Cardiac Development Flashcards

1
Q

Why is there a right to left shunt in the foetal heart and what causes it?

A
  1. Reduced blood flow to the lungs as they are not in use.

2. Patent foramen ovale and patent ductus arteriosus, high resistance pulmonary arteries and low resistance aorta.

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2
Q

What is the physiological way that the right to left shunt in the heart reduces at birth?

A
  1. Lungs expand with first breath reducing pulmonary vascular resistance.
  2. Increased oxygenation, reduced PGs from placenta and increased lung metabolism of PGs.
  3. Causes ductus arteriosus to close within 3-7 days of life.
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3
Q

What is the pathophysiological cause of acyanotic heart disease in newborns?

A
  1. Defect in the septum causes blood to flow left to right.
    Or
  2. Patent ductus arteriosus persists which causes blood to flow from aorta to pulmonary artery.
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4
Q

What are the clinical features of acyanotic heart disease and what causes them?

A
  1. Increased pulmonary blood flow, pulmonary oedema - SoB, high respiratory rate.
  2. Reduced systemic cardiac output - high heart rate, vasoconstriction, sweaty.
  3. Generally unwell, grow poorly (failure to thrive)
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5
Q

If the heart murmur is louder in a child, how big is the defect?

A

Small (smaller gap for blood to rush through)

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6
Q

What are the signs of heart failure in a baby?

A
  1. Pale and sweaty
  2. Cool peripheries, long CRT, tachycardia
  3. Poor feeding (exhausted), poor weight gain
  4. Tachypnoea
  5. Heart murmur
  6. Subcostal and intercostal recession
  7. Sacral oedema
  8. Hepatomegaly, ascites
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7
Q

What is the most common congenital defect?

A

Ventricular septal defect

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8
Q

Where are ventricular septal defects found and what are they?

A

Most are small, single holes in the membranous portion of the ventricular septum.

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9
Q

What is this a presentation of?

Asymptomatic, normal heart sounds, harsh pansystolic murmur at lower left sternal border. Newborn.

A

Small ventricular septal defect.

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10
Q

What is this a presentation of?

Heart failure, soft pansystolic murmur, palpable thrill. Newborn.

A

Large ventricular septal defect

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11
Q

How is a ventricular septal defect investigated?

A
  1. CXR - cardiomegaly, pulmonary oedema
  2. ECG - right ventricular hypertrophy
  3. Echo confirms diagnosis
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12
Q

What is the management for a small ventricular septal defect?

A

Monitor every 6 months and usually close spontaneously.

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13
Q

What is the management for a large ventricular septal defect?

A
  1. Surgical repair to prevent Eisenmenger’s syndrome.
  2. Heart failure - diuretics, ACEi, BB, fluid restriction, oxygen if hypoxia.
  3. High calorific NG tube for impaired feeding.
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14
Q

What is this describing?
Large left to right shunt transforms into right to left shunt as pulmonary hypertension as a result of heart failure has caused pulmonary pressure to become higher than systemic. Cyanosis develops.

A

Eisenmenger’s syndrome

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15
Q

What is the treatment for Eisenmenger’s syndrome?

A

Pulmonary vasodilation, heart and lung transplant.

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16
Q

What is this a presentation of?

Asymptomatic, split fixed S2, ejection systolic murmur 2nd left intercostal space.

A

Atrial septal defect

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17
Q

What age group does and atrial septal defect typically present in?

A

Young adults

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18
Q

How is an atrial/atrioventricular septal defect investigated?

A

CXR, ECG, Echo confirms diagnosis.

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19
Q

What is the management for an atrial/atrioventricular septal defect?

A
  1. Monitor and may close spontaneously.

2. Surgical repair

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20
Q

What are the two types of atrioventricular septal defects and what is the difference in where they are found?

A
  1. Complete - involves AV valves, creates one unifying valve.
  2. Partial - hole in lower portion of atrial septum with no ventricular or AV valve defect.
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21
Q

What are the clinical features of an atrioventricular septal defect?

A

No murmur, or ejection systolic murmur, or apical pansystolic murmur (mitral regurgitation - valve involved).

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22
Q

What is this a presentation of?

Preterm infant. Loud, continuous, harsh, low-pitched, ‘machinery’ murmur below left clavicle.

A

Patent ductus arteriosus

23
Q

How is a patent ductus arteriosus investigated?

A
  1. CXR and ECG usually normal

2. Echo to ensure no duct dependent circulation

24
Q

What is the management for a patent ductus arteriosus?

A
  1. Indomethacin (inhibits PG synthesis) to close duct at 24 and 48 hours.
  2. If preterm then will probably close without treatment.
  3. Consider endovascular surgery if persistent >1 month since birth.
25
Q

What is the pathophysiology of obstructive heart disease?

A
  1. Narrowed heart valves/vessels.
  2. Causes resistance to blood flow.
  3. Heart chamber must pump harder to overcome.
  4. Hypertrophy of heart muscle.
26
Q

What are the three types of aortic/pulmonary stenosis in infants?

