Congenital heart disease

Question 1

What is congenital heart disease?

Answer 1

An abnormality of the structure of the heart (doesn’t cover cardiomyopathies, anything to do with the funciton of cells or the electrical wiring of the heart)

Question 2

What is a mild congenital heart disease?

Answer 2

Asymptomatic, may resolve spontaneously (may progress to moderate or severe in adult hood) - small VSD, PFO, ASD, PDA

Question 3

What is a moderate congenital heart disease?

Answer 3

Requires specialist intervention and monitoring in a cardiac centre. Mild or moderate AS, PS, larger ASD, VSD

Question 4

What is a congenital heart disease?

Answer 4

Present severely ill /die in newborn period or early infancy - all cyanotic lesions, all duct dependent lesions, truncus

Question 5

How does congenital heart disease present?

Answer 5

Screening - antenatal, newborn baby check
Well baby with clinical signs
Unwell baby - cyanosis, shock, cardiac failure

Question 6

How does cardiac failure present in a newborn?

Answer 6

Reduced feeding, failure to thrive, breathlessness, sweatiness

Question 7

How is antenatally diagnosed CHD managed?

Answer 7

Depends on the disease, expert team avaliable and plans in place
May decide to deliver in cardiac surgical centre, PGE2 infusion if duct dependent lesion

Question 8

What is done in a newborn check?

Answer 8

Clinical exam around 24 hours of age
Femoral pulses, heart sounds and presence of murmurs
Will detect any condition causing a murmur, obvious cyanosis or abnormal pulses

Question 9

Why can a baby be cyanosed?

Answer 9

Any condition that causes deoxygenated blood to bypass the lungs and enter the systemic circulation
Any condition where mixed oxygenated and deoxygenated blood enters the systemic circulation from the heart

Question 10

What is the differential diagnosis of cyanosis in a newborn?

Answer 10

Cardiac disease
Respiratory disease
PPHN

Question 11

How is cardiac cyanosis differentiated from respiratory?

Answer 11

Tend to be blue with little or no resp distress, may have pre-post ductal differences

Question 12

How is respiratory cyanosis differentiated from cardiac?

Answer 12

Usually associated with increased work of breathing, xray changed

Question 13

What is the most common cyanotic condition to present in new-borns?

Answer 13

Transposition of the great arteries (RV connected to the aorta and LV connected to the PA) There must be mixing of blood via the foramen ovale or the baby would be dead.

Question 14

What will transposition of the great arteries present as?

Answer 14

Cyanosis

There will also be a build up of lactic acid so the babies will be acidotic

Question 15

What will collapse at the time of duct closure present as?

Answer 15

Severe cyanosis, tachypnoea, distress, rapid deteriotation to death
Prolonged CTR, poor or absent pulses, hepatomegaly, crepitations
Acidotic

Question 16

What is the differential diagnosis if the newborn collapses at time of duct closure?

Answer 16

Sepsis
Metabolic conditions
Cardiac conditions

Question 17

What is the treatment if the baby depends on the duct and it closes?

Answer 17

ABC
PGE2 to attempt to reopen the duct
Multisystem supportive treatment
Transfer to cardiac surgical centre for definitive management

Question 18

What are cardiac examples of duct dependent conditions?

Answer 18

Hypoplastic left heart, critical aortic stenosis, interrupted aortic arch, critical coarctation of aorta
Tricuspid atresia
Pulmonary atresia

Question 19

What is hypoplastic left heart syndrome?

Answer 19

A very small LV with a very small aortic valve and a very small ascending aorta. There is a shunt at atrial level to allow blood to flow from the left side to the right side of the circulation. There will therefore be mixed blood in the RA and ventricle and oxygenated blood will flow via the duct

Question 20

What are the clinical signs of hypoplastic left heart syndrome?

Answer 20

No palpable pulses
Hepatomegaly
Increasing acidosis

Question 21

What is pulmonary atresia?

Answer 21

Thinning of the pulmonary arteries, there is thereofre retrograde filling of the pulmonary arteries via the ductus arteriosus. Usually associated with a VSD

Question 22

How will a large VSD present?

Answer 22

No murmur at baby check, but the murmur will develop as pulmonary pressures drop over the first few weeks
Increased pulmonary circulation, congestive heart failure

Question 23

What are the long term managements of CHD?

Answer 23

Surgical management - repair vs palliation
Developmental problems - hypoxia, bypass time
Need for further surgery - valves, stenosis, transplant
Emotional/social issues of family