?????? Congenital Heart Disease (CHD)

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?????? Congenital Heart Disease (CHD)
(?)
Department of Pediatrics Soochow University
Affiliated Childrens Hospital

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Patent Ductus Arteriosus(PDA)??????
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Learning objectives

• You should
• Know the signs , symptoms, diagnostic features
  and management of the common acyanotic congenital
  heart disease PDA

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PDAconcept 1

1. Which is ductus Arteriosus?

2. Ductus close in response to the rise in Po2
,blood pH and prostacyclin after birth
3. If this mechanism fails or is reserved
by prostaglandin E2, the resulting connection
allows blood to flow under pressure from the
aorta into the pulmonary arteries
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PDAconcept 2

1. In a term infant ,ductus ateroisus closed
   spontaneously in 3 months in most infant cases.
2. Ductus arteroisus remained patent after one year
   old or more named PDA

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PDAconcept 3
1.In a term infant ,PDA is the result of a
deficiency in the structural framework of the
vessel wall. 2. In the preterm infant is the
result of a delay in closure. Therefore, although
100 of premature babies born at 29weeks of
gestation will have a PDA, in the vast majority
this closes spontaneously. 3. In contrast, 6 of
all term newborn have a persistent connection
between the bifurcation of the pulmonary arteries
and the aortic arch.
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Patent Ductus Arteriosus(PDA)

1. L--R shunt CHD
2. 10 of CHD
3. Twice as common in females as in males
4. In preterm infant weighing less than 1500Kg,the
   frequency of PDA 20-60
5. Associated lesions CoA ,or VSD(sometimes)

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Types of PDA
funnel??
tubiform??
window??
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Hemodynamics changes

• The blood in lung field increased, Blood in
  systemic circulation decreased
• Pulmonary hypertension(PH) ,reversible --------
  irreversible Eisenmenger syndrome
• Cardiac enlargement (LV,LA)
• Diameter of ascending aorta is large to normal
• A widened Pulse Pressure

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Symptoms (depending on the shunt of
PDA)

• None (most common)
• recurrent chest infections
• Heart failure with large shunt

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Signs (depending on the shunt of PDA)

1. None (most common)
2. Pink, normal or large volume, bounding
   /collapsing pulse
3. BP shows wide Pulse Pressure
4. Precordium is hyperdynamic with LV impulse at
   apex
5. Thrill at left infraclavicular area and second
   left intercostal space possible
6. Loud P2 with pulmonary hypertension
7. Third heart sound (S3) with CCF
8. Pulmonary crepitations and hepatomegaly with CCF
9. Continuous waterwheel/machinery murmur loudest at
   upper LSE, left infraclavicular area and back

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Practice typical murmur of PDA
e.g. Grade ?--? /? continuous, machinery murmur
at the second LSE

Typical murmur of PDA 1. Continuous
waterwheel/ machinery murmur 2. Loudest
murmur at upper LSE, left infraclavicular area
and back
CM
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Investigation

• Chest X-ray (CXR)
• Electrocardiography(ECG)
• Echocardiography(2DE)
• Cardiac catheterization and angiocardiography

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Chest X-ray

1. Pulmonary plethora
2. The main pulmonary artery segment dilated
3. Cardiomegaly (LV,LA)
4. Diameter of ascending aorta is Large to normal

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2
1
3
PDA case
Normal
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ECG (typical PDA)

1. Normal or left axis deviation
2. LA enlarged , LV hypertrophy

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Echocardiography

• The anatomic location
• (the size and shunt of PDA)
• Color flow doppler
• (the direction of the shunt)
• estimate the pressure
• pulmonary pressure or hypertension

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Cardiac catheterization and angiocardiography
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Course and prognosis

• Closure spontaneously in infant in the vast
  majority
• Adults with corrected defect have normal quality
  of life

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Management

• Medical management
• 1.fliud restriction
• 2.indomathacin and prostacyclin
• Interventional therapy
• 1.Implantation of various umbrella or coil
  device
• 2.The first choice of treatment
• Surgery ligation in premature infant

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Device for PDA closure
Amplatzer occluder device Diameter of PDAgt2.5mm.
Coiloccluder device (???) Diameter of
PDAlt2.5mm.
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Summary

• PDA is a kind of L to R shunt CHD,
• The symptom of PDA depends on the shunt.
• The characteristic heart murmur and P2
• Complication (1)Respiratory infection
  (2)congestive heart failure (3)endocarditis

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Summary

1. PDA can close spontaneously in infant
2. Enlarged chambers (LV,LA) can be observed by CXR
   , 2DE ,and ECG
3. Preventing PH is the key point during the
   management of PDA patients

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Question

• How to detect and estimate the PH in PDA patient
  in clinical experience? Why?
• Important Concept
• Pulmonary hypertension
• differential cyanosis (Eisenmenger syndrome)
• A widened Pulse Pressure

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Tetralogy of Fallot (TOF)?????
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Learning objectives

• You should
• Know the signs , symptoms, diagnostic features
  and management of the commonest cyanotic
  congenital heart disease-TOF

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Questions for TOF

• 1.The mechanism and clinical findings of
  hypercyanotic episode (spells)?
• How to treat it?
• 2. The mechanism of squatting suddenly in TOF
  patient?

