Congenital Heart Disease in Neonates

1. Congenital Heart Disease in Neonates EGM Hoosen Paediatric Cardiology Inkosi Albert Luthuli Central Hospital

2. How common is cardiac disease in children? • Congenital Heart Disease: 8/1000 • 3/1000 : cardiac disease needing intervention in the first year.

3. 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

4. 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

5. Antenatal diagnosis • 20weeks gestation • detection rate • average: 23% • range: 3 – 68% • advantage • early detection • delivery in high risk unit

6. Consequences of late/missed diagnosis • Mortality • Ischemic brain injury • Multiorgan failure • Higher postoperative morbidity

7. 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

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

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

11. Urgent referral • Diagnosis:

12. 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

13. Congenital heart disease presenting with cyanosis at or soon after birth • Pulmonary atresia/VSD (1:3500 live births) • Transposition of Great vessels (1:3500) • Pulmonary atresia /Intact ventricular septum • Critical pulmonary stenosis

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

15. Management of pulmonary atresia • Careful assessment by cardiologist • Neonatal surgery – Blalock Taussig shunt

16. Transposition of great arteries

17. 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.

19. Management • Inotropes • Antibiotics • Prostaglandin administration • Acidosis corrected • Glucose 1.6mmols initially – corrected • Referred for cardiac evaluation

20. Diagnosis

21. Congenital heart disease presenting with shock in the neonate • Coarctation • Interrupted aortic arch • Critical aortic stenosis • Hypoplastic left heart syndrome

22. 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.

23. Most common differential diagnoses of critically ill neonates with congenital heart disease • Septic shock • Persistent pulmonary Hypertension of the Newborn • Respiratory disease

24. Pulse oxymetry • Proper use of equipment • Saturations persistently less than 96% • Differential saturations

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

26. 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

27. 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

28. 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 2006;91:F263-7

29. Obstetrics Neonatology Paediatric cardiology Paediatric Cardiac Surgeons Anaesthetists Intensive Care Doctor Nursing staff Technologist Perfusion Technologists Physiotherapists etc Successful Outcome depends on: