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Cardiovascular Block: Week 7 > Pathology of Congenital Heart Disease > Flashcards

Pathology of Congenital Heart Disease Flashcards

1
Q

What is the commonest heart disease in children?

A

Congenital heart disease

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2
Q

What is the commonest heart disease in children?

A

Congenital heart disease

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3
Q

What is congenital heart disease?

A

Abnormalities of the heart present at birth.

  • Most arise from faulty embryogenesis during gestational weeks 3-8 when major cardiovascular structures develop.
  • Usually a structural abnormality.
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4
Q

What are possible outcomes of congenital heart disease?

A

More severe anomalies may be incompatible with intra-uterine life.
Some produce manifestations soon after birth whilst others may not become evident until adult life.
- Most can be surgically corrected.

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5
Q

What proportion of live births present with congenital heart disease?

A

1% of live births

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6
Q

What is the etiology of congenital heart disease?

A
  1. Idiopathic
  2. Environmental (maternal rubella, diabetes, teratogens)
  3. Multiple genes involved in morphogenesis of the heart e.g. Downs Syndrome
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7
Q

What are some of the clinical consequences of congenital heart disease?

A
  1. Cyanosis
  2. Pulmonary vascular hypertension
  3. Cardiac enlargement
  4. Impaired growth and development
  5. Cerebral thrombosis (polycythemia)
  6. Paradoxical embolus leading to brain infarcts
  7. Infective endocarditis
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8
Q

What is a shunt?

A

Abnormal communication between chambers or blood vessels

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9
Q

Congenital heart defects can be classified into what 3 groups?

A
  1. Right to Left Shunts
  2. Left to Right Shunts
  3. Obstructive Vascular Flow
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10
Q

What occurs in right-to-left shunting?

A

The pulmonary circulation is bypassed.
- Poorly oxygenated blood enters systemic circulation.
= Cyanosis

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11
Q

What occurs in left-to-right shunting?

A

Increased pulmonary blood flow

- Not associated with cyanosis (initially)

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12
Q

What changes occur due to left-to-right shunting?

A

Expose the low pressure, low resistance pulmonary circulation to increased pressures and volumes.
- Leads to adaptive changes that increase lung vascular resistance
= Resulting in right ventricular hypertrophy and eventually failure

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13
Q

What is Eisenmenger Syndrome?

A

With time, increased pulmonary resistance can cause shunt reversal (changes from left-to-right to right-to-left shunt)
- Reversal of flow and shunting of deoxygenated blood into systemic circulation is called Eisenmenger Syndrome
= Cyanosis

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14
Q

What is Obstructive Vascular Flow?

A

Narrowing of the chambers, valves or major blood vessels

- Can be associated with a shunt

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15
Q

What are the three left-to-right shunts?

A
  1. Atrial septal defect (ASD)
  2. Ventricular septal defect (VSD)
  3. Patent ductus arteriosus (PDA)
    * NB: All = acyanotic
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16
Q

What are the three left-to-right shunts?

A
  1. Atrial septal defect (ASD)
  2. Ventricular septal defect (VSD)
  3. Patent ductus arteriosus (PDA)
    * All = acyanotic
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17
Q

What is the Foramen Ovale?

A

Opening that allows oxygenated blood from the maternal circulation to flow from the right to the left atrium, sustaining fetal development.
- 20% ASD = patent foramen ovale at birth (usually closes spontaneously following birth

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18
Q

What is ASD?

A

An abnormal fixed opening in the atrial septum

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19
Q

How does ASD present clinically?

A

Asymptomatic until adulthood, then results in pulmonary hypertension

20
Q

What is the most common congenital cardiac anomaly/

A

VSD

21
Q

What proportion of VSDs occur in isolation?

A

20-30% of VSDs occur in isolation

- most are associated with other cardiac malformations

22
Q

What proportion of VSDs occur in isolation?

A

20-30% of VSDs occur in isolation

- most are associated with other cardiac malformations

23
Q

What symptoms are associated with small VSDs?

A

Small VSDs may be asymptomatic

24
Q

What symptoms are associated with larger VSD defects?

A

Larger defects result in chronic severe left-to-right shunting, complicated by pulmonary hypertension and congestive heart failure
- Also get reversal of shunt and cyanosis (Eisenmenger)

25
Q

How should VSDs be treated?

A

Require early surgical intervention

26
Q

What is the ductus arteriosus?

A

The ductus arteriosus arises from the left pulmonary artery and joins the aorta just distal to the origin of the left subclavian artery.

27
Q

What is the purpose of the ductus arteriosus in intrauterine life?

A

Permits blood flow from the pulmonary artery to the aorta, thereby bypassing the unoxygenated lungs

28
Q

How soon after birth does the ductus arteriosus close?

A

Closed after 1 - 2 days

29
Q

What proportion of PDAs are isolated?

A

The great majority of patent ductus arteriosus (90%) are isolated defects

30
Q

What symptoms are associated with a small PDA?

A

No symptoms

31
Q

What symptoms are associated with a large PDA?

A

Larger PDA eventually leads to reversal of shunt and cyanosis

32
Q

When is a PDA beneficial?

A

May be necessary to keep the ductus arteriosus patent if other cardiac abnormalities are present e.g. aortic atresia which would otherwise result in death of the infant

33
Q

What are the two right-to-left shunts?

A
  1. Tetralogy of Fallot
  2. Transposition of the Great Arteries
    * NB: These = cyanosis!
34
Q

What is the prevalence of Tetralogy of Fallot?

A

Most common cause of cyanotic congenital heart disease, accounts for 5% of all congenital cardiac malformations.

35
Q

What are the 4 features of Tetralogy of Fallot?

A
  1. VSD (usually large)
  2. Right ventricular outflow tract obstruction (subpulmonic stensosis)
  3. Overriding of the VSD by the aorta
  4. Right ventricular hypertrophy
36
Q

What morphology is seen in Tetralogy of Fallot?

A
  1. Heart is large and “boot-shaped” as a consequence of right ventricular hypertrophy
  2. Proximal aorta is dilated
  3. Pulmonary trunk is hypoplastic
  4. Right ventricular hypertrophy
  5. Left sided chambers normal size
37
Q

What are the clinical features of Tetralogy of Fallot?

A
  1. Cyanosis
  2. Outflow obstruction protects lung from pressures
  3. Sequelae of cyanosis: polycythemia, hyperviscosity, thrombotic diathesis, IE, paradoxical embolism
38
Q

What is a paradoxical embolism?

A

Embolus that arises from the right rather than the left side (Emboli usually arise from the left)

39
Q

What is transposition of the great arteries?

A

Aorta arises from the right ventricle

Pulmonary artery arises from the left ventricle

40
Q

What is the result of transposition of the great arteries?

A

Separation of the systemic and pulmonary circulations, incompatible with postnatal life unless a shunt is present (VSD) for adequate mixing of blood and delivery of oxygenated blood to the aorta.

41
Q

What is a stable shunt in transposition of the great arteries?

A

VSD (present in ⅓)

42
Q

What is an unstable shunt in transposition of the great arteries?

A

PDA (can close therefore not stable)

43
Q

What are the two types of aortic coarctation?

A
  1. “Infantile” (preductal) coarctation

2. “Adult” (postductal) coarctation

44
Q

What is “Infantile” (preductal) coarctation?

A

Characterized by the circumferential narrowing of the aortic segment between the left subclavian artery and the ductus arteriosus.

45
Q

What is “Adult” (postductal) coarctation?

A

The aorta is sharply constricted by a tissue ridge adjacent to the non-patent ligamentum arteriosum.

Cardiovascular Block: Week 7 (5 decks)

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