Paediatric Cardiology

Question 1

Give 4 CYANOTIC congenital heart defects

Answer 1

• Tetralogy of Fallot
• Transposition of the Great Arteries
• Tricuspid Atresia
• Truncus Arteriosus

Question 2

Give 4 ACYANOTIC congenital heart defects

Answer 2

• Atrial Septal Defect
• Ventricular Septal Defect
• Patent Ductus Arteriosus
• Coarctation of the Aorta

Question 3

When does TOF normally present?

Answer 3

Usually around 1-2 months. It is the most common CHD except at birth which is TGA.

Question 4

What are the 4 cardiac defects involved in ToF?

Answer 4

• Pulmonary Stenosis (leads to) (functional due to overriding aorta)
• Right ventricular hypertrophy
• Ventricular Septal Defect
• Overriding aorta (onto VSD)

Question 5

What is the direction of the shunt in ToF and why?

Answer 5

• Right to left
• Pressure in RV > LV
• Due to RV hypertrophy
• Results in mixing of oxygenated and deoxygenated blood = cyanosis

Question 6

What are the cardiac presentations of ToF? (5)

Answer 6

• Ejection systolic murmur @ upper left sternal border
• Cyanosis +/- tet spells
• Breathing difficulty
• Clubbing at 1-2 months
• Polycythaemia? Chronic hypoxia

Question 7

What is a tet spell?

Answer 7

• Period of cyanosis upon exertion or anxiety etc
• sudden marked increase in cyanosis followed by syncope
• child may instinctively squat to ↑peripheral vascular resistance (↑pressure in femoral arteries) = ↑ pressure in LV to reverse the shunt

Question 8

Give 2 other, non-cardiac features of ToF

Answer 8

• Difficulty feeding

- Not growing properly/failure to thrive

Question 9

What are the investigations for ToF?

Answer 9

• Echocardiogram + Doppler
• CXR - has a boot shaped heart
• Screen for DiGeorge Syndrome as conditions are often associated

Question 10

What are 4 risk factors for ToF?

Answer 10

Non-modifable
- Maternal age >40

Modifiable

• Maternal alcohol use during pregnancy
• Maternal Diabetes
• Maternal Rubella infection during pregnancy

Question 11

What is transposition of the great arteries?

Answer 11

• Pulmonary Artery and Aorta switch
• Aorta now onto RIGHT VENTRICLE via pulmonary valve
• Pulmonary artery now onto LEFT VENTRICLE via aortic valve

Question 12

How is TGA compatible with life?

Answer 12

Another heart defect, usually a VSD (or ASD or PDA) is present so that oxygenated and deoxygenated blood can mix

Question 13

How does TGA usually present?

Answer 13

• At birth

- Initially ok but then presents as cyanosis that may rapidly deteriorate as the PDA closes.

Question 14

Give a risk factor for TGA

Answer 14

Maternal Diabetes

Question 15

What is the immediate and long term management of TGA?

Answer 15

• Immediate = give prostaglandins as will keep the ductus arteriosus open
• Cardiac Repair Surgery
• Arterial Switch
  May lead to:
  Arrhythmias
  Right ventricular hypertrophy
  Tricupsid Regurgitation

Question 16

What is tricuspid atresia?

Answer 16

Tricuspid valve is underdeveloped so doesn’t open/ is just some tissue.

Right Ventricle becomes hypoplastic too

RV is dysfunctional = ↓ blood to lungs

Question 17

How is tricuspid atresia compatible with life?

Answer 17

• An atrial septal defect AND a ventricular septal defect are both needed for compatibility with life:
• ASD so that mixed blood can get into the LA to LV and onto the body
• VSD so that blood can get from LV to RV and into the lungs

Question 18

What are the cardiac presentations of tricuspid atresia?

Answer 18

Progressive cyanosis

Pansystolic murmur in mitral area (valve closed when LV contracts = turbulent flow)

Left Ventricular Hypertrophy (palpable apex beat?)

Tachypnoea for first 2 weeks of life

Question 19

What is the murmur associated with tricuspid atresia?

Answer 19

Pansystolic murmur in mitral area (valve closed when LV contracts = turbulent flow)

Question 20

What is the treatment for tricuspid atresia?

