1. Sanjay Gandhi Postgraduate Institute of Medical Sciences
Lucknow

2. Welcomes participants in this presentation
Department of Cardiovascular & Thoracic Surgery and Telemedicine 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. "Birth defects of Heart, its Presentation and Treatment"
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. CHEST PAIN Rarely cardiac in origin. Look for extra cardiac causes:
Skin,
Musculoskeletal,
Costochondral joints,
Pleural membranes,
Pericardium,
Referred pain

23. "Birth defects of Heart, its Presentation and Treatment"
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: Diastolic murmur:
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

51. General guidelines: At birth Blueness at birth or immediately after
Murmur of the heart
Rapid breathing
Low blood pressure

52. General guidelines: At 2 – 6 months
Difficulty in feeding- baby is unable to suck properly, sweats or starts rapid breathing while feeding
Blue nails and toes and fainting spells
Inadequate weight gain
Recurrent chest infections

53. General guidelines: In first 3 years of life Fainting spells
Abnormal heart beats
Child avoids rigorous activities
Unable to play with his mates

54. General guidelines for couples
Drugs to avoid during pregnancy
Strict “NO” to Isoretinoin, Thalidomide, Estrogens, Oral contraceptives, ACE inhibitors, Chloramphenicol, Chlorpropamide, Erythromycin, Tetracycline and Haloperidol.
Anti-cancer drugs and Phenytoin are harmful but benefits outweigh the side effects.
Epinephrine, Ephedrine, B-blockers and Promethazine do not pose any significant risk, though the research is inadequate

55. In the end…
“There are a million times more patients in India with congenital heart diseases than polio, but the government’s budget for the treatment of congenital heart diseases is miniscule in comparison”

56. ….so the gap is of more than billions magnitude

57. Thanks for your attention friends
Can I have your questions, please.