Antenatally Diagnosed Major Congenital Heart Disease
Management at Delivery and in NICU

 

Approved by Clinical Practice Committee
June 2013
Clinical Guidelines Back Newborn Services Home Page
Management in Labour Immediate Delivery Room Management Immediate Management in NICU
Duct-dependent for Systemic Blood Flow Duct-dependent Cyanotic Lesions Rhythm Disturbances
Non Duct-Dependent Lesions   Suspected Arch Hypoplasia or Ventricle Disproportion

See also the guideline on screening First Degree Relatives
See also the S.T.A.B.L.E. Cardiac module
See also PreOp Cardiac Care

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:

Management in Labour

Immediate Delivery Room Management

Initial Management in NICU

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

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.

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).

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

Tachyarrhythmias

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.

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.