Paeds Cardio Flashcards

1
Q

What is tetralogy of fallot and its 4 features ?

A
  • key issue: interior dislocation of the septum which partially obstructs RVOT and PA
    1. ventricular septal defect: leading to equal ventricular pressures
    2. pulmonary stenosis
    3. overriding aorta
    4. RV hypertrophy
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2
Q

What is the pathophysiology of a VSD and overriding aorta in tetralogy of fallot?

A
  • VSD allows blood to flow between ventricles
  • overriding aorta: aortic valve is above VSD allowing deoxygenated blood into aorta
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3
Q

What is stenosis of the pulmonary valve in tetralogy of fallot?

A
  • greater resistance against blood flow from RV
  • encourages blood to flow through VSD and into aorta
  • causing R to L shunt and cyanosis
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4
Q

What is RV hypertrophy in tetralogy of fallot?

A
  • inc muscular strain on RV
  • trying to pump blood against resistance of LV
  • causes RV hypertrophy and thickening of heart muscle
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5
Q

How is cyanosis caused in tetralogy of fallot?

A
  • all factors cause R to L cardiac shunt
  • blood bypasses lungs and is not oxygenated
  • deoxygenated enters systemic circulation
  • causes cyanosis
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6
Q

What is the presentation of tetralogy of fallot?

A
  • cyanosis
  • clubbing
  • dyspnoea on feeding, crying, exertion
  • Tet spells: sudden onset dyspnoea/cyanosis
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7
Q

What are the signs of Tetralogy of Fallot?

A
  • ejection systolic murmur
  • heard loudest in pulmonary area: 2nd intercostal space on left sternal border
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8
Q

What are risk factors for Tetralogy of Fallot?

A
  • rubella
  • age of mother >40
  • alcohol in pregnancy
  • diabetic mother
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9
Q

What is seen on investigation of tetralogy of fallot?

A
  • echocardiogram
  • doppler flow studies
  • boot shaped heart due to RV thickening
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10
Q

What is the pathophysiology of a Tet spell?

A
  • intermitten symptomatic period where R to L shunt gets worse
  • occurs when pulmonary vascular resistance inc or systemic resistance dec
  • blood going from RV to aorta and bypassing pulmonary vessels
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11
Q

What is the presentation of a Tet spell?

A
  • waking, crying
  • irritability, cyanosis, SOB
  • Severe: reduced consciousness and seizures
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12
Q

How is a Tet spell treated?

A
  • older children squat
  • younger: knees to chest
  • increases systemic resistance
  • supplementary oxygen
  • β blockers
  • IV fluids
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13
Q

How is tetralogy of fallot managed?

A
  • VSD patched
  • PV widened or replaced
  • neonates: prostaglandin infusion to maintain ductus arteriosus
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14
Q

What is a ventricular septal defect?

A
  • most common form of congenital heart disease
  • malformation of ventricular septum
  • if LV pressure > RV then blood flows LV to RV
  • inc blood flow through lungs
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15
Q

Why are patients acyanotic in VSD?

A
  • blood flows L to R and flows around the lungs again
  • therefore blood is oxygenated
  • L to R shunt causes R side overload
  • R heart failure and inc flow into pulmonary vessels
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16
Q

What is Eisenmenger syndrome?

A
  • inc blood in RV > inc pulmonary > pulmonary hypertension
  • causes R to L shunt due to inc pressure in R side
  • patient becomes cyanotic as blood is bypassing lungs
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17
Q

What genetic conditions is a VSD associated with?

A
  • Down’s syndrome
  • Turner’s syndrome
18
Q

How does a VSD present on examination?

A
  • pan systolic murmur at left lower sternal border
  • in 3rd-4th intercostal spaces
  • systolic thrill on palpation
19
Q

How does a VSD present?

A
  • poor feeding
  • dyspnoea
  • tachypnoea
  • failure to thrive
  • can be symptomless and present in adulthood
20
Q

How is a VSD diagnosed?

