Congenital cardiac disease

Question 1

Name 4 features of an innocent cardiac murmur

Answer 1

Early/ejection systolic
Never limited to diastole
Varies with position (louder when supine) and respiration (increases with inspiration)
Normal heart sounds (especially normal splitting of S2)
No click or thrill
Heard loudest of left sternal edge with minimal radiation

Question 2

What are the 4 main types of innocent murmurs? Give a feature of each type

Answer 2

Still’s murmur - low-medium pitched, early systolic, crescendo-decrescendo over L lower sternal edge
Pulmonary flow murmur - medium-high pitch, ejection/mid-systolic, L upper sternal edge
Branch pulmonary stenosis - radiates to axilla or back
Venous hum - low pitch, continuous, hear over low anterior neck

Question 3

What two pathological murmurs can a pulmonary flow murmur mimic? What distinguishes between them?

Answer 3

Atrial septal defect - ASD has fixed splitting of S2

Pulmonary valve stenosis - can have an ejection click or thrill

Question 4

What causes normal splitting of S2? Is the splitting more pronounced in inspiration or expiration and why?

Answer 4

Splitting of S2 from aortic valve closing before pulmonary valve. Heard louder in inspiration - reduced intrathoracic pressure = greater venous return = greater pressure in RV = takes longer for pulmonary valve to close

Question 5

Name 3 indications to refer a child with a murmur to a cardiologist

Answer 5

If under 1 year of age
If murmur is loud, diastolic or continuous
Fixed splitting of S2
Absent respiratory variation

Question 6

What 3 conditions lead to a left-to-right shunt? Name 3 symptoms that this causes. How long after birth do these symptoms usually manifest? What is a serious long term outcome of an uncorrected left-to-right shunt?

Answer 6

ASD, VSD and PDA. Left-to-right shunt = more blood in pulmonary vasculature = reduced pulmonary compliance = increased WOB, tachypnoea, failure to thrive. Also increases catecholamine release = sweating, irritable, tachycardic.

Usually starts a few weeks after birth (have to wait for pulmonary vasculature to reduce resistance)

Without correction, can lead to Eisenmenger’s syndrome - irreversible pulmonary HT, which has a terrible prognosis

Question 7

Are left-to-right shunt conditions the same as congestive cardiac failure? Why/why not?

Answer 7

Different - the reason for the symptoms are because of increased pulmonary blood volume, not from LV failure

Question 8

Go through the characteristics of a VSD murmur

Answer 8

Pansystolic, loudest over left lower sternal edge

Question 9

What are the effects of a large VSD on heart shape and size?

Answer 9

Large VSD = blood shunted from LV to the top of the RV and out the pulmonary artery. Causes volume loading of left heart and LV dilatation, but minimal effect to RV as the blood doesn’t pool in the RV

Question 10

Name 2 medical interventions for a VSD

Answer 10

Diuretics (frusemide, spironolactone) - reduce blood volume
Afterload reduction (ACE I) - reduced blood volume = reduced systemic vascular resistance = more blood will go through aorta than pulmonary artery

Question 11

What is the definitive treatment of a VSD?

Answer 11

Surgery

Question 12

Go through the characteristics of an ASD murmur

Answer 12

Ejection systolic murmur over pulmonary area with fixed splitting of S2

Question 13

Why do you get fixed splitting of S2 with an ASD?

Answer 13

In ASD, expiration promotes pulmonary venous return = more blood shunts from L to R = greater RV pressure = the delay between the pulmonary and aortic valve closure remains.

Question 14

What is the difference between the effects of an ASD and VSD on ventricular size?

Answer 14

In an ASD all shunted blood goes into the RV = RV dilatation, where as the RV is usually normal in a VSD

Question 15

When do symptoms of an ASD usually begin and why?

Answer 15

Usually in third-fourth decade of life; at this age, the systemic blood pressure has increased (HT) so that it forces more blood through pulmonary circulation. Then start to see symptoms of pulmonary HT, heart failure and arrhythmias

Question 16

Do you always have to have surgery to fix an ASD? Why/why not?

Answer 16

Not always - 80% of small lesions will fix themselves before they’re 2

Question 17

What is the main characteristic of a patent ductus arteriosus murmur?

Answer 17

Continuous murmur through systole and diastole

Question 18

After what time do PDAs rarely close in a fullterm?

Answer 18

Beyond 2 weeks

Question 19

What are the 3 main obstructive congenital heart lesions?

Answer 19

Pulmonary stenosis
Coarctation of aorta
Aortic stenosis

Question 20

What is coarctation of the aorta? What sort of murmur does it give rise to?

Answer 20

Narrowing of the aorta. Murmur - systolic, heard loudest over upper sternum.

Question 21

What sign on CXR can undiagnosed coarctation of the aorta give rise to in an adult?

Answer 21

Rib notching (from patent anastomoses)

Question 22

What is the murmur of pulmonary stenosis?

Answer 22

Ejection systolic with ejection click, loudest over LUSE and delay in P2

Question 23

What is the murmur of aortic stenosis?

Answer 23

Ejection systolic with ejection click, loudest over RUSE and delay in A2

Question 24

Name 2 congenital cyanotic lesions

Answer 24

Tetralogy of Fallot - pulmonary stenosis, overriding aorta, VSD and RV hypertrophy
Transposition of Great Arteries (TGA)