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Year 4 - Paediatrics > Cardiology - Valve Stenosis > Flashcards

Cardiology - Valve Stenosis Flashcards

1
Q

What is coarctation of the aorta?

A

Narrowing of the aortic arch around the ductus arteriosus

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2
Q

What genetic condition is coarctation of the aorta associated with?

A

Turners syndrome

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3
Q

What happens to blood pressure in aortic coarctation?

A

Distal to the narrowing = pressure is decreased

Proximal to narrowing = pressure is increased

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4
Q

How does coarctation of the aorta present?

A
  • Weak femoral pulses
  • Systolic murmur below left clavicle and below left scapula
  • Tachypnoea
  • Poor feeding
  • Grey and floppy baby

After a period of time due to development
- LV heave, due to hypertrophy
- Underdeveloped left arm where there is reduced blood flow to left subclavian
- Underdevelopment of legs

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5
Q

What can be done to reveal coarctation of the aorta?

A

Four limb blood pressure

High blood pressure in limbs before narrowing

Low in limbs after narrowing

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6
Q

How is coarctation of the aorta managed?

A

Mild cases are symptom free until adulthood

Severe cases needed emergency surgery after birth

Critical cases
- Risk of heart failure and death
- Prostaglandin E used to keep ductus arteriosus open while waiting for surgery
- Surgery then performed to correct coarctation and ligate ductus arteriosus

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7
Q

What are the aortic valve leaflets called?

A

Aortic sinuses of Valsalva

Patients with congenital aortic valve stenosis can have 1,2,3 or 4 leaflets instead of the usual 3

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8
Q

How does aortic valve stenosis present?

A

Mild
Asymptomatic
Discovered as incidental murmur during routine examination

Significant
Symptoms of fatigue
SOB
Dizziness and fainting

Symptoms are worse on exertion as outflow from LV cannot keep up with demand

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9
Q

How does severe aortic stenosis present months after birth?

A

Heart failure

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10
Q

What are the signs of aortic stenosis?

A
  • Ejection systolic murmur heard best at the aortic area, crescendo-decrescendo character that radiates to carotids
  • Ejection click just before murmur
  • Palpable thrill during systole
  • Slow rising pulse
  • Narrow pulse pressure
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11
Q

What investigations are used for aortic stenosis?

A

Gold standard
Echocardiogram

ECGs
Exercise testing
(to monitor progression of the condition)

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11
Q

How is aortic stenosis treated?

A

Percutaneous balloon aortic valvopalsty
Surgical aortic valvotomy
Valve replacement

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12
Q

What are the complications of aortic stenosis?

A

Left ventricular outflow tract obstruction
Heart failure
Ventricular arrhythmia
Bacterial endocarditis
Sudden death, on exertion

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13
Q

What happens in congenital pulmonary valve stenosis?

A

Valve leaflets develop abnormally, becoming thickened or fused

Causes narrow pulmonary valve

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14
Q

What is congenital pulmonary valve stenosis associated with?

A

Tetralogy of Fallot
William syndrome
Noonan syndrome
Congenital rubella syndrome

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15
Q

How does pulmonary stenosis present?

A

Often completely asymptomatic, discovered incidentally as a murmur on routine baby checks

More signicant
- Fatigue on exertion
- SOB
- Dizziness and fainting

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16
Q

What are the signs of pulmonary stenosis?

A

Ejection systolic murmur heard best at the pulmonary area
Palpable thrill in the pulmonary area
RV heave due to RVht
Raised JVP with giant a waves

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17
Q

What investigations are used for pulmonary valve stenosis?

A

Gold standard
Echocardiogram

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18
Q

How are patients with pulmonary valve stenosis managed?

A

Mild- Watchful waiting

Symptomatic or more significantly stenosed
- Balloon valvuloplasty via venous catheter
- Open-heart surgery (second line)

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19
Q

What is Tetralogy of Fallot?

A

POSH

Pulmonary stenosis
Overriding aorta
Septal defect (ventricular)
Hypertrophy of RV

20
Q

What is an overriding aorta?

A

Aortic valve is further to the right than normal

Above the VSD

So when the RV contracts and blood sent upwards, aorta is in the direction of travel of the blood

More deoxygenated blood enters the aorta from the right side of the heart

21
Q

What causes right to left shunting in TOF?

A

Stenotic pulmonary valve increases resistance

This increases the pressure in the RV, encoruage blood to flow from right to left

Increased strain leads to RVht

Pressure in the RV > LV, blood bypasses lungs causes cyanosis

22
Q

What is the key factor in right to left shunting in TOF?

A

Severity of pulmonary stenosis

23
Q

What are the risk factors of TOF?

A

Rubella
Increased age of mother (over 40)
Alcohol during pregnancy
Diabetic mother

24
Q

What investigations are used for TOF?

