Essential of paediatric cardiology

1. Essential of paediatric cardiology Robyn Smith Department of Physiotherapy UFS 2011

2. CHD affects 1% of children First 8-10 weeks of gestation critical in development of the heart

3. Foetal circulation • Not dependant on the lungs for respiration • Placenta is used for gaseous exchange • All of the blood flowing through the chambers of the heart, arteries and veins is rich in Oxygen • Pulmonary vessels are vasoconstricted

4. Change in circulation after birth • As the baby takes its first breath the lungs expand, causing the lung P to fall and air enter lungs. • Once the lungs are filled with air and the oxygen level in the child’s blood rises resulting muscle wall of the ductus arteriosus contracts no longer allowing blood to flow through it (10-15 hours after birth) • This forces pulmonary circulation into action • Now child has separate oxygenated and de-oxygenated blood and relies fully on the lungs for gaseous exchange

5. Classification of CHD

6. Acyanotic lesions

7. Conditions where there is a left to right shunting of blood

8. PatendDuctusArteriousus(PDA)

9. PatendDuctusArteriousus (PDA) • Ductusarteriousus open in the foetal circulation • Vascular connection between the main pulmonary trunk and the aorta • Closes soon after birth • If it stays open excessive blood shunts from the aorta ton the lungs • Resulting in pulmonary oedema • Common in premature infants

10. PatendDuctusArteriousus (PDA)

11. Clinical signs and symptoms of a PDA: • Poor feeding • Failure to thrive (below weight for and height for age) • Sweating with crying or play • Persistent tachypnoea or breathlessness (dyspnoea) • Easy tiring • Tachycardia • Frequent lung infections • A bluish or dusky skin tone • Developmental delay

12. Medical management PDA • Management Closing of PDA: • Medication (indomethacin) induces closure • Surgical closing via a thoracotomy

13. AtrialSeptal Defect (ASD)

14. AtrialSeptal Defect (ASD) • Opening or whole in the wall separating the atria • Free communication of blood between the two atria. • Seen in 10% of all congenital heart disease • Rarely presents with signs of congestive heart failure or other cardiovascular symptoms • The right atrium and ventricle may enlarge over time • Cyanosis does not occur unless pulmonary hypertension is present.

15. Clinical signs and symptoms of ASD • Most are asymptomatic • May have easy fatigability or mild growth failure.

16. AtrialSeptal Defect (ASD)

17. Medical management of ASD • Surgery of catheterization

18. VenticularSeptal Defect (VSD)

19. VenticularSeptal Defect (VSD) • Abnormal opening in the ventricular septum. • Allows free communication between the right and left ventricles • Oxygen rich blood in the left ventricle pumped into the right ventricle instead of to the body. • In a large VSD excessive blood is pumped to the lungs resulting in congestion and shortness of breath. • In return excessive amounts of blood are pumped back from the lungs to the left heart overburdening and enlarging it resulting in CHF

20. VenticularSeptal Defect (VSD)

21. Clinical signs and symptoms of VSD • Small VSD most children are asymptomatic • In the case of moderate to large VSD the child will be symptomatic. • dyspnoea • feeding difficulties • failure to thrive • recurrent respiratory infections • profuse sweating

22. Medical management of VSD • In case of a small VSD 50% will close spontaneously by age 2yrs • Large VSD’s are usually closed surgically

23. AtrioventricularSeptal Defect (AVSD)

24. AtrioventricularSeptal Defect (AVSD) • Result from incomplete fusion of : • tendocardial cushions which help to form the lower portion of the atrial septum, • the membranous portion of the ventricular septum and the • septalleaflets of the triscupid and mitral valves. • 4% of all CHD • Commonly associated with chromosomal disorders such as Down Syndrome

25. AtrioventricularSeptal Defect (AVSD)

26. Clinical signs and symptoms of an AVSD • The child may present with: • CHF in infancy • recurrent respiratory infections • failure to thrive • exercise intolerance • easy fatigability

27. Medical management of AVSD • Surgery is always required. • Prior to surgery congestive symptoms are treated. • Pulmonary banding maybe required in premature infants or infants < 5 kg. • Correction is done during infancy to avoid irreversible pulmonary vascular disease.

28. What is pulmonary banding? • Primary aim procedure is to reduce excessive pulmonary blood flow • In order to protect the pulmonary vasculature from hypertrophy and • irreversible (fixed) pulmonary hypertension.

29. Other less common conditions

30. Surgical complications: Pulmonary hypertension • Can be a severe complication post operatively • Children at risk of are those with excessive shunting of blood from left to right e.g. VSD, AVSD • This results in excessive blood flow to the lungs resulting in distension and damage to the pulmonary artery wall which becomes muscularised • Pulmonary artery is then unable to dilate and vulnerable to reactive vasoconstriction

31. May predispose the child to a hypertensive crisis. The following may predispose a child to a hypertensive crisis: • Hypoxaemia • Hypercapnea • Metabolic acidosis as well as • relentless handling (including by the physiotherapist) • Tracheal suctioning may predispose the child to a hypertensive crisis.

32. Surgical complications: Pulmonary hypertension • In the case of a pulmonary hypertensive crisis the pulmonary arteries constrict resulting in an increase in pulmonary artery pressure and CVP. • The systemic blood pressure drops suddenly resulting in cardiac arrest. • Treatment includes sedation, paralysis and the administration of Nitric Oxide and 100% oxygen to try and facilitate pulmonary vasodilatation

34. Conditions where there is a obstructive cause

35. Coarctation of the aorta

36. Coarctation of Aorta • Congenital narrowing of the aorta as it leaves the heart. • Resulting in: • increased pressures in the arteries nearest the heart, head and arms • decreased circulation in lower extremities. • 7 % of all CHD • Male: Female ratio 3:1

37. Coarctation of Aorta • This is often not evident in the newborn until the ductusarterious closes • The blood in the left ventricle has then to be pumped out against the constriction. • Resulting in : • left ventricular hypertrophy • left ventricular failure with congestive heart failure (CHF)

38. Clinical signs and symptoms of coarctation • The child may present with: • CHF • “Hard” breathing” • Wheezing • Excessive sweating

39. Coarctation of Aorta

40. Medical management of a Corarctation of Aorta • With severe coarctation maintaining the ductus with prostaglandin E is essential • Early surgical repair and resection of the stenosis is imperative • Simple coarctation repair have a extremely low mortality but in complex cases mortality might be higher • A rare complication of surgical repair is paraplegia (longer cross clamping times during surgery) • In 18% of children undergoing surgery re-coarctationoccurs requiring further surgical intervention

41. Obstructive causes Asymptomatic in mild cases, More severe cases fatigue, syncope and dyspnoea Treatment is surgical repair Symptoms include dyspnoea, exercise intolerance, fatigue, CHF and hypoxaemia Treatment is surgical repair Aortic Stenosis Pulmonary Stenosis

42. Obstructive causes Aortic Stenosis Pulmonary Stenosis

43. Cyanotic heart lesions

45. Tetrallogy of Fallot (TOF)

46. Tetrallogy of Fallot (TOF) • Most common cyanotic heart lesion • Has 4 components: • A high VSD • Pulmonary stenosis • Anomalous position aorta • RV hypertrophy • Results in a right to left shunting of blood with low oxygen levels in the arteries and in the body tissues • Resulting in cyanosis, easy fatigability, fainting and shock. • Clubbing may be observed

47. Tetrallogy of Fallot (TOF)

49. Medical management of TOF • Early surgical intervention (TOF repair) is usually required • Palliative care by means of anestomosis and pulmonary valvotomy can be done

50. Hypoplastic Left Heart Syndrome