Congenital Heart Disease in Congenital Heart Disease in NeonatesNeonates

EGM Hoosen

Paediatric Cardiology

Inkosi Albert Luthuli Central Hospital

How common is cardiac How common is cardiac disease in children?disease in children?

Congenital Heart Disease: 8/1000

3/1000 : cardiac disease needing intervention in the first year.

UK study 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

   

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

Antenatal diagnosisAntenatal diagnosis

– 20weeks gestation– detection rate

average: 23% range: 3 – 68%

– advantage– early detection

– delivery in high risk unit

Consequences of late/missed Consequences of late/missed diagnosisdiagnosis

Mortality

Ischemic brain injury

Multiorgan failure

Higher postoperative morbidity

Case 1Case 1

Day 7 term neonate

–severe cyanosis – Respiratory Distress

– Was discharged one day after a normal delivery – Became suddenly ill and rushed to hospital

Clinical findingsClinical findings

?Respiratory Disease– Clinical examination– CXR– Oxygen administration - – Blood gas: pH 7.18 PO2 :4kPa PCO2: 3.5kPa

BE :-16

ManagementManagement

Discusssed urgently – ?cyanotic congenital heart disease

Stabilised :– acidosis corrected– Temperature – Glucose– Commenced on prostaglandins– Iv fluids– Monitored for apneoa

Urgent referral Diagnosis:

Why cyanotic congenital heart Why cyanotic congenital heart disease is disease is oftenoften missed at birth missed at birth1. Cyanosis is not always apparent or always

treated seriously immediately after birth.2. Cyanosis, particularly peripheral cyanosis, is

common in newborns.3. Cyanosis that worsens on crying must be

investigated further.

4. Newborns with cyanotic congenital heart disease often look completely well initially-until the duct begins to close

Congenital heart disease Congenital heart disease presenting with cyanosis at or presenting with cyanosis at or

soon after birthsoon after birthPulmonary atresia/VSD (1:3500 live births)Transposition of Great vessels (1:3500)Pulmonary atresia /Intact ventricular septumCritical pulmonary stenosis

Prostaglandin administrationProstaglandin administration

Maintain a patent ductus arteriosusIntravenous infusion – Prostaglandin

E1(alprostadil)Oral prostaglandins: Prostaglandin E2 Side effects:

– Apneoa– Fever– Gastrointestinal etc

Management of pulmonary Management of pulmonary atresiaatresia

Careful assessment by cardiologist

Neonatal surgery – Blalock Taussig shunt

Transposition of great arteriesTransposition of great arteries

Case 2Case 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.

ManagementManagement

InotropesAntibiotics Prostaglandin administration Acidosis correctedGlucose 1.6mmols initially – correctedReferred for cardiac evaluation

DiagnosisDiagnosis

Congenital heart disease Congenital heart disease presenting with shock in the presenting with shock in the

neonateneonate Coarctation Interrupted aortic arch Critical aortic stenosis Hypoplastic left heart

syndrome

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.

Most common differential Most common differential diagnoses of critically ill diagnoses of critically ill

neonates with congenital heart neonates with congenital heart diseasedisease

Septic shockPersistent pulmonary Hypertension of the

NewbornRespiratory disease

Pulse oxymetryPulse oxymetry

Proper use of equipment

Saturations persistently less than 96%

Differential saturations

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

Important clinical cluesImportant 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

Important clinical cluesImportant 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

small team examining predischarge + structured referral pathway – 90% detection

does not matter whether physician or registered nurse

experienced team structured referralstructured referral

Arch Dis Child Fetal Neonatal 2006;91:F263-7

Successful Outcome depends Successful Outcome depends on: on:

Obstetrics Neonatology Paediatric cardiology Paediatric Cardiac

Surgeons Anaesthetists Intensive Care

Doctor Nursing staff Technologist Perfusion

Technologists Physiotherapists etc