Cardiology 2

Question 1

What is a persistent ductus arteriosus? (PDA)

Answer 1

Failure of ductus arteriosus to close by 1 month after the EDD due to a defect in the constrictor mechanism of the duct (in a preterm infant PDA is not caused by congenital heart disease but is due to prematurity)
Blood flows from the aorta to the pulmonary artery (i.e. left to right)

Question 2

How does a PDA present?

Answer 2

Usually asymptomatic, if the duct is large there’ll be HF and pulmonary hypertension
Continuous murmur beneath the left clavicle, continues into diastole
Bounding or collapsing pulse due to increased pulse pressure

Question 3

How is a PDA treated?

Answer 3

NSAIDs or surgical closure with a coil or occlusion device introduced via a cardiac catheter at about 1 year of age

Question 4

What are right to left shunts?

Answer 4

Blood flow from the pulmonary artery to the aorta

Presentation is with cyanosis (blue, sats <94, collapse), usually in the 1st week of life

Question 5

What is tetralogy of fallot?

Answer 5

a large VSD
pulmonary stenosis
right ventricular hypertrophy
an overriding aorta (blood from both ventricles enters the aorta)
Clubbing, murmur at left sternal edge

Question 6

What is the treatment for tetralogy of fallot?

Answer 6

Open heart surgery at 6 months-1 year
Tet spells:
Knee chest position (blocks the femoral to increase peripheral resistance)
Oxygen, IV morphine, IV beta blocker 
Surgery:
Closure of VSD
Relieving the right ventricular outflow tract obstruction
(increasing size of pulmonary valve)

Question 7

What is the management for very cyanosed patients with tetralogy of fallot?

Answer 7

BT shunt- An artificial tube placed between subclavian and pulmonary arteries
Balloon dilatation- of the right ventricular outflow tract

Question 8

What is transposition of great arteries?

Answer 8

The aorta is connected to the right ventricle and the pulmonary artery is connected to the left ventricle (discordant ventriculo-
arterial connection)

Question 9

How does transposition of great arteries present?

Answer 9

Usually on day 2, when the duct closes 
always present cyanosis
loud and single second heart
sound
usually no murmur (but may be if
there is other abnormality present too)

Question 10

What is the treatment for transposition of great arteries?

Answer 10

Maintain patency of ductus arteriosus with a prostaglandin E1 infusion is mandatory
Life-saving balloon atrial septostomy
Arterial switch procedure

Question 11

What are the features of Eisenmenger syndrome?

Answer 11

No murmur
Right heart failure (late)
Medication to delay transplantation

Question 12

What syndromes are associated with congenital heart disease?

Answer 12

Down syndrome- ASDs
Noonan syndrome- pulmonary valve stenosis, ASDs, hypertrophic cardiomyopathy
Marfan’s syndrome- mitral valve prolapse, aortic aneurysm
Turner syndrome- coarctation of aorta

Question 13

What is coarctation of the aorta?

Answer 13

Due to arterial duct tissue encircling the aorta just at the point of the insertion of the duct

Question 14

How does coarctation of the aorta present?

Answer 14

Normal examination on first day of life
Acute circulatory collapse at 2 days of age when the duct closes
Older children may be asymptomatic or present with chest pain/claudication with physical exertion.
Heart failure
Absent femoral pulses
Severe metabolic acidosis

Question 15

What are the investigations for coarctation of the aorta?

Answer 15

Cardiomegaly from heart failure and shock on CXR Older children: murmurs, pulses, blood pressure

Question 16

What us the treatment for coarctation of the aorta?

Answer 16

Resection of the narrowed segment

Question 17

What is hypoplastic left heart syndrome?

Answer 17

Underdevelopment of the entire left side of the heart
Small or atretic mitral valve
Diminutive (extremely small) left ventricle
Usually aortic valve atresia
Very small, to the point of coarctation,
ascending aorta

Question 18

How does hypoplastic left heart syndrome present?

Answer 18

After birth- sickest of all neonates with duct-dependent circulation
Profound acidosis
Rapid CV collapse
weakness or absence of all peripheral pulses (in contrast to weak femorals in
coarctation of aorta)

Question 19

What is the treatment for hypoplastic left heart syndrome?

Answer 19

Norwood procedure

Some may require revision of surgery

Question 20

How does supraventricular tachycardia present?

Answer 20

HR: 250-300
Poor cardiac output and pulmonary oedema
Causes hydrops fetalis and IU death

Question 21

How does myocarditis present?

Answer 21

Most commonly to viral infection, but also bacterial or immune in origin
Chest pain, decreased exercise tolerance, arrythmia

Question 22

What are the normal heart ranges for children?

Answer 22

<1: 110-160
2-5: 95-140
5-12: 80-120
>12: 60-100

Question 23

What is Eisenmenger’s syndrome?

Answer 23

Long standing left to right shunt causes pulmonary hypertension and reversal of shunt to cyanotic right to left
Considered irreversible, heart transplant required

Question 24

What are the symptoms of Eisenmenger’s syndrome?

Answer 24

Cyanosis 
Clubbing
Syncope 
Polycythemia 
Heart failure 
Infections 
Kidney problems 
Gallstones/gout

Question 25

What is the management for heart failure?

Answer 25

Bed rest in semi-upright position
Supplemental oxygen (not in L to R shunt)
Sufficient calorie intake
Diuretics and ACE-1 (captopril)- R to L shunt and high pulmonary flow
BB and digoxin
Prostaglandin infusion- duct dependent circulation

Question 26

What are the signs of a pathological heart murmur

Answer 26

All diastolic & pansystolic murmurs
Late systolic murmurs
Loud murmurs- >3/6
Continuous murmurs
Associated with cardiac abnormalities

Question 27

What are the features of a venous hum?

Answer 27

Innocent hear murmur
Heard above right clavicle and over right jugular vein
Heard throughout cardiac cycle
Placing finger on jugular vein will abolish sound
May disappear if patient is supine or if the patient turns their head to one side