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HD2 > Congenital Heart Disease and other Abnormalities > Flashcards

Congenital Heart Disease and other Abnormalities Flashcards

1
Q

What is the cardiac embryology?

A
  • clusters of angiogenic cells from mesodermal cardiogenic plate arising from right and left endocardial tubes
  • day 21: lateral folding brings tubes together forming primitive heart tube
  • day 23: heart beats
  • tube lengthens and folds further into bulboventricular loop
  • day 28: septation of the atria, ventricles and outflow tract
  • over 2nd and 3rd trimester: long periods and maturation
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2
Q

What does failure of the septation of the atria cause?

A

CHD

  • early (AVSD from endocardial cushion)
  • late (simple ASD or VSD)
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3
Q

What are the anatomical connections in fetal circulation?

A
  • foramen ovale (bypasses pulmonary circulation)
  • ductus arteriosus
  • ductus venosus (1/3 of blood supply from umbilical vein)
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4
Q

What is the purpose of anatomical connections in fetal circulation?

A
  • high resistance pulmonary circulation

- low resistance systemic circulation

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5
Q

What is the purpose of high pressure in fetal circulation?

A

High pressure pulmonary circulation forces most blood through ductus arteriosus into systemic circulation (diverts it from pulmonary circulation)

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6
Q

What closes during pregnancy?

A
  • closure of ductus arteriosus
  • closure of foramen ovale
  • closure ductus venosus
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7
Q

What is the purpose of the ductus arteriosus closing?

A
  • increased oxygen levels

- in utero ductus is kept open under PG E1 influence

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8
Q

What is the purpose of formen ovale closing?

A
  • drop in pressure in pulmonary circulation/right side of heart
  • shunting reversed and valve closes
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9
Q

What is the purpose of ductus venosus closure?

A
  • decrease in blood flow in IVC
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10
Q

What is persistent pulmonary hypertension of the newborn?

A
  • if problems with lungs (pneumonia, aspirate meconium(
  • increased pressure in pulmonary artery keeping foramen ovale open
  • shunting of deoxygenated blood into systemic circulation
  • cyanotic baby
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11
Q

What is cyanosis?

A
  • patient blue
  • blue produced by amounts of deoxygenated Hb (g/l) not percentage saturation
  • Hb>50g/l in capillaries OR 34/l in arterial blood
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12
Q

How does CHD present?

A
  • normal alveolar gas exchange
  • no dyspnoea
  • normal pulmonary venous saturations
  • results from shunting of deoxygenated blood from right to left
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13
Q

How does lung disease present?

A
  • impaired alveolar gas exchange
  • tachypnoea and recession
  • reduced pulmonary venous saturations
  • results from oxygen diffusion problems or ventilation-perfusion mismatch within the lung
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14
Q

What is the main difference between lung disease and CHD?

A

CHD - no problem with functioning of lungs so normal oxygenation
Lung disease - limited gas exchange so body not perfused

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15
Q

What occurs in transposition of great vessels?

A
  • aorta connected to RV, pulmonary connected to LV forming 2 separate connections
  • baby stays alive because of blood mixing in foramen ovale and ductus arteriosus
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16
Q

What is teratology of fallot?

A
  • ventricular septal defect
  • overriding aorta (aorta above septal defect)
  • right ventricular hypertrophy
  • stenosis of RV (narrow outflow)
17
Q

What are other forms of CHD?

A
  • tricuspid atresia (complete valve closure)
  • pulmonary valve atresia
  • critical pulmonary stenosis
  • truncus arteriosus (single artery from heart, large ventricular septal defect below valve of trunk)
  • total anomalous pulmonary venous drainage (pulmonary veins not connected to LA but to one of veins draining back to RA)
18
Q

Define acyanotic?

A

Patient pink

19
Q

What are the 2 major groups of acyanotic congenital problems?

A
  • left to right shunts: increase pulmonary blood flow = pulmonary oedema/hypertension
  • left heart outflow tract obstruction: pulmonary oedema/impaired tissue perfusion/lactic acidosis (increased back pressure on pulmonary veins -> pulmonary circulation)
20
Q

How can cyanosis occur as a secondary feature of acyanosis?

