Cardiology: Aortic & Pulmonary Valve Stenosis, Ebstein's

Question 1

What is congenital aortic valve stenosis?

Answer 1

Patients are born with a narrow aortic valve that restricts blood flow from the left ventricle into the aorta.

The severity of the stenosis varies between patients and will determine the symptoms.

Question 2

How many leaflets does the aortic valve normally have?

What are these called?

Answer 2

3 - called the aortic sinuses of Valsalva

Question 3

Purpose of the leaflets in the aortic valve?

Answer 3

These allow blood to flow from the left ventricle into the aorta, but prevent blood from flowing back into the left ventricle.

Question 4

How many aortic leaflets may patients with aortic stenosis have?

Answer 4

Patients with aortic stenosis may have one, two, three or four leaflets.

Question 5

How can mild aortic stenosis present?

Answer 5

Mild aortic stenosis can be completely asymptomatic, discovered as an incidental murmur during a routine examination.

Question 6

How can more significant aortic stenosis present?

Answer 6

1) fatigue
2) SOB
3) dizziness
4) syncope

These symptoms are typically worse on exertion as the outflow from the left ventricle cannot keep up with demand.

Question 7

When will severe congenital aortic valve stenosis present?

Answer 7

Severe aortic stenosis will present with heart failure within months of birth.

Question 8

What is the key examination finding in aortic stenosis?

Answer 8

An ejection systolic murmur heard loudest at the aortic area (2nd intercostal space, right sternal border).

Question 9

Describe the murmur heard in aortic stenosis

Answer 9

• Ejection systolic
• Heard loudest 2nd intercostal space, right sternal border (aortic area)
• It has a crescendo-decrescendo character
• Radiates to carotids

Question 10

Where does an aortic stenosis murmur radiate to?

Answer 10

Carotids

Question 11

Cardiac examination findings in aortic stenosis?

Answer 11

1) Ejection click just before the murmur
2) Palpable thrill during systole
3) Slow rising pulse and narrow pulse pressure

Question 12

What is the gold standard for diagnosing aortic stenosis?

Answer 12

Echo

Question 13

Progression of congenital aortic stenosis?

Answer 13

Congenital aortic stenosis tends to be a progressive condition that worsens over time.

Patients need regular follow-up under a paediatric cardiologist, with echocardiograms, ECGs and exercise testing to monitor the progression of the condition.

Patient with more significant stenosis may need to restrict physical activities.

Question 14

3 options for treating congenital aortic stenosis?

Answer 14

1) Percutaneous balloon aortic valvoplasty
2) Surgical aortic valvotomy
3) Valve replacement

Question 15

Complications of congenital aortic stenosis?

Answer 15

1) Left ventricular outflow tract obstruction

2) Heart failure

3) Ventricular arrhythmia

4) Bacterial endocarditis

5) Sudden death, often on exertion

Question 16

How many leaflets does the pulmonary valve usually consist of?

Answer 16

3

Question 17

What is congenital pulmonary valve stenosis?

Answer 17

When the leaflets in the pulmonary valve develop abnormally, becoming thickened or fused.

This results in a narrow opening between the right ventricle and the pulmonary artery.

Question 18

Congenital pulmonary valve stenosis often occurs without any associations.

What 4 conditions can it be associated with?

Answer 18

1) Tetralogy of Fallot
2) William syndrome
3) Noonan syndrome
4) Congenital rubella syndrome

Question 19

Presentation of pulmonary stenosis?

Answer 19

• Often completely asymptomatic, discovered as an incidental finding of a murmur during routine baby checks.
• Fatigue on exertion, shortness of breath, dizziness and fainting.

Question 20

What murmur is heard in pulmonary stenosis?

Answer 20

Ejection systolic murmur heard loudest at the pulmonary area (second intercostal space, left sternal border).

Question 21

Cardiac exam findings in pulmonary stenosis?

Answer 21

1) Ejection systolic murmur heard loudest at the pulmonary area (second intercostal space, left sternal border)

2) Palpable thrill in the pulmonary area

3) Right ventricular heave due to right ventricular hypertrophy

4) Raised JVP with giant a waves

Question 22

What is the gold standard investigation for establishing a diagnosis of pulmonary stenosis?

Answer 22

Echo

Question 23

Management of mild pulmonary stenosis without symptoms?

Answer 23

Patients generally do not require any intervention and are followed up by a cardiologist with a “watching and waiting” approach.

Question 24

What is the treatment of choice of symptomatic pulmonary stenosis?

Answer 24

Balloon valvuloplasty via a venous catheter.

This involves inserting a catheter under xray guidance into the femoral vein, through the inferior vena cava and right side of the heart to the pulmonary valve, and dilating the valve by inflating a balloon. If valvuloplasty is not appropriate or fails open-heart surgery can be performed.

Question 25

What is Ebstein’s anomaly?

Answer 25

Ebstein’s anomaly is a congenital heart defect where the tricuspid valve is set lower in the right side of the heart (towards the apex), causing a bigger right atrium and a smaller right ventricle.

This leads to poor flow from the right atrium to the right ventricle, and therefore poor flow to the pulmonary vessels.

Question 26

What defect is Ebstein’s anomaly often associated with?

Answer 26

It is often associated with a right to left shunt across the atria via an atrial septal defect.

When this happens blood bypasses the lungs, leading to cyanosis.

Question 27

What 2 conditions is Ebstein’s anomaly associated with?

Answer 27

1) ASD or patent foramen ovale (PFO) (80% patients) –> resulting in a shunt between the right and left atria

2) Wolff-Parkinson-White syndrome

Question 28

Exposure to what medication in utero can cause Ebstein’s anomaly?

Answer 28

Lithium

Question 29

Clinical features of Ebstein’s anomaly?

Answer 29

1) Evidence of heart failure (e.g. oedema)

2) Gallop rhythm heard on auscultation characterised by the addition of the third and fourth heart sounds

3) Cyanosis

4) Shortness of breath and tachypnoea

5) Poor feeding

6) Collapse or cardiac arrest

7) Hepatomegaly

8) Tricuspid regurgitation –> pansystolic murmur, worse on inspiration

Question 30

Investigation for diagnosing Ebstein’s anomaly?

Answer 30

Echocardiogram

Question 31

Management of Ebstein’s anomaly?

Answer 31

1) Medical management includes treating arrhythmias and heart failure.

2) Prophylactic antibiotics may be used to prevent infective endocarditis.

3) Definitive –> surgery