A

Echocardiogram, investigation of choice for diagnosis

Doppler flow studies, assessing severity of the abnormality and shunt

CXR
Shows characteristic boot shaped heart due to RVht

25
Q

How does TOF present?

A
  • Most picked up before birth during antenatal scans
  • Ejection systolic murmur caused by pulmonary stenosis heard on new-born baby check
  • If severe will present with heart failure before 1
  • In milder cases can present as older children when they develop heart failure symptoms
26
Q

What are the signs of TOF?

A

Tet spells
Cyanosis
Clubbing
Ejection systolic murmur
Poor feeding
Poor weight gain

27
Q

What are tet spells?

A

Intermittent symptomatic periods where right to left shunt is worsened precipitating a cyanotic episode `

28
Q

What is the physiology of a tet spell?

A

Pulmonary vascular resistance increases or systemic vascular resistance decreases

e.g. if lots of exertion, carbon dioxide acts as a vasodilator, decreasing systemic vascular resistance

Blood therefore will be pumped from RV to aorta bypassing lungs

29
Q

What are the causes of a tet spell?

A

Waking
Physical exertion
Crying

Severe spells
Can cause reduced consciousness
Seizures
Death

30
Q

How are tet spells treated?

A

Older children may squat
Younger children positioned with knees to their chest

Increases the systemic vascular resistance

Medical treatment
- Supplementary oxygen
- Beta blockers, relaxes RV
- IV fluids to increase pre-load to increase blood flow to pulmonary vessels
- Morphine, decreases respiratory drive
- Sodium bicarbonate- metabolic acidosis
- Phenylephrine infusion- increase systemic vascular resistance

31
Q

What can be given in neonates to allow blood flow to pulmonary arteries in TOF?

A

Prostaglandin infusion to maintain ductus arteriosus

Bloods flows back from aorta to pulmonary vessels

32
Q

What is the definitive management of TOF?

A

Total surgical repair by open heart surgery

5% mortality

33
Q

What is the prognosis of TOF?

A

Poor without treatment

With corrective surgery, 90% live to adulthood

34
Q

What is Ebstein’s anomaly?

A

Congenital heart condition where tricuspid valve is set lower in right side of the heart

Causes a bigger RA and smaller RV

35
Q

What does Ebstein’s anomaly lead to?

A

Poor flow from RA to RV, therefore poor flow to pulmonary vessels

Associated with a right to left shunt across ASD

Leads to cyanosis

36
Q

What is Ebstein’s anomaly associated with?

A

Wolf-Parkinson-White syndrome

37
Q

How does Ebstein’s anomaly present?

A

Evidence of heart failure
Gallop rhythm on auscultation
Cyanosis
SOB and tachypnoea
Poor feeding
Collapse or cardiac arrest

38
Q

When do symptoms of Ebstein’s anomaly present?

A

Few days after birth when ductus arteriosus closes

When there is right to left shunt via ductus arteriosus allows blood to get oxygenated

39
Q

How is Ebstein’s anomaly diagnosed?

A

Echocardiogram

And used for assessing severity

40
Q

How is Ebstein’s anomaly managed?

A

Medical management
- Treat arrhythmias
- Treat heart failure
- Prophylactic antibiotics for potential endocarditis

Surgical
- Fix the underlying defect

41
Q

What is transposition of the great arteries?

A

Condition where attachments of aorta and pulmonary trunk are swapped

RV pumps blood into aorta
LV pumps blood into pulmonary vessels

Two separate circulations that don’t mix

42
Q

What is transposition of the great arteries associated with?

A

Ventricular septal defect
Coarctation of the aorta
Pulmonary stenosis

43
Q

What happens during pregnancy with transposition of the great arteries?

A

Gas and nutrient exchange occurs at placenta

After birth there is immediate threat to life

Baby will be cyanosed

44
Q

What does immediate survival depend on in transposition of the great arteries?

A

Shunt between systemic circulation and pulmonary circulation

Allows some oxygenation to occur

PDA
ASD
VSD

45
Q

How is TOGA diagnosed?

A

Diagnosed during pregnancy with antenatal ultrasounds

Should be arranged in a hospital capable of managing the condition

46
Q

How does TOGA present?

A

Cyanosis at or within a few days of birth
Respiratory distress
Tachycardia
Poor feeding
Poor weight gain
Sweating

47
Q

How is TOGA managed?

A

VSD allows some mixing of blood to provide time for definitive management

Prostaglandins
- Used to maintain ductus arteriosus

Balloon sptostomy
- Catheter into foramen ovale via umbilicus
- Inflating balloon to create a large ASD

Open heart surgery- Definitive management
- Cardiopulmonary bypass machine used to perform an arterial switch procedure within a few days of birth, can fix any VSDs or ASDs at same time

Year 4 - Paediatrics (25 decks)

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