A
  • pulmonary oedema impairs gas exchange
  • pulmonary hypertension causes right to left shunting as there is higher pressure on right side = Eisenmenger shunt
  • dangerous = if closed will develop right heart failure
21
Q

What is ventricular septal defect?

A
  • pressure in left ventricle is higher than right -> shunt
22
Q

What is preductal coarctation of the aorta?

A
  • causes pulmonary oedema as increased pulmonary pressure

- preductal: before ductus arteriosus

23
Q

What are other forms of cyanotic CHD?

A
  • atrial septal defect
  • atrioventricular septal defect
  • critical aortic stenosis
  • patent ductus arteriosus
24
Q

What are the main complex mixed presentations?

A

Hypoplastic left heart

Double outlet right ventricle (aorta connects to right ventricle instead of left)

25
Q

What is hypoplastic left heart?

A
  • no blood flow through left ventricle to aorta
  • patient reliant on fetal circulation to stay alive
  • blood flows through foramen ovale into right ventricle -> pumped to rest of body
26
Q

How may ductus arteriosus and foramen ovale delayed presentation/treatment?

A
  • bypass obstruction (teratology of fallot, pulmonary atresia, coarctation, hypoplastic left heart)
  • allow mixing (transposition)
  • both delay presentation as mild cyanosis easily missed and only get symptoms once ductus closes
  • reopen ductus with PG E/enlarge foramen ovale with balloon septostomy transposition
27
Q

How do you treat CHD?

A
  • depends on condition
  • monitoring
  • diuretics for pulmonary oedema
  • re-open ductus arteriosus with PG E
  • surgery and catheter procedures
28
Q

Symptoms of acyanotic CHD?

A
  • expectant small vascular VSDs PDA and ASD/PFO may close spontaneously
  • diuretics and prostaglandins
29
Q

What would definitely correct acyanotic CHD?

A
  • percutaneous catheter closure of PDA
  • balloon dilatation of valvular stenosis
  • repair of coarctation
  • open heart surgery for VSD/ASD
30
Q

What are the limiting factors in treatment of CHD?

A
  • anatomical@ disuse atrophy (cannot grow new ventricles)

- functional: chronically elevated pulmonary blood flow (irreversible pulmonary hypertension)

31
Q

What neural tube defects are there?

A
  • failure of normal zipping of neural tube
  • spina bifida occulta (failure of one or more vertebrae in spine to form properly)
  • meningocoele (meninges protrude form spinal column)
  • encephalocoele (protrusion of neural tissue from head)
  • anencephaly (absence of major portion of brain, skull and scalp)
32
Q

What is the most serious form of spina bifida occulta?

A
  • myelonmengocoele: neural tissue exposed on babies back
33
Q

How is myelomeningocele treated?

A
  • closure reduces risk of infection but does not restore neural function
  • closure may lead to hydrocephalus (plastic catheter through valve running under skin into peritoneal cavity to be rapidly absorbed into circulation)
34
Q

What are the neurological consequences of lumbar myelomeningocele?

A
  • mixed sensory, motor and autonomic problems (dependent on level of lesions and degree of neural disruption)
  • loss of bladder control, faecal incontinence and loss of sensation in legs
35
Q

What are the abdominal wall defects?

A

Gastroschisis

Exomphalos

36
Q

What are the features of gastrochisis?

A
  • full thickness small defect in abdominal wall lateral to umbilicus (bowel free within abdominal cavity)
  • surgical closure possible
  • bowel may take 1-3 months to start functioning normally
37
Q

What is exomphalos?

A
  • wide based defect
  • membrane covers herniated viscera
  • may be associated with other abnormalities/genetic disorders
38
Q

What is cleft lip?

A
  • common defect where maxilla fail to fuse with frontonasal process
  • complete surgical correction possible
  • can affect eustachian tube function (risk of conductive hearing loss)
39
Q

What is cleft palate?

A
  • common defect
  • failure of secondary palate to fuse
  • philtrum formed
  • tongue too large and stops plated from turning horizontally
  • general failure of rotation
  • can affect eustachian tube function (risk of conductive hearing loss)

HD2 (22 decks)

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