Congenital Heart Disease in Neonates

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Congenital Heart Disease in Neonates

• EGM Hoosen
• Paediatric Cardiology
• Inkosi Albert Luthuli Central Hospital

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How common is cardiac disease in children?

• Congenital Heart Disease 8/1000
• 3/1000 cardiac disease needing intervention in
  the first year.

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UK study

• More than half of babies with undiagnosed
  congenital heart disease which comes to light in
  infancy are missed by routine neonatal
  examination and more than one third by the 6 week
  examination  
• Wren et al
•    

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• A normal neonatal examination does not guarantee
  that the baby is normal and certainly does not
  exclude life threatening cardiovascular
  malformation
• A persistent murmur or any other sign of
  congenital heart disease should warrant prompt
  paediatric cardiac evaluation

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Antenatal diagnosis

• 20weeks gestation
• detection rate
• average 23
• range 3 68
• advantage
• early detection
• delivery in high risk unit

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Consequences of late/missed diagnosis

• Mortality
• Ischemic brain injury
• Multiorgan failure
• Higher postoperative morbidity

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Case 1

• Day 7 term neonate
• severe cyanosis
• Respiratory Distress
• Was discharged one day after a normal delivery
• Became suddenly ill and rushed to hospital

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Clinical findings

• ?Respiratory Disease
• Clinical examination
• CXR
• Oxygen administration -
• Blood gas pH 7.18 PO2 4kPa PCO2 3.5kPa
• BE -16

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Management

• Discusssed urgently ?cyanotic congenital heart
  disease
• Stabilised
• acidosis corrected
• Temperature
• Glucose
• Commenced on prostaglandins
• Iv fluids
• Monitored for apneoa

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• Urgent referral
• Diagnosis

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Why cyanotic congenital heart disease is often
missed at birth

• Cyanosis is not always apparent or always
  treated seriously immediately after birth.
• Cyanosis, particularly peripheral cyanosis, is
  common in newborns.
• Cyanosis that worsens on crying must be
  investigated further.
• Newborns with cyanotic congenital heart disease
  often look completely well initially-until the
  duct begins to close

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Congenital heart disease presenting with
cyanosis at or soon after birth

• Pulmonary atresia/VSD (13500 live births)
• Transposition of Great vessels (13500)
• Pulmonary atresia /Intact ventricular septum
• Critical pulmonary stenosis

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Prostaglandin administration

• Maintain a patent ductus arteriosus
• Intravenous infusion Prostaglandin
  E1(alprostadil)
• Oral prostaglandins Prostaglandin E2
• Side effects
• Apneoa
• Fever
• Gastrointestinal etc

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Management of pulmonary atresia

• Careful assessment by cardiologist
• Neonatal surgery Blalock Taussig shunt

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Transposition of great arteries
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Case 2

• D6 neonate
• Shock
• Cardiomegaly with gallop rhythm
• Severe metabolic acidosis with respiratory
  distress
• Normal at birth kept in hospital as mum unwell.
• Murmur noted soon after birth thought to be VSD
  elective appointment.

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Management

• Inotropes
• Antibiotics
• Prostaglandin administration
• Acidosis corrected
• Glucose 1.6mmols initially corrected
• Referred for cardiac evaluation

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Diagnosis
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Congenital heart disease presenting with shock in
the neonate

• Coarctation
• Interrupted aortic arch
• Critical aortic stenosis
• Hypoplastic left heart syndrome

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• Congenital heart disease must be excluded in all
  neonates presenting with shock or cardiac failure
• Careful comparison of upper and lower limb pulses
  essential in all neonates repeat if neonate
  becomes ill later
• Early maintenance of ductal patency can be
  lifesaving.

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Most common differential diagnoses of critically
ill neonates with congenital heart disease

• Septic shock
• Persistent pulmonary Hypertension of the Newborn
• Respiratory disease

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Pulse oxymetry

• Proper use of equipment
• Saturations persistently less than 96
• Differential saturations

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• Neonates and infants with central cyanosis or
  cardiac failure are an emergency irrespective
  of their clinical state.

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Important clinical clues

• Persistent unexplained central cyanosis or
  desaturation even if mild initially.
• Desaturation or cyanosis that does not improve
  with oxygen or ventilation
• A significant persistent difference in upper and
  lower limb saturations

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Important clinical clues

• Signs suggestive of cardiac failure
• Unexplained respiratory distress
• Hepatomegaly
• Cardiomegaly
• Poor perfusion and metabolic acidosis
• Prominent or visible epigastric pulsations
• Weak or absent pulses in the lower limbs
• Persistent murmur

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• small team examining predischarge structured
  referral pathway 90 detection
• does not matter whether physician or registered
  nurse
• experienced team
• structured referral
• Arch Dis Child Fetal Neonatal 200691F263-7

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Successful Outcome depends on

• Obstetrics
• Neonatology
• Paediatric cardiology
• Paediatric Cardiac Surgeons
• Anaesthetists
• Intensive Care

• Doctor
• Nursing staff
• Technologist
• Perfusion Technologists
• Physiotherapists
• etc

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