Cyanotic Heart Diseases Flashcards

1
Q

What are the 5 main cyanotic heart diseases

A
  1. Truncus arteriosus
  2. Transposition of the great arteries
  3. Tricuspid atresia
  4. Tetralogy of fallot
  5. Total anomalous pulmonary vascular return (TAPVR)
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2
Q

How does cyanotic heart disease generally present

A

Blue baby
Feeding problems or failure to thrive
Exertional dyspnoea/tachypnoea
Hypoxaemia
Symptoms of HF
Nail clubbing

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3
Q

What test can be done when a cyanotic baby does not respond to oxygen supplementation

A

Hyperoxia/nitrogen washout test
1. Infant given 100% O2 for 10 minutes
2. ABG taken
3. PO2 <15kPa = cyanotic heart disease

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4
Q

What are the most likely causes of cyanosis according to timeline

A

within minutes - TGA
Days - Tricuspid atresia
Months - tetralogy of fallot

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5
Q

Describe truncus arteriosus

A

Pulmonary artery and aorta become one vessel
Survival depends on the presence of a shunt - VSD

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6
Q

What is transposition of the great arteries and its risk factors

A

Anatomical reversal of the aorta and pulmonary artery → two closed loop systems

Fatal unless there is a shunt
- intracardiac (PFO/VSD/ASD)
- extracardiac (PDA)

RF: maternal diabetes

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7
Q

How does transposition of the great arteries present (symptoms and signs)

A

Postnatal cyanosis at rest (within minutes of birth)
Unaffected by supplemental oxygen
Tachypnoea
No murmur heard
Prominent right ventricular impulse

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8
Q

Investigation and Management of Transposition of the great arteries

A

CXR: Egg on a string sign (cardiomegaly)

Immediate pGE1 infusion e.g. alprostadil
or
Balloon septoplasty (artificial VSD)
→ 2 weeks later - surgery

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9
Q

What is tricuspid atresia

A

Absence of the tricuspid valve

Fatal unless there is a shunt (ASD + VSD)

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10
Q

How does tricuspid atresia present (Symptoms and Signs)

A

Central cyanosis

Pansystolic murmur (Due to ASD and VSD)

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11
Q

How is tricuspid atresia managed

A

Immediate PGE1 infusion e.g. alprostadil
→ 2 weeks - surgery

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12
Q

What is tetralogy of fallot

A

Simultaneous occurrence of 4 defects:
1. Right ventricular outflow tract obstruction (RVOTO) - pulmonary stenosis
2. Right ventricular hypertrophy
3. Ventricular septal defect (large)
4. Overriding aorta - shifts to sit on top of the VSD

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13
Q

What are the risk factors for tetralogy of fallot

A

DiGeorge Syndrome
Down’s syndrome
Maternal diabetes and alcohol consumption

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14
Q

How does tetralogy of fallot present (symptoms and signs)

A

Severe cyanosis (depending on RVOTO extent)
2-4 months: Hypercyanotic tet spells in response to stress (pain, crying, feeding)

Untreated: squatting relieves symptoms

Harsh ejection systolic murmur heard

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15
Q

What are the investigations and management for tetralogy of fallot

A

CXR: boot-shaped heart
ECG: RVH/Right axis deviation

Knee-chest position
High flow oxygen
Severe: PGE1 → surgical intervention within the 1st year

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16
Q

What is total anomalous pulmonary venous return

A

The pulmonary veins merge to form a connecting vein that drains into the systemic venous circulation e.g. vena cava instead of the left atrium

Survival depends on a shunt - usually ASD, sometimes PDA

17
Q

What is Ebstein’s anomaly and what are its risk factors

A

Atrialisation of the right ventricular, where the tricuspid valve is displaced downwards → small and hypocontractile RV → tricuspid regurg → RA dilation → RHF

In-utero lithium exposure

18
Q

How does Ebstein’s anomaly present

A

Depends on the degree of displacement
Mild: cyanosis
Severe: in-utero hydrops fetalis

Pansystolic murmur
Mid-diastolic murmur

19
Q

Ebstein’s anomaly investigations and management

A

Echo: confirms
CXR: cardiomegaly (RA enlargement)

Surgery

20
Q

What are ductal dependent conditions and how are they managed

A

A heart condition that requires a patent PDA to sustain systemic blood flow

Systemic blood flow:
- Transposition of the great arteries
- Coarctation of the aorta

Pulmonary blood flow:
- Tricuspid atresia
- Severe RVOTO i.e. in tetralogy of fallot

Management = PGE1 infusion