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Anatomy of TOF
The aorta straddles both L and R ventricle
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Boot-shaped heart
Beneath the aortic outlet
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(RVOTO)
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Resulting from RVOTO
Bay(??) /Oligaemia (????)
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Hemodynamics changes

1. The blood in lung field decreased (oligemia)
2. Cardiomegaly (RV,RA)
3. Diameter of ascending aorta is larger to
   normal.

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Symptoms depending on the degree of RVOT
obstruction

• Cyanosis (variable, progressive)
• hypercyanotic episode /blue spells /
• Hypoxemic spells ????
• aged 2years or less
• Squat suddenly after exertion
• to ward off hypercyanotic spells
• Exercise tolerance poor

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• Cyanosis (variable, progressive)

1.At birth the RVOT obstruction is usually not
severe and cyanosis may not be obvious.
2.but this becomes evident with increasing
activity, often when crawling commences around 10
months of age
3.Progressive hypoxemia results in compensatory
polycythaemia, including clubbing fingers and
toes (?????)
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Symptoms depending on the degree of RVOT
obstruction

• Cyanosis (variable, progressive)
• hypercyanotic episode /blue spells /
• Hypoxemic spells ????
• aged 2years or less
• Need to lie down/ Squat suddenly after exertion
• to ward off hypercyanotic spells
• Exercise tolerance poor

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Squat after exertion
Need to lie down/ Squat suddenly after exertion
to ward off hypercyanotic spells
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Symptoms depending on the degree of RVOT
obstruction

• Cyanosis (variable, progressive)
• hypercyanotic episode /blue spells /
• Hypoxemic spells ????
• aged 2years or less
• Squat suddenly after exertion
• to ward off hypercyanotic spells
• Exercise tolerance poor Need to lie down/

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Hypoxemic spells(????)

• Paroxysmal hypercyanotic episodes arise in
  untreated young children aged less than 2 years,
• Following defecation ??,crying or feeding .

• Blue spells are characterised by
• 1.Increasing irritability
• 2.Prolonged crying
• 3. Rapid deep respiratory movement
• 4.A dramatic exacerbation of cyanosis

• During blue spells, a significant increase in
  RVOT obstruction, blood flow through the outflow
  decrease ,and the systolic murmur disappears.
  (mechanism)

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signs depending on the degree of RVOT
obstruction

• Central cyanosis
• Plethoric appearance
• Hyperdynamic precordium with RV heave at left
  sternal edge
• Palpable systolic thrill at upper LSE in50
  patients
• S2 aortic and single (due to absent pulmonary
  component)
• Heart murmur Grade?--? /? rough ESM at upper
  LSE radiating to back

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Practice typical murmur of TOF
e.g. Grade ?--? /? ESM, P2 weaken or disappeared

1. Loud ESM at the upper LSE due to turbulence
   caused by the infundibular stenosis

ESM

1. The large VSD little turblence and therefore does
   not produce a murmur.

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Complications of TOF

1. Progressive cyanosis is associated with failure
   to thrive
2. Hypercyanotic spells may be associated with
   syncopal attacks
3. Cerebral ischaemia and thromboses usually occur
   in the first 2 years of life
4. Cerebral abscess develop in older children
5. Bacterial endocarditis and CCF are rare

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Investigation

• Blood routine
• Erythrocytosis , hyperglobulism and
  plasmahyperviscositysyndrome
• Avoiding dehydration such as diarrhea,
  vomiting and sweating
• Chest X-ray (CXR)
• Electrocardiography(ECG)
• Echocardiography(2DE)
• Cardiac catheterization and angiocardiography

?????
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Chest X-ray
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TOF Boot-shaped heart
Normal
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1. Pulmonary oligaemia
2. Small pulmonary conus, (concave)
3. Cardiomegaly (RV,RA)
4. Diameter of ascending aorta is larger

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ECG (typical TOF)

1. Right axis deviation
2. RV hypertrophy

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Echocardiography

• The anatomic location
• Color flow doppler
• the direction of the shunt
• estimate the pressure gradient
• of RVOT

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VSD
VSD
Over-riding ventricle septum
Over-riding ventricle septum
VSD
RV outflow obstruction
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Cardiac catheterization and angiocardiography

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Medical management

• Attempts to improve weight gain are essential
• An adequate haemoglobin should be maintained
  ,especially in patients with severe cyanosis and
  those with hypercyanotic spells
• Emergent treatment for.hypercyanotic spells
• 1.placed knee to chest position
  (stimulated squatting)
• 2.Given oxygen
• 3.intravenous sodium bicarbonate
  (acidosis ???)
• 4.Intravenous morphine
  (sedation, relief pain and RVOTO)
• 5. Regular oral Propranolol
  (???) until surgery

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Management---Surgery

• The palliative blalock-Taussig shunt
• improves pulmonary blood flow ,It is
  employed in severely cyanosed infants aged less
  than 6 months ,those who are medically unfit for
  a major procedure, and those with hypercyanotic
  spells
• The definitive repair
• involves total reconstruction of the RV
  outflow tract and closure of VSD, The operative
  mortality is less than 5

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Summary

• The commonest cyanotic CHD,
• R to L shunt
• The typical symptom
• 1. Cyanosis after the neonatal period
• 2. Hypercyanotic spells during
  infancy
• 3. Squatting suddenly after
  infancy
• The characteristic heart murmur and P2 decreased