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

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

4. 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

5. 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

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

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

8. 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

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

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

12. Urgent referral Diagnosis:

13. Why cyanotic congenital heart disease is often missed at birth 1. 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

14. 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

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

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

17. Transposition of great arteries

18. 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.

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

21. Diagnosis

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

23. 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.

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

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

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

27. 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

28. 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

29. small team examining predischarge + structured referral pathway – 90% detection does not matter whether physician or registered nurse experienced team structured referral structured referral Arch Dis Child Fetal Neonatal 2006;91:F263-7

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