A
  • gold standard: echocardiogram
  • CXR, ECG
21
Q

How is a VSD managed?

A
  • small VSDs can close spontaneously
  • transvenous catheter closure via femoral vein
  • percutaneous/open heart surgery, patch application
  • risk of infective endocarditis: Abx prophylaxis
22
Q

What is an atrial septal defect?

A
  • congenital heart defect causing a shunting of blood from L to R atria
  • inc flow into right heart and lungs
  • secundum ASD: patent foramen ovale
  • leads to right heart dilation in severe
23
Q

What is the pathophysiology of an ASD?

A
  • defect in the endocardial cushion leads to ASD
  • leads to shunt from L to R atrium
  • blood flows through PV and lungs
  • inc flow causes R sided overload and strain
  • can lead to R side HF
24
Q

What are complications of ASD?

A
  • stroke (VTE)
  • AF
  • pulmonary hypertension
  • eisenmenger syndrome
25
Q

How does an ASD present on examination?

A
  • mid-systolic, crescendo-descendo murmur
  • loudest at upper left sternal border
  • pulmonary flow murmur
  • fixed split S2 heart sound as PV and aortic valve close at diff times
26
Q

How does ASD present?

A
  • SOB
  • difficulty feeding
  • poor weight gain
  • LRTI
27
Q

How is ASD managed?

A
  • transvenous catheter closure
  • open heart surgyer
  • anticoags to dec risk of clot and stroke
28
Q

What is an atrio-ventricular septal defect (AVSD)?

A
  • Involves the ventricular septum, the atrial septum, the mitral and tricuspid valves
  • Can be complete or partial
29
Q

What is a patent ductus arteriosus (PDA)?

A
  • Persistence of the connection between the aorta and pulmonary artery
  • normally rise in PaO2 and decline in prostaglandins close the duct
30
Q

What is the pathophysiology of a PDA?

A
  • pressure in aorta higher than pulmonary vessels
  • creates L to R shunt
  • blood flows from aorta to PA
  • leads to pulmonary hypertension, R heart strain, RV hypertrophy
  • inc blood flow > LV hypertrophy
31
Q

How does a patent ductus arteriosus present?

A
  • tachycardia and tachypnoea
  • SOB
  • difficulty feeding
  • poor weight gain
  • LRTI
32
Q

How does a PDA present on examination?

A
  • continuous crescendo-descendo murmur
  • 2nd heart sound difficult to hear
33
Q

How is PDA diagnosed and managed?

A
  • diagnosis: echocardiogram
  • management: usually closed via cardiac catheterisation
34
Q

What is coarctation of the aorta and how does this present in severe and mild patients?

A
  • narrowing of the aorta at the site of the ductus arteriosus
  • presents with htn and murmur
35
Q

How does coarctation of the aorta present?

A
  • right arm hypertension
  • bruits over scapulae and back
  • murmur
36
Q

How is coarctation of the aorta managed?

A
  • repaired by surgical or percutaneous intervention - narrow section removed
37
Q

What is transposition of the great arteries?

A
  • where the aorta and pulmonary trunk are swapped
  • RV pumps blood into aorta and LV into PA
38
Q

What conditions is transposition of the great arteries associated with?

A
  • VSD
  • coarctation of the aorta
  • pulmonary stenosis
39
Q

Why is transposition of the great arteries life threatening?

A
  • two circulations don’t mix
  • baby is cyanosed as deoxygenated blood is going to the body
40
Q

How does transposition of the great arteries present?

A
  • diagnosed in antenatal USS scans
  • if undetected, presents with cyanosis at birth
  • resp distress, tachycardia
  • poor feeding, poor weight gain
41
Q

How is transposition of the great arteries managed with other defects?

A
  • if VSD, this allows time for treatment
  • prostaglandin infusion maintains ductus arteriosus
  • balloon septostomy to create larger ASD
42
Q

What is the definitive management of transposition of the great arteries?

A
  • open heart surgery
  • cardiopulmonary bypass machine
  • perform arterial switch