Congenital CV Conditions

Question 1

Name 9 congenital CV conditiosn

Answer 1

Tetralogy of Fallot
Ventricular Septal Defect (VSD)
Atrial Septal Defect (ASD)
Coarctation of the Aorta
Double Aortic Arch
Transposition of the Great Vessels
Aberrant Subclavian Artery
Patent Ductus Arteriosus
Bicuspid Aortic Valve

Question 2

What are the 4 characteristics of the tetralogy of fallot

Answer 2

• VSD
• Aorta overiding the VSD
• Pulmonary Stenosis
• Right Ventricular Hypertrophy

Question 3

What causes the tetralogy of fallot?

Answer 3

An abnormality in the seperation of the truncus arteriosus into the aorta & pulmonary trunk

Question 4

What are the symptoms of the tetralogy of Fallot?

Answer 4

Pulmonary Stenosis Murmur
Cyanosis
Clubbing
RV heave
Small children may show agitation, crying, feeding difficulty, failure to thrive and squatting (they do this because it increases TPR)

Question 5

How would you investigate tetralogy of fallot/

Answer 5

CXR - Boot shaped heart
Echo - Degree of Stenosis
CT/MRI - Helps you plan surgery
FBC - Polycythaemia

Question 6

What treatments can be given for tetralogy of fallot?

Answer 6

O2 and morphine acutely
Surgery to close the VSD and correct the pulmonary stenosis
B-blockers to reduce the strain on the right ventircle in particular

Question 7

How does a VSD arise non-congenitally?

Answer 7

The ventricular septum can rupture in an MI

Question 8

What are symptoms of VSD?

Answer 8

Heave
Systolic Thrill
Pansystolic Murmur
cysanosis & dyspnoea with occasionally severe heart failure

Question 9

How do we investigate/treat a VSD?

Answer 9

An ECG, CXR & Echo
Medical treatment until it closes spontaneously and failing that surgery

Question 10

In what section of the atrial septum is are ASDs most often found?

Answer 10

The upper part or ostium secundum

Question 11

What are the sympoms/signs of an ASD?

Answer 11

Cyanosis - arrhythmia - Haemoptysis - Chest pain - Dyspnoea
Pulmonary Hypertension - High JVP - Pulmonary Ejection Murmur - Atrial Fibrillation

Question 12

How do we investigate/treat and ASD?

Answer 12

ECG - CXR - Echo
Treat with a transcatheter closure or surgical closure

Question 13

What is coarctation of the aorta>

Answer 13

A narrowed Aorta, mainlt on the arch close to the ligamentum arteriosus

Question 14

What are the signs/symptoms of a coarctated aorta?

Answer 14

Heart Failure - Failure to Thrive
Radial-Radial and Radial-Femoral delay
Weak Femoral Pulse + Cold Legs
Systolic Murmur
Hypotension in lower limbs + ~hypertension above coarctation

Question 15

What is a double aortic arch?

Answer 15

A 2nd arch grows from the ascending aorta and passes behind the trachea & oesophagus before rejoing the left
This can cause difficulty swallowing and breathing

Question 16

What is transposition of the great vessels:

Answer 16

Due to the neural crest cells migrating defectively and the aorticopulmonary septum failing to spiral.
The Aorta feeds off the RV & the Pulmonary trunk off the LV

Question 17

What are the symptoms of great vessel transposition?

Answer 17

Cyanosis.
Regularly comes with ASD, VSD & PDA which are needed to keep blood passing between the circulations and the patient alive

Question 18

What is an aberrant subclavian artery:

Answer 18

The right 4th aortic arch and dorsal aorta regress abnormally.
So the right subclavian emerges from the aortic arch on your left side & crosses behind the oesophagus to supply the right arm.

Question 19

What is patent ductus arteriosus? (PDA)

Answer 19

The ductus arteriosum (connecting aorta & pulmonary trunk) fails to close & become the ligamentum arteriosum after birth

Question 20

What are the symptoms of PDA?

Answer 20

Icnreased work of breathing
Poor weight gain
Congestive heart failure as you age

Continuous murmur and large pulse pressure

Question 21

What can a bicuspid aortic valve develop and how can we fix it?

Answer 21

Aortic Stenosis
Aortic Regurgitation
Aortic Dissection

Aortic Valve Replacement Surgery

Question 22

How do we investigate and fix a coarctated Aorta?

Answer 22

By CXR (rib notching) and CTA or MRA.

We can fix it with bypass surgery or angioplasty + Stenting.

Question 23

What is persistant truncus arteriosus?

Answer 23

A failure of the aorticopulmonary septum to form leading to a combined pulm trunk and aorta along with a VSD at their base

Question 24

How does Persistant Truncus Arteriosus present?

Answer 24

Cyanosis - HF within weeks - Wide Pulse Pressure - Loud Second Heart Sound - Systolic Ejection Murmur - Pulm Hypertension

Question 25

How do we investigate and treat Persistant truncus arteriosus?

Answer 25

An ECHO and CXR
Surgery:
- Repair VSD
- Add Pulm Artery
- The Truncus Arteriosus becomes the Aorta

Question 26

How do we investigate Transposition of the great vessels?

Answer 26

CXR - Egg on a string heart
Echo

Question 27

How do we treat transposition of the great vessels?

Answer 27

Surgery
In the mean time prostoglandins to keep the ductus arteriosus from closing

Question 28

How do we investiate and treat PDA?

Answer 28

CXR/ECG/ECHO - all show LVH
Surgery if after birth
Prostoglandin synthesis inhibitors if you catch it neonatally or in premature babies