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Cardiac - management of antenatally diagnosed major congenital heart disease at delivery and in NICU

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National Women's Health acts as the primary delivery unit nationally for infants with an antenatal diagnosis of major congenital heart disease who are likely to need surgical intervention in the newborn period. A fetal cardiology service is provided by the Starship Paediatric and Congenital Cardiac Service  and in most instances the anatomical and physiological lesion is able to be identified accurately prior to delivery.

The major cardiac lesions diagnosed antenatally can be generally divided into four groups:

  • Duct-dependent for systemic blood flow (e.g. Hypoplastic Left Heart Syndrome, critical aortic stenosis, interrupted aortic arch)
  • Duct-dependent cyanotic lesions (e.g. pulmonary atresia, transposition of the great arteries) 
  • Rhythm disturbances (e.g. congenital heart block, fetal supraventricular tachycardia) 
  • Non duct dependent lesions such as (Tetralogy of Fallot, VSD and AV canal defects 

Management in labour

  • A copy of the fetal echocardiogram report(s), and Fetal Medicine Panel report if applicable, should be obtained so accurate information is available. 
  • The obstetric service should notify the Level 3 neonatal registrar or NS-ANP (pager 93-5535) that delivery is anticipated. The registrar or NS-ANP should inform the neonatal unit clinical charge nurse and the neonatal specialist on duty or on call. 
  • The paediatric cardiologist on call should be informed during normal working hours if the mother is in labour or is going to be induced or electively delivered by caesarean section. 
  • The paediatric cardiologist has usually already met with the parents to explain what is planned post-delivery. 

Immediate Delivery Room management

  • Most infants with major congenital heart disease will not require additional resuscitation at birth and will be asymptomatic of their cardiac disease for hours or days postnatally. 
    An infant who is cyanosed and bradycardic at birth requires effective resuscitation, and the cause of the cyanosis and bradycardia should be assumed to be respiratory and not cardiac.  Resuscitation measures may include the administration of oxygen and positive pressure ventilation.
  • Cardiac lesions that are responsible for an infant being in poor condition at birth are rare (e.g. severe Ebstein's anomaly, or other cardiac conditions such as arrhythmia, particularly if accompanied by fetal hydrops).
  • Following resuscitation and assessment, the infant should be transferred to NICU as soon as practical. The parents should be given the opportunity to hold their baby if the baby's condition allows this.

Initial management in NICU

Initial management will depend on the underlying cardiac lesion and the anticipated neonatal problems.

  • Infants should be admitted to Level 3 NICU.
  • Cardiorespiratory and oxygen saturation monitoring should be commenced as soon as possible.
  • If the infant is unwell or requiring significant support, take blood cultures and commence antibiotics.
  • Intravenous access
    If the infant requires significant ventilatory support, arterial and venous access should be obtained.
    Infants with lesions dependent on the duct for systemic blood flow, a double lumen umbilical venous cather should be inserted.
    For infants with other lesions it is not necessary to insert umbilical catheters if the baby is otherwise well.
  • Ongoing Care in NICU

Duct-dependent for Systemic Blood Flow

With severe left-sided obstructive lesions systemic blood flow is dependent on right-to-left flow through a patent ductus arteriosus, so these babies are duct-dependent. Examples: Hypoplastic Left Heart Syndrome, critical aortic stenosis, coarctation of aorta, interrupted aortic arch.

  • Insert a double lumen umbilical venous catheter
  • Commence a prostaglandin infusion at an initial dose of 10 nanograms/kg/min.
  • Do not over-oxygenate the infant (over-oxygenation will result in increased pulmonary blood flow and reduced systemic blood flow).
  • Accept oxygen saturations of 75% or above. Reduce inspired oxygen if saturations >85%.
  • Contact the paediatric cardiologist on call between 12 midnight and 6.30 a.m.if saturations < 75%, otherwise inform on call paediatric cardiologist at 06.30 a.m.
  • The baby is to remain nil by mouth.
  • If the infant requires assisted ventilation, ensure that the baby is not over-ventilated. The aim should be to initially ventilate to keep a low-normal arterial pH. Sedation, muscle relaxation, and controlled hypoventilation to further reduce arterial pH may be necessary if there is excessive pulmonary blood flow and reduced systemic blood flow (oxygen saturations >85%, low MAP, tachycardia, cool peripheries).

Duct-dependent Cyanotic Lesions

These lesions are duct-dependent either to ensure adequate pulmonary blood flow (e.g. pulmonary atresia, critical pulmonary stenosis) or to ensure adequate mixing between the systemic and pulmonary circulations (transposition of the great arteries).

