3. Patent ductur arteriosus (PDA)

Question 4

What is the most common type of atrial septal defect and why is it caused?

Answer 4

A patent foramen ovale. A hole in the atrium-atrium chambers, due to either the osteum secundum or the foramen ovale are just too big, they overlap, and they cannot close.

Question 5

What pharmacological use is associated with Ebstein anomaly?

Answer 5

Associated with maternal lithium use.

Question 6

How does an Atrial septal defect sound like?

Answer 6

Loud S1 and wide, fixed splitting S2.

Question 7

What is the most common type of congenital heart defect?

Answer 7

Ventricular septal defect.

Question 8

How does a Patent ductus arteriousus sound like?

Answer 8

A continuous machine-like murmur.

Question 9

When does the Patent ductus arteriosus is supposed to close?

Answer 9

Within the first 24 hours of life.

Question 10

What is the treatment of Patent Ductus Arteriosus?

Answer 10

Indomethacin: blocks prostaglandin synthesis.

Question 11

When should we keep the Patent Ductus Arteriousus and what can we use to keep it open?

Answer 11

In certain right to left shunts such as transposition of the great vessels, use prostaglandins to keep it open.

Question 12

What is Eisenmenger syndrome?

Answer 12

Severe longstanding uncorrected left to right shunts leads to this syndrome. A severe left to right shunt leads to an overflow into the pulmonary circulation, creating pulmonary hypertension. Since the right ventricle needs to work harder to pump against pulmonary resistance, leading to right ventricle hypertrophy. Once right ventricle pressure goes up enough to exceed the left side of the heart, the shunt switches to right to left shunt.

Question 13

What is coarctation of the aorta and what are the two types.

Answer 13

Focal circumferential narrowing of the aorta. The adult distal to the ductus arteriosus. The infantile type is coarctation proximal to the insertion of the ductus arteriosus.

Question 14

Which syndrome is related to the coarctation of the aorta?

Answer 14

Turner syndrome.

Question 15

What do we see in X-ray in coarctation of the aorta?

Answer 15

Notching of the arteries of the ribs on chest x-ray due to intercostal arteries becoming dilated, carving indentation on the inferior aspect of the ribs.

Question 16

What three cardiac pathologies are associated with Turner syndrome?

Answer 16

Infantile coarctation, bicuspid aortic valves, and infective endocarditis.

Question 17

What heart pathologies are associated with Down Syndrome?

Answer 17

Endocardial cushion defect.

Question 18

What heart pathology is associated with DiGeorge syndrome?

Answer 18

Tetralogy of Fallot or Truncus arteriosus.

Question 19

What heart pathology is associated with Congenital rubella?

Answer 19

PDA or Pulmonary artery stenosis.

Question 20

What heart pathology is associated with Aortic insufficiency?

Answer 20

Marfan syndrome.

Question 21

What happens in Right to left shunt?

Answer 21

Blood is being shunted from deoxygenated right side to the left side, bypassing the lung circulation.

Question 22

What are the five causes of Right to left shunts AKA cyanotic diseases?

Answer 22

1. persistent Truncus arteriosus, 2. Transposition of the great vessels, 3. Tricuspid atresia, 4. Tetralogy of Fallot, 5. Total anomalous pulmonary venous return.
   - They all start with T.
   - The 5 Ts.

Question 23

What is Persistent Truncus Arteriosus?

Answer 23

Failure of the trucus arteriosus to divide into the aorta and pulmonary trunk, mixing deoxygenated blood mixing in the circulation.

Question 24

What is transposition of the great vessels?

Answer 24

When the aorta and the pulmonary artery have been switched; the left ventricle pumps into the pulmonary circulation and the right ventricle into the circulation. This creates two completely closed groups, needing a shunt to be compatible with life.

Question 25

What pathologies are fetus of diabetic mothers more likely to suffer?

Answer 25

Transposition of the great arteries (TGA), shoulder dystocia (shoulder gets caught under the maternal pelvic bone), large for gestacional age (LGA).

Question 26

What is tricuspid atresia?

Answer 26

When the tricuspid valve does not develop. There is no opening from the right atrium to the right ventricle. Needs two shunts to be compatible with life (ASD, VSD).

Question 27

What are the two shunts needs for the fetus to be compatible with life?

Answer 27

Atrial septal defect and ventricular septal defect.

Question 28

What is Ebstein anomaly?

Answer 28

It is when tricuspid leaflets are displaced inferior into right ventricle, there is hypoplastia of right ventricle and tricuspid regurgitation or stenosis. Their is 80% of patients have a patent foramen ovale with right to left shunt.

Question 29

What increased risk does the dilated right atrium give in patients with Ebstein anomaly?

Answer 29

Increased risk of Supraventricular tachycardia (SVT) and Wolf-Parkinson-White (WPW).

Question 30

What do we find in the physical exam of the heart of someone with Ebstein anomaly?

Answer 30

Widely split S2 and tricuspid regurgitation murmur.

Question 31

What are the 4 defects of Tetralogy of Fallot?

Answer 31

[PROVe]

1.Pulmonary valvular stenosis. 2. Right ventricular hypertrophy. 3.Ventricular septal defect. 4. Overriding aorta.

Question 32

Why do kids with Tetralogy of Fallot squat?

Answer 32

Kids with Tet of Fallot have left to right shunt which is acyanotic, but there may be episodes when it turn to right to left shunt like when the cry or exercise, and become cyanotic. Older kids learn to squat on these cyanotic spells because squating increases the systemic vascular resistance, forcing it back into left to right shunt.

Question 33

What is the classic X-ray finding in tetralogy of Fallot?

Answer 33

The boot-shaped heart due to right ventricular hypertrophy.

Question 34

What is Total anomalous pulmonary venous return?

Answer 34

Where the pulmonary veins drain into the systemic circulation; instead of oxygenated blood coming from the pulmonary veins into the left atrium, it might empty to the superior vena cava, or the coronary sinus, but at the end empties into the right atria; a completely closed loop, needing a type of shunt to live.

Question 35

What is the most common congenital cardiac anomaly?

Answer 35

Ventricular septal defect.

Question 36

Explain how the great vessels are attached in a transposition of the great vessels?

Answer 36

The right ventricle is connected into the aorta and the left ventricle is connected to the pulmonary trunk.

Question 37

A 45 y.o. man presents with a BP of 160/90 on the right arm and 170/92 on the left arm,. There are no palpable pulses in the feet/ankle. What problem does this patient most likely have?

Answer 37

Coarctation of the aorta: the hypertension of the upper extremities and hypotension in lower extremities.

Question 38

RFF: Continuous machinery-like heart murmur.

Answer 38

Patent ductus arteriosus.

Question 39

RFF: “Boot shaped heart”

Answer 39

Tetralogy of Fallot.

Question 40

RFF: Rib notching.

Answer 40

Coarctation of the aorta.

Question 41

RFF: Most common congenital cardiac anomaly.

Answer 41

Ventricular septal defect.

Question 42

RFF: Most common congenital cause of early cyanosis.

Answer 42

Wdxw

Question 43

Describe blood flow through a PDA.

Answer 43

Normally, in utero the blood flows thru the ductus arteriosus from right to left shunt, from pulmonary artery to aorta, bypassing pulmonary circulation. In a Patent ductus arteriosus, is going from the aorta to the pulmonary artery, causing a left to right shunt.