CVS Congenital Abnormalities

Question 1

What two types of ASD are there and what three reasons?

Answer 1

1) Septum primum defect
2) Septum secundum defect

Too short
Resorbed
Septum secundum too small

Question 2

Which are the 4 cyanotic congenital HD from lecture?

Answer 2

Tetralogy of fallot
Univentricular heart
Transposition of great arteries
Total anomalous pulmonary venous drainage

Question 3

What are the three distinguishing features of TofF and what can this lead to? How is it treated?

Answer 3

1) Overriding aorta
2) VSD
3) Pulmonary stenosis

Can lead to RVH

Surgery

Question 4

What cells are partly to blame for TofF

Answer 4

Neural crest cells - important for septation (VSD here)

Question 5

What are two theories of the cause of hypoplastic heart (underdeveloped LV)? What happens?

Answer 5

1) Atresia - abnormal development of mitral and aortic valves - small LV hole, limited flow
2) Osteum secundum too small for R–>L flow so L atrophies as not used fully

LV small, Ao small, need PDA as Right heart supports systemic circulation so will need to go to lungs, need ASD for blood returning from lungs to left to then shunt to right to go via PA–>PDA—> body

Question 6

What can happen if the tricuspid valve fails to form?

Answer 6

Tricuspid atresia - means there is no RV inlet so relies on VSD or PDA to flow to lungs and left side

Question 7

What happens if the great arteries fail to rotate during development? What does this kind of abnormality rely on at birth? How to treat?

Answer 7

Transposition of great arteries -
RV connected to Ao
LV connected to PA

Need two of the ducts to communicate:
PDA, ASD, VSD

Surgery

Question 8

Give an example of bidirectional shunting (L->R and R->L)

Answer 8

Transposition of great arteries

Question 9

What is the cause of a univentricular heart?

Answer 9

Failure to septate

Question 10

What happens in pulmonary atresia?

Answer 10

Failure of formation of pulm valve
No RV outlet
Whole of RV has to go via left side through atria
Blood flows to lungs via PDA

Question 11

If someone presented later in life, with arrhythmia and RHF what would your clinical suspicion be? How about LHF?

Answer 11

ASD

LHF in infancy and RHF and Pulm HTN if untreated

Question 12

How can coarctation occur? How can the body compensate? What might occur in adulthood as a result of a coarctation?

Answer 12

PDA normally closes on contraction at birth - ductal tissue on Ao can be contracted in - coarctation

Compensate with collateral vessels

Can present with HTN in adults