A
  1. Valvular narrowing (2 leaflets instead of 3)
  2. Supravalvular narrowing
  3. Subvalvular narrowing
27
Q

What is this a presentation of?
Slow rising carotid pulse, thrill over carotid artery, left ventricular heave, ejection systolic murmur over aortic area, radiates to carotids.

A

Aortic stenosis

28
Q

What other clinical feature is present in severe aortic/pulmonary stenosis and severe aortic coarctation?

A

Secondary heart failure

29
Q

What other clinical feature is present in older children with aortic/pulmonary stenosis?

A

Aortic - syncope

Pulmonary - breathlessness

30
Q

How is aortic stenosis investigated in infants?

A
  1. Diagnose on Echo
  2. CXR - prominent left ventricle
  3. ECG - LVH
  4. Low systolic BP
31
Q

What is the management for aortic/pulmonary stenosis?

A
  1. Annual Echo

2. Severe - balloon valvoplasty/open valvotomy

32
Q

What is this a presentation of?

Right ventricular heave, thrill in pulmonary area, ejection systolic murmur in pulmonary area.

A

Pulmonary stenosis

33
Q

How is pulmonary stenosis investigated in infants?

A
  1. Diagnose on Echo
  2. CXR - if severe, large RA and RV
  3. ECG - RAD, RVH
  4. Normal BP
34
Q

What is this describing?

Narrowing of the descending aorta, close to ductus arteriosus, distal to left subclavian artery.

A

Coarctation of the aorta

35
Q

When and why does coarctation of the aorta present?

A
  1. Either at birth of gradually in the weeks after birth.

2. Patent ductus arteriosus bypasses narrowing so presents once this closes.

36
Q

What is this a presentation of?
Weak femoral pulses (no femoral pulse if critical), radiofemoral delay, pansystolic murmur in left infraclavicular area radiating to back.

A

Coarctation of the aorta

37
Q

How is aortic coarctation investigated in infants?

A
  1. Diagnose on Echo and 4 limb blood pressure
  2. CXR - prominent LV, rib notching from collateral vessels.
  3. ECH - LVH
  4. Increased systolic BP in upper limbs
38
Q

What is the management of coarctation of the aorta in infants?

A
  1. Neonatal surgery - resection of narrowed segment and two healthy ends sewn together.
  2. If recurrence/diagnosed in older children - balloon dilation
39
Q

Which heart defects cause acyanotic heart disease?

A

VSDs, ASDs, AVSD, PDA

40
Q

Which heart defects cause obstructive heart disease?

A

Aortic/pulmonary stenosis and coarctation of the aorta

41
Q

Which heart defects cause cyanotic heart disease?

A

Tetralogy of Fallot, transposition of the great arteries

42
Q

What is this describing?

Cyanotic, good systemic flow, no pulmonary oedema, blue baby, generally well and grow well.

A

Cyanotic heart disease

43
Q

What are the four aspects of a Tetralogy of Fallot?

A
  1. Large membranous VSD
  2. Pulmonary stenosis
  3. Overriding aorta
  4. Right ventricular hypertrophy
44
Q

What is the pathophysiology of Tetralogy of Fallot?

A
  1. Blood bypasses lungs because of right to left shunt.

2. Cyanosis occurs as blood is preferentially moved through VSD rather than through narrowed pulmonary valve.

45
Q

What is this a presentation of?

Pink at birth, cyanosis in first few weeks of life, ejection systolic murmur on left sternal edge, digital clubbing.

A

Tetralogy of Fallot

46
Q

How is Tetralogy of Fallot investigated?

A
  1. Sats 80-low 90%
  2. Diagnose on Echo
  3. CXR - boot shaped heart
  4. ECG - RAD, RVH
47
Q

What is the management for Tetralogy of Fallot?

A
  1. Discharge with surgical correction at 6-12 months - pulmonary stenosis and VSD closed.
  2. Administering oxygen to babies with cyanotic heart defects does NOT make a difference.
48
Q

What is a TET spell and how does it present?

A
  1. Sudden narrowing in Tetralogy of Fallot.

2. Blue skin/nails/lips after crying, feeding, or when agitated due to rapid drop in oxygenation.

49
Q

What is the management for a TET spell in Tetralogy of Fallot?

A

Squatting - increases TPR which reduces right to left shunt and increases pulmonary blood flow.

50
Q

What is this describing?

Aorta and pulmonary artery have swapped over, results in parallel circulation.

A

Transposition of the great arteries

51
Q

How can the parallel circuits in transposition of the great arteries mix?

A

If a VSD or patent ductus arteriosus is also present.

52
Q

What is this a presentation of?

Cyanosed from birth, no murmur, digital clubbing, low sats.

A

Transposition of the great arteries

53
Q

How is transposition of the great arteries investigated?

A
  1. Echo to diagnose
  2. CXR - ‘egg on string’ appearance, narrow upper mediastinum
  3. Normal ECG
54
Q

What is the management for transposition of the great arteries?

A
  1. Start immediately on prostaglandin infusion (Alprostadil) to maintain patent ductus arteriosus.
  2. Balloon septostomy (form hole in septum) quickly.
  3. Definitive arterial switch surgical procedure.
  4. Avoid oxygen as it can cause ductus arteriosus to close.