Congenital Heart Disease Flashcards

1
Q

What is congenital heart disease?

A
  • Abnormality of the structure of the heart
  • Present at birth
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2
Q

What are the three stages in the spectrum of severity of congenital heart disease and how are they defined?

A
  1. Mild - asymptomatic and may resolve spontaneously (can progress)
  2. Moderate - requires specialist intervention and monitoring in a cardiac centre
  3. Severe – present severely ill / die in newborn period or early infancy
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3
Q

What is major congential heart disease?

A

Heart disease that requires surgery within the first year of life

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4
Q

What is antenatal screening?

A
  • Ultrasound at 18-22 weeks
  • 4 chamber heart view and outflow tract view
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5
Q

Outline the management of an antenatally diagnosed CHD

A
  • Disease dependant
  • Expert team puts plan in place
  • May decide to deliver in cardiac surgical centre
  • Prostaglandin infusion if duct dependant lesion
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6
Q

What is newborn screening?

A
  • Clinical examination at 24hrs of age
  • Femoral pulses, heart sounds and presence of murmurs
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7
Q

What would happen if there was a murmur found on newborn screening pointinf to a small muscular VSD?

A
  • Murmur early in life
  • No haemodynamic consequences
  • May close spontaneously
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8
Q

What is the differential of cyanosis in the newborn?

A
  • Cardiac disease (blue with litte/no respiratory distress, may have pre/post ductal differential)
  • Respiratory disease (increased WOB and xray changes)
  • Persistent pulmonary hypertension of the newborn (PPHN) - (otherwise very unwell babies, large pre-post ductal differential)
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9
Q

What is transposition of the great vessels?

A
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10
Q

When does the duct close normally in transpostion of the great vessels and what symptoms present?

A
  • 2-7 days
  • Severe cyanosis or pallor
  • Tachyponea, distress
  • Rapid deterioration to death
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11
Q

What are the signs of duct closure with transposition of the great vessels?

A
  • Pallor
  • Prolonged CRT
  • Poor or absent pulses
  • Hepatomegaly
  • Crepitations
  • Acidosis
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12
Q

What is the treatment of duct closure with transposition of the great vessels?

A
  • ABC
  • Prostaglanding E2 to open duct
  • Multisystem supportive treatment
  • Transfer to cardiac surgical centre for definitive management
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13
Q

What are some duct dependant systemic circulation conditions?

A
  • Hypoplastic left heart
  • Critical aortic stenosis
  • Interrupted aortic arch
  • Critical coarctation of aorta
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14
Q

What are some duct dependant pulmonary circulation conditions?

A
  • Tricuspid atresia
  • Pulmonary atresia
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15
Q

What is hypoplastic left heart?

A
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16
Q

What is pulmonary atresia?

A
17
Q

When do babies normally present with cardiac failure?

A
  • Seen with moderate to large left to right shunts
  • Increased pulmonary flow, decreased ventricular load
  • Tend to present a few weeks as pulmonary pressures drop
18
Q

What are the clinical signs of heart failure in babies?

A
  • Failure to thrive
  • Slow / reduced feeding
  • Breathlessness (especially when feeding)
  • Sweatiness
  • Hepatomegaly
  • Crepitations
19
Q

When do moderate/large VSD murmurs present? Why?

A
  • There is a big defect so less of a gradient
  • Often no murmur at baby check
  • Murmur develops as pulmonary pressures drop over first week
  • Increased pulmonary circulation, congestive cardiac failure
20
Q

What is the repair for patent ductus arteriosus?

A
  • Catheter procedure
  • Couple of follow ups
    • Ensure flow stopped
    • Device in correct position
  • Discharged
21
Q

What is the repair for VSD?

A
  • Closure by patch
  • Follow up during childhood/adolescence
  • Rhythm or valve problems
  • Generally expect to go on to a normal life
22
Q

What is the repair for HLHS?

A
  • 3 Stage complex surgery
  • Significant mortality at each stage and between
  • Ends with RV supplying systemic circulation
  • Will fail over time
  • Transplant