Answer 20

Treatment
Prostaglandins to keep PDA open
Cardiac repair surgery

Question 21

What is a ventricular septal defect?

Answer 21

There is a hole in the ventricular septum (usually the membranous part) allowing blood to flow from LEFT to RIGHT.

Forms a LEFT to RIGHT shunt as pressure is higher on the LEFT

Question 22

What could be a complication of a VSD?

Answer 22

↑ blood flow to lungs may lead to pulmonary hypertension and therefore pulmonary oedema etc. AND may reverse the shunt (Eisenmengers Syndrome)

Question 23

Which conditions are associated with a VSD?

Answer 23

• Foetal alcohol syndrome

- Down’s Syndrome

Question 24

What are the symptoms of a VSD?

Answer 24

• Often asymptomatic but depends on severity
• Often ↓growth/ not growing as well as they should
• If more severe then symptoms of heart failure

Question 25

What is the murmur associated with a VSD?

Answer 25

- Pansystolic murmur at lower left sternal edge | - Thrill here

Question 26

What are the investigations for CHD?

Answer 26

Echocardiogram + doppler is diagnostic

Question 27

What is the treatment for a VSD?

Answer 27

- Cardiac Repair Surgery between 3-6 months - ↑ calorie intake (growth bit) - Diuretics if HF

Question 28

What is an atrial septal defect? | What are the two types?

Answer 28

- There is a gap in the septum between the atria - 2 kinds: - Secundum (more common) - involved Foramen Ovale - Atrioventricular Septal Defect - communication between atria at bottom of septum + abnormal valves - Blood is shunted from LEFT to RIGHT

Question 29

Is an ASD always pathological?

Answer 29

No Opening within the septum is physiological in utero so that lungs can be bypassed Oxygenated blood is received from the placenta via the umbilical vein →RA →LA →LV →Body Foramen Ovale should close at birth

Question 30

Which conditions are associated with ASDs?

Answer 30

- Foetal Alcohol Syndrome | - Down's Syndrome

Question 31

What is a potential complication of an ASD?

Answer 31

- Paradoxical embolism - Embolism returns to R heart and moves into L - Can potentially travel to the brain and cause a stroke etc

Question 32

What is the murmur associated with an ASD?

Answer 32

- Ejection Systolic murmur at upper left sternal border - Partial AVSD may also have a pansystolic murmur at the apex - S2 heart sound splits

Question 33

What are the symptoms of an ASD?

Answer 33

Most often is asymptomatic but can have recurrent chest infections

Question 34

What are the treatment options for an ASD?

Answer 34

Occlusion Device | Cardiac Repair Surgery

Question 35

What is the function of the ductus arteriosus?

Answer 35

Connects the pulmonary artery to the descending aorta in utero Normally closes after birth if born at term Blood can flow from aorta into pulmonary artery and to lungs so ↑ pulmonary blood flow

Question 36

When is the ductus arteriosus counted as PATENT?

Answer 36

If it hasn't closed one week after the estimated date of delivery

Question 37

What are the symptoms of a PDA?

Answer 37

often asymptomatic but may have symptoms of RIGHT heart failure

Question 38

What is the murmur associated with a PDA?

Answer 38

Continuous machinery murmur under the LEFT clavicle Bounding pulse

Question 39

What is the role of echo + doppler in PDA?

Answer 39

- Diagnosis | - Rules out duct dependent circulation before closure of the defect

Question 40

How can a PDA be closed?

Answer 40

- Ibuprofen or endovascular surgery before 1 year old

Question 41

Why is a PDA therapeutically closed?

Answer 41

- Close the duct to ↓risk of bacterial endocarditis/pulmonary vascular disease in later life

Question 42

What is Ebstein's Anomaly?

Answer 42

CHD due to the use of Lithium during pregnancy

Question 43

What is the pathophysiology of Ebstein's Anomaly?

Answer 43

Posterior leaflets of the tricuspid valve become anteriorly displaces towards the apex causes tricuspid regurgitation AND tricuspid stenosis AND right atrium enlargement

Question 44

What are the murmurs associated with Ebstein's anomaly?

Answer 44

- Pansystolic @ upper left sternal edge (tricuspid regurgitation) - mid diastolic (tricuspid stenosis)