  • Commence a prostaglandin infusion at an initial dose of 10 nanograms/kg/min.
  • Babies with TGA should be nil by mouth until atrial septostomy has been performed. Babies who are duct dependent for pulmonary but not systemic blood flow (Pulmonary Atresia and Tricuspid Atresia) may receive EBM feeds if stable.
  • Ensure that at least one extra IV leur is available in the event that the PGE1 infusion tissues.
  • If the systemic oxygen saturation is below 75%, call the paediatric cardiologist on call.
  • If the infant develops apnoea or the systemic oxygen saturation is below 75% despite prostaglandin, they should be ventilated.
  • If the infant develops apnoea but has a systemic oxygen saturation of 75% or above, the dose of prostaglandin can be reduced (but not below 5 nanograms/kg/min). If apnoea continues, the infant should be ventilated.
  • If the infant is delivered after midnight but is stable, the paediatric cardiologist should be contacted in the morning by 0700 hours. If unstable, contact the paediatric cardiologist on call.
  • OBSTRUCTIVE LEFT HEART LESIONS: ie HLHS, Interrupted aortic arch, Co-arctation, Aortic or Mitral atresia - PICU within 24 hours after discussion with NICU/PICU/Cardiology consultants.

Rhythm Disturbances

Many infants are asymptomatic despite rhythm disturbances which have been detected antenatally or postnatally. Some infants may require significant resuscitation, particularly if they are hydropic. Hydropic infants require the attendance of a neonatal specialist. Severely hydropic infants may require emergency insertion of intercostal and/or abdominal drains at delivery.

Congenital Heart Block

  • In the case of complete heart block with fetal hydrops, delivery should be planned in consultation with the paediatric cardiologist and/or paediatric cardiac surgeon on call as urgent pacing may be necessary.
  • Transfer to NICU as quickly as possible.
  • Intravenous access should be obtained.
  • It is preferable but not essential to obtain a 12-lead ECG soon after admission to NICU.
  • If the heart rate is above 55bpm and the infant is stable, contact the paediatric cardiologist non-urgently.
  • If the heart rate is below 55bpm, contact the paediatric cardiologist on call.
  • Do not commence chronotropic agents (e.g. isoprenaline) without first discussing management with the paediatric cardiologist.

Tachyarrhythmias

  • Be aware that some pregnant mothers are treated with one or more anti-arrhythmic medications when the fetus has SVT, in order to treat the fetus, thus the baby may have anti-arrhythmic medication(s) on board at delivery.
  • If the tachycardia is still present after delivery, transfer to NICU as quickly as possible
  • Intravenous access should be obtained.
  • Obtain a 12-lead ECG soon after admission to NICU. During normal working hours contact the ECG technicians (pager 93 5367); after-hours, medical or nursing staff will need to perform the ECG.
  • Contact the paediatric cardiologist on call to discuss further management.
  • If the tachycardia has resolved by delivery and the infant is stable, the baby can be admitted to the postnatal ward under paediatric care. The baby should have Q4H observations initially. Arrange review by the neonatal specialist on call the following morning with a 12 lead ECG available.

Non Duct Dependent Lesions

This includes: Tetralogy of Fallot, VSD and others such as AV canal defect. Although these lesions are not duct dependent the baby should be closely observed until formal assessment is completed.

  • These infants do not require PGE1 infusion but peripheral IV access should be established.
  • There is no requirement for the baby to be nil by mouth.
  • If any special postnatal management is required then this will be specified in the antenatal notes or Fetal Medicine Panel letter.
  • If the infant is delivered after midnight but is stable, the paediatric cardiologist should be contacted in the morning at 06.30 hours. If unstable or cyanosed contact the paediatric cardiologist on call.

Note: Suspected Arch Hypoplasia or Ventricle Disproportion

For suspected arch hypoplasia / antenatal appearance of ventricle disproportion the postnatal management may vary based on individual assessment. Admission should initially be onto Level 3 for assessment. Antenatal documentation should include instructions from the paediatric cardiologists on the use of Prostin and the requirement to be nil by mouth. If the infant is well ECHO can be performed the following day.

Screening for congenital heart disease in first degree relatives

The baseline prevalence of congenital heart disease (excluding PDA in preterm infants, mitral valve prolapse, and biscuspid aortic valves) is approximately 0.5-0.8% of all live-births.

The recurrence risk for siblings of children with congenital cardiac malformations have been variably estimated in the region between 1% and 4%, provided there is no strong family history of that particular malformation. The risk is higher in first degree relative where the lesion is Hypoplastic Left Heart Syndrome (HLHS, 19.3%) and coarctation of the aorta (9.4%). 1 The most common finding in relatives was of a bicuspid aortic valve (BAV), but some individuals had significant left-sided obstructive lesions including HLHS and coarctation.

If a neonate is born who has a first degree relative (parent or sibling) who has been diagnosed with HLHS, coarctation, or BAV:

  • The baby requires a complete cardiovascular examination, ideally on the first day but also later in the first week. 
  • Infants with symptoms or signs of congenital heart disease should be assessed as indicated. 
  • Those infants with a normal examination should be referred to the Paediatric and Congenital Cardiac Service outpatient clinic at Starship Hospital for assessment (ext 23621), preferably within the first few weeks of life when sedation for an echocardiogram should not be necessary.

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Document Control

  • Date last published: 04 June 2013
  • Document type: Clinical Guideline
  • Services responsible: Neonatology
  • Owner: Newborn Services Clinical Practice Committee
  • Editor: Sarah Bellhouse
  • Review frequency: 2 years