Congenital cardiac abnormalities

Question 1

What are the main types of VSD?

Answer 1

Subaortic
Perimembranous
Muscular

Question 2

What is VSD associated with?

Answer 2

Down’s

Turner’s

Question 3

What is the presentation of VSD?

Answer 3

Pansystolic murmur- harsh, loudest at lower left sternal edge
Poor feeding
Dyspnoea, tachypnoea
Failure to thrive
LR shunt- right heart failure, pulmonary hypertension
Severe- heart failure

Question 4

What is the management of VSD?

Answer 4

Closure- trans catheter or surgical

Question 5

What is a complication of VSD?

Answer 5

Eisenmenger syndrome

Question 6

What is Eisenmenger’s syndrome?

Answer 6

Pressure in R heart greater than L heart, so shunt reverses R to L –> cyanosis

Question 7

What is the presentation of ASD?

Answer 7

Generally well in early childhood
Failure to thrive, poor feeding, dyspnoea
Wide fixed splitting of 2nd heart sound
Pulmonary flow murmur- cresendo decrescendo murmur
L–> R shunt- right heart failure, pulmonary hypertension

Question 8

What is the management of ASD?

Answer 8

Good chance of spontaneous closure

Closure- trans catheter, surgical

Question 9

What are the complications of ASD?

Answer 9

AF
Heart failure
Pulmonary hypertension
Eisenmenger’s syndrome

Question 10

What is AVSD?

Answer 10

Single AV valve with ostrium primum ASD and high VSD

Question 11

What is AVSD associated with?

Answer 11

Down’s

Question 12

What is patent ductus arteriosus?

Answer 12

Connection between pulmonary trunk and descending aorta- usually closes with first few days due to prostaglandins

Question 13

Who is PDA common in?

Answer 13

Pre term babies

Question 14

What is the presentation of PDA?

Answer 14

Left subclavicular thrill
Continuous murmur
Large volume bounding collapsing pulse

Question 15

What is the management of PDA?

Answer 15

Indomethacin- works in majority of cases

Closure with umbrella device

Question 16

What is coarctation of the aorta?

Answer 16

Congenital narrowing of descending aorta

Question 17

What is coarctation of the aorta associated with?

Answer 17

Turner’s

Males > females

Question 18

What is the presentation of coarctation of the aorta?

Answer 18

Heart failure
Radio femoral delay
Weak femoral pulses
Hypertension in arms, hypotension in legs
Midsystolic murmur, maximal over back

Question 19

What is th management of coarctation of aorta?

Answer 19

Mild= watch and wait
Severe= prostaglandin A to keep PDA open until surgery

Question 20

What are the pathologies in tetralogy of Fallot?

Answer 20

VSD
Overriding aorta
Pulmonary stenosis
RV hypertrophy

Question 21

What are the risk factors for Tetralogy of Fallot?

Answer 21

Maternal rubella

Question 22

What s the presentation of Tetralogy of FAllot?

Answer 22

Ejection systolic murmur
Heart failure before 1 yo
Cyanosis
Tet spells- R to L shunt becomes worse upon exertion

Question 23

What investigations are done for tetralogy of FAllot?

Answer 23

Echo and Doppler

CXR- boot shaped heart

Question 24

What is th management of tetralogy of FAllot?

Answer 24

Total surgical repair by open heart surgery

Question 25

What is transposition of the great arteries?

Answer 25

Aorta connected to RV and pulmonary trunk to LV

Question 26

What is essential for survival with TGA?

Answer 26

Shunt between circulations- ASD, VSD, PDA

Question 27

What is the presentation pf TGA?

Answer 27

Failure to thrive, cyanosis, tachycardia

Question 28

What is the diagnosis and management of TGA?

Answer 28

Often diagnosed during pregnancy | Immediate arterial switch procedure

Question 29

What is aortic stenosis associated with?

Answer 29

William's

Question 30

What is the presentation of aortic stenosis?

Answer 30

Usually asymptomatic Ejection systole murmur- upper R sternal border, radiation to carotids Slow rising pulse, narrow pulse pressure

Question 31

What is the presentation of severe aortic atenosis?

Answer 31

Reduced exercise tolerance Exertional chest pain Syncope

Question 32

What investigations are done for aortic stenosis?

Answer 32

Echo ECG Exercise tolerance test

Question 33

What is the management of aortic stenosis?

Answer 33

Percutaneous balloon valvuloplasty Surgical valvotomy Valve replacement

Question 34

What is associated with pulmonary stenosis?

Answer 34

Noonan | Tetralogy of Fallot

Question 35

What is the presentation of pulmonary stenosis?

Answer 35

Mild= asymptomatic Ejection systolic murmur- upper left sternal border. radiation to back Severe- severe external dyspnoea, fatigue, raised JVP, RV heave and hypertrophy

Question 36

How is pulmonary stenosis investigated?

Answer 36

Echo

Question 37

What is the management of pulmonary stenosis?

Answer 37

Mild= watch and wait Balloon valvuloplasty Failure= open heart surgery

Question 38

What is Ebstein's anomaly?

Answer 38

Low insertion of tricuspid valve resulting in large R atrium and small ventricle

Question 39

What is Ebstein's anomaly associated with?

Answer 39

Lithium in utero | Wolff Parkinson White

Question 40

What is the management of Ebstein's anomaly?

Answer 40

Surgery