Sanjay Gandhi Postgraduate Institute of Medical Sciences

1. Sanjay Gandhi Postgraduate Institute of Medical Sciences Lucknow

2. Department of Cardiovascular & Thoracic SurgeryandTelemedicine network at SGPGIMS Welcomes participants in this presentation

3. "Birth defect of Heart, its Presentation and Treatment" Nirmal Gupta Head Department of Cardiovascular and Thoracic Surgery SGPGIMS, Lucknow. U.P.

4. Magnitude: Birth defects of Heart in India • Every year 2 lakh children are born with congenital heart defects • At least 60,000 of these need treatment in the 1st year of life • Only 5000 get treatment because of lack of awareness amongst public in general and GP’s: delayed diagnosis • Poor socio-economic status of families: delayed treatment

5. Current facilities • Requires highly trained and dedicated team of diagnostic facilities, Surgeons and Nurses • Poor availability of facilities even in best hospitals • Not a financially viable option for private setups • Lack of trained manpower in the country (only 5 dedicated units other than SGPGIMS)

6. SYMPTOMS "Birth defects of Heart, its Presentation and Treatment"

7. Neonatal History • Cyanosis • Failure to thrive • Exercise intolerance • Shortness of breath • Syncope • Palpitation • Chest pain

8. NEONATAL HISTORY • Cyanosis, shortness of breath. • Did the child need to stay in the hospital after maternal discharge?

9. Neonatal history • Cyanosis • Failure to thrive • Exercise intolerance • Shortness of breath • Syncope • Palpitation • Chest pain

10. CYANOSIS • > 5g/dl of deoxygenated Hb • False positive........... polycythemia • False negative...........anemia • Pathophysiology leading to cyanosis: • Obstruction of systemic venous blood flow to the lungs • Shunting of deoxygenated blood to left heart • Desaturation of systemic arterial blood

11. Neonatal history • Cyanosis • Failure to thrive • Exercise intolerance • Shortness of breath • Syncope • Palpitation • Chest pain

12. FAILURE TO THRIVE • Poor cardiac output and increased myocardial energy consumption coupled with poor feeding due to S.O.B.

13. Neonatal history • Cyanosis • Failure to thrive • Shortness of breath • Exercise intolerance • Syncope • Palpitation • Chest pain

14. EXERCISE INTOLERANCE • Baby................ poor ability to suck and feed • Child.................sedentary • Pathophysiology leading to exercise intolerance: • Poor cardiac output. • Increased energy consumption by an overworked heart.

15. Neonatal history • Cyanosis • Failure to thrive • Exercise intolerance • Shortness of breath • Syncope • Palpitation • Chest pain

16. SHORTNESS OF BREATH • Some children may be short of breath without appearing in distress "Happily tachypnoec" • Pathophysiology of S.O.B.: • Increase pulmonary blood flow • Interstitial edema • Decreased oxygen diffusion • Hypoxemia

17. Neonatal history • Cyanosis • Failure to thrive • Shortness of breath • Exercise intolerance • Syncope • Palpitation • Chest pain

18. SYNCOPE • Pathophysiology: • Inability to increase cardiac output suddenly due to restricted left ventricular outflow, e.g. severe aortic stenosis, IHSS. • Abnormal vasomotor tone resulting in vasodilatation when vasoconstriction is needed to maintain adequate blood pressure.

19. Neonatal history • Cyanosis • Failure to thrive • Exercise intolerance • Shortness of breath • Syncope • Palpitation • Chest pain

20. PALPITATION • Pathophysiology: • Irregular rhythm • Tachycardia • Awareness of normal rate and rhythm.

21. Neonatal history • Cyanosis • Failure to thrive • Exercise intolerance • Shortness of breath • Syncope • Palpitation • Chest pain

22. Rarely cardiac in origin. Look for extra cardiac causes: Skin, Musculoskeletal, Costochondral joints, Pleural membranes, Pericardium, Referred pain CHEST PAIN

23. SIGNS "Birth defects of Heart, its Presentation and Treatment"

24. SIGNS • Inspection • Palpation • Auscultation

25. INSPECTION • Does the child appear ill? • Decreased tissue oxygenation due to poor cardiac output or severe cyanosis • Respiratory distress due to pulmonary edema or hypoxemia. • Cyanosis • Edema • Distended neck veins due to increased right heart pressure leading to systemic venous congestion • Clubbing of digits • Chronic peripheral tissue hypoxemia

26. SIGNS • Inspection • Palpation • Auscultation

27. PALPATION • Peripheral perfusion, normal 1-2 seconds. • Reflection of cardiac output. • FA=BA, • Normal = full • Diastolic runoff = bounding • Poor stroke volume = thready

28. PALPATION ( Contd.) • Precordium • Increased cardiac output, ventricular hypertrophy = hyperactive • Highly turbulent blood flow = thrill • Indicators of ventricular hypertrophy or atrophy = RV, LV impulses • Aortic stenosis, turbulent blood flow in ascending aorta = Suprasternal notch: thrill? • Hepatomegaly, check below right and left costal margins.

29. SIGNS • Inspection • Palpation • Auscultation

30. AUSCULTATION • LUNGS • Pulmonary edema = rales, crackles • HEART • First heart sound (S1): • Closure of atrio-ventricular valves. • Second heart sound (S2): • A2: closure of aortic valve • P2: closure of pulmonary valve • Single S2 = absent pulmonary or aortic component or delayed closure of A2 superimposing P2 • inaudible P2 in TGA • Does the splitting of S2 vary with respiration? • Added sounds: • Gallop rhythm: S3, S4

31. AUSCULTATION (Contd.) • Murmurs • Grade: 1-6, one being the softest and six being the loudest. • By definition grade four murmur is associated with a palpable thrill. • Systolic murmur: • Holosystolic: Shunting of blood between two structures , the pressure in one structure is higher than the other throughout systole • Harsh: VSD • Soft: Atrio-ventricular valve regurgitation  • Ejection: Increase in blood flow turbulence as systole progresses due to an increasing amount of blood flow through a restricted orifice • Aortic stenosis • Pulmonary stenosis • Small VSD

32. AUSCULTATION (Contd.) • Mid-systolic: Increase volume of blood flowing through normal valves • ASD • Anemia • Diastolic murmur: Early: • Regurgitant blood flow from aorta or pulmonary artery into the ventricles • Aortic insufficiency • Pulmonary insufficiency Late: • Austin Flint murmur • Aortic regurgitation blood flow causes vibration of left ventricular free wall •  Systolic and diastolic murmur: Pressure difference between two structures during systole and diastole. • PDA & Shunts and collaterals

33. Congenital Heart Diseases and their Treatment "Birth defects of Heart, its presentation and treatment"

34. Normal heart and its ECHO

35. Atrial Septal Defect: ASD

36. Coarctation of aorta

37. Ventricular Septal Defect: VSD

38. Patent Ductus Arteriosus: PDA

39. Pulmonary Stenosis: PS

40. Aortic Stenosis: AS

41. Atrio-Ventricular Canal:A-V Canal Defect

42. Tetralogy of Fallot: TOF

43. Transposition of Great Arteries: TGA

44. Transposition of Great Arteries: D-TGA

45. Truncus arteriosus: Truncus

46. Tricuspid Atresia: TA

47. Pulmonary Atresia: PA

48. Total Anomalous Pulmonary Venous Drainage: TAPVD

49. Hypoplastic Left Heart Syndrome: HLHS

50. Ebstein’s Malformation: Ebstein’s