Congenital Heart Defects

1. Congenital Heart Defects Nilima Malaiya Consultant Paediatric Cardiologist Royal Manchester Children�s Hospital Central Manchester University Hospitals NHS Foundation Trust

2. Objectives The heart and normal anatomy Cardiac problems in children How they present, signs & symptoms, radiological findings Acyanotic & Cyanotic CHD Eisenmenger�s Syndrome Central vs peripheral cyanosis Cardiac Failure & Rx MCQs

3. What is a Heart? First functional organ to be formed in embryological life. Mesenchymal cells migrate from Splanchnic mesoderm to form a �cardiac tube� which forms 4 chambers by 40 days (looping & septation) Looping Septation A hollow organ made of specialised muscle A pump to circulate blood to all the tissues in the body

4. Anatomy of the Heart

5. Heart Problems in Children Congenital: Acyanotic Cyanotic Acquired: Myocarditis Rheumatic heart disease Inherited: Hypertrophic Cardiomyopathy (HOCM) Marfan�s syndrome, etc.

6. Congenital Heart Disease (CHD) Incidence : 8 per 1000 live births ( 0.8 % ) Third of these are Cyanotic HD Diagnosed : 30-40% within 2 Wks of age. 60% within 4 Wks of age.

7. Presentation ANTENATAL diagnosis: Routine Scan Family history 1st degree relative POSTNATAL: Routine check Symptomatic

8. Acyanotic CHD Septal defects: Ventricular (VSD) or Atrial (ASD) Aortic/Pulmonary stenosis Coarctation of Aorta Patent Ductus Arteriosus (PDA) Mitral/Tricuspid Stenosis

9. Acyanotic CHD cont.

10. Coarctation of Aorta

11. Cyanotic CHD Generalised Blueness (Central Cyanosis) Occurs in 30% of congenital heart disease Fallot�s Tetralogy Transposition of Great arteries (TGA) Complete Atrio-Ventricular Septal defects (CAVSD) Tricuspid Atresia,Aortic/Mitral Atresia Total Anomalous Pulmonary Venous Drainage (TAPVD) Pulmonary atresia / intact septum Double outlet right ventricle (DORV) Single Ventricle Anomalous systemic venous drainage

13. Atrio-Ventricular Septal Defect Partial Complete Higher incidence in trisomy 21 Common A-V valve Left axis - ECG

14. Eisenmenger�s Syndrome Reversal of left to right shunt secondary to development of irreversible pulmonary hypertension. Complication of untreated large left to right shunts. Eg: large VSD/ASD/PDA Patients develop cyanosis and clubbing. Loud 2nd heart sound (P2)

16. Peripheral Cyanosis

18. Cardiac Failure When heart struggles to maintain circulation �pump failure� Symptoms/Signs in children: Shortness of breath: ? rate (tachypnoea) ? difficulty (dyspnoea) Poor feeding: Wt. ? or ? ? Heart rate (Tachycardia) Hepatomegaly Poor pulses Acidosis Sweating

19. Management of Cardiac Failure Diuretics: Furosemide, Amiloride etc. ACE inhibhitors: Captopril Enalapril Oxygen Prostaglandins: Prostin Diet / Fluid intake Inotropes: Dopamine, Dobutamine Catheter intervention e.g. Balloon angioplasty etc. Surgery

20. Rx: Cardiac Failure cont. Higher energy requirements Higher calorie feeds Infratini SMA high energy milk Calorie Supplements Polycal Duocal Small, frequent feeds Nasogastric tube feeds Gastrostomy

24. Chest X ray - Lungs Plethoric L to R shunts: VSD, PDA Cardiac Failure Oligaemic Fallot�s Tetralogy Pulmonary Atresia / stenosis

27. Summary CHD: 2 forms (Cyanotic & Acyanotic) Rx is different for both types Important to recognise signs & symptoms Holistic approach Cardiac, pulmonary & dietary factors to consider

28. MCQs: Best of five

29. a. Presents with central cyanosis in infancy b. Ejection systolic murmur c. Pulmonary oligaemia on CXR d. Pan systolic murmur e. Is always associated with left to right shunt Q1. Which one of the following is most typical of VSD?

30. a. Presents with central cyanosis in infancy b. Ejection systolic murmur c. Pulmonary oligaemia on CXR d. Pan systolic murmur e. Is always associated with left to right shunt A1. Which one of the following is most typical of VSD?

31. Q2. A 6 month old in cardiac failure will not have which of the following? a. Poor weight gain b. Clubbing c. Breathlessness d. Poor feeding e. Hepatomegaly

32. A2. A 6 month old in cardiac failure will not have which of the following? a. Poor weight gain b. Clubbing c. Breathlessness d. Poor feeding e. Hepatomegaly

33. Q3. Which one of the following is false of Fallot�s tetralogy? a. Over-riding aorta b. Pulmonary Stenosis c. Aortic Stenosis d. Boot shaped heart on CXR e. VSD

34. A3. Which one of the following is false of Fallot�s tetraology? a. Over-riding aorta b. Pulmonary Stenosis c. Aortic Stenosis d. Boot shaped heart on CXR e. VSD

35. Q4. Which of the following is not true of coarctation of aorta. a. Poor femoral pulses on clinical examination b. Upper limb hypertension c. Can present in cardiac failure d. Associated with bicuspid aortic valve e. Diastolic murmur

36. A4. Which of the following is not true of coarctation of aorta. a. Poor femoral pulses on clinical examination b. Upper limb hypertension c. Can present in cardiac failure d. Associated with bicuspid aortic valve e. Diastolic murmur

37. Q5. Which of the following is not correct for patent ductus arteriosus. Prostaglandin E1 encourages closure of the PDA Associated with continuous murmur Can close spontaneously ECG can be normal Associated with bounding pulses

38. A5. Which of the following is not correct for patent ductus arteriosus. Prostaglandin E1 encourages closure of the PDA Associated with continuous murmur Can close spontaneously ECG can be normal Associated with bounding pulses

39. Thank You