1. Acyanotic Congenital Heart Disease
Rajesh Shenoy, M.D.
Section of Pediatric Cardiology
The Children’s Hospital at Montefiore p
Department of Pediatrics
Albert Einstein College of Medicine

2. Classification Lesions producing volume overload
L R shunt at atrial level: ASD, PAPVR
L R shunt at ventricular level: VSD
L R shunt at arterial level: PDA, A-P window
Lesions producing pressure overload
Subvalvar obstruction: HOCM
Valvar obstruction: AS, PS
Supravalvar obstruction: Supravalve AS/PS, PPS, Coarctation

3. ASD: Embryology
                                                              

4. ASD: Types Ostium secundum ASD Ostium primum ASD Sinus venosus ASD
Most common form
More common in females
Ostium primum ASD
May occur in isolation
In combination with AVSD, cleft mitral valve
Sinus venosus ASD
May be superior or inferior

5. ASD: Pathophysiology
Left-to-right shunt occurs because RA pressure < LA pressure
Gradual volume overload of RA/RV/PA
Eventually may lead to pulmonary hypertension

6. ASD: Symptoms Depend on size and nature of ASD
May be totally asymptomatic
4% of children go into CHF
Unrepaired ASDs might have decreased effort tolerance in the long run

7. ASD: Signs Anthropometry: No FTT in most cases
Inspection: Precordial bulge, RV heave
Auscultation:
Heart sounds
S1-N S2-fixed wide split
Murmurs:
II/VI ejection systolic murmur at LUSB, radiating to axillae
II/IV middiastolic rumble at LLSB

8. ASD: Investigations ECG: CXR: ECHO: Cath:
rSR’ pattern in right precordial leads
Upright T wave in right precordial leads
CXR:
Prominent pulmonary notch
Apex shifted up
Increased vascularity
ECHO:
Diagnostic
Cath:
Not necessary unless suspicion of pulmonary hypertension, or for therapeutic reasons

9. ASD: ECG
                                                                                                           

10. ASD: X-Ray Chest

11. ASD: Echocardiography

12. ASD: Prognosis
Very good prognosis even if repaired up to age of 65 years
Repairs > 4-5 years: slightly decreased effort tolerance
Secundum ASD smaller than 7 mm may close spontaneously upto 11 years
Primum ASDs almost never close
Risk of paradoxical embolism with small ASD

13. ASD: Therapeutic options
Surgery
Tried and tested
Morbidity and mortality close to 0%
Intervention
FDA approved
Newer and better devices

14. ASD: Long-term follow-up
Excellent long-term results
2-3% however, develop atrial arrhythmias

15.                        
VSD: Embryology

16. VSD: Classification (Anatomic)
                             
VSD: Classification (Anatomic)
                             
Inlet Outlet
Membranous Muscular

17. VSD: Classification (Physiologic)
Tiny
Small
Moderate
Large (>75% dimension of aortic valve)
Depends on symptoms, signs, echo appearance

18. VSD: Pathophysiology
RV pressure starts to fall after 72 hrs, nadir by 2 months
Depending on size of defect, left-to-right shunt
Initially LA/LV enlargement
Next, increased PA pressures
Finally, RV enlargement/hypertrophy

19. VSD: Symptoms Spectrum from asymptomatic to crippling CHF
Tachypnea, Feeding difficulty, FTT
Tachycardia, Sweating, Cold extremities
Sleep-suck-sleep cycle

20. VSD-Signs Depend on size of VSD and degree of CHF Anthropometry: FTT
Inspection:
Precordial bulge, Hyperdynamic precordium, LV apex
Auscultation:
Sounds:S1, S2, S3 gallop
Murmur: Pansystolic murmur, increasing intensity with smaller defects Middiastolic rumble at apex
Resp:
Tachypnea, Crackles at lung bases
Abdomen:
Hepatomegaly

21. VSD: Investigations ECG: CXR: ECHO: Cath: Initially LAE/LVH, Later BVH
Cardiomegaly with LV apex
Increased vascularity
Frequent pneumonia
ECHO:
Diagnostic
Monitor LV size
Cath:
Not essential unless pulmonary hypertension has ensued or for therapeutic reasons

22. VSD: ECG
                                                                                                            

23. VSD-X-Ray Chest

24. VSD: Echocardiography

25. VSD: Management CHF Management Surgery Interventional
Medications: Digoxin, Furosemide
Improving caloric intake: Calorie-dense formula, Moducal/Polycose/MCT oil
Continuous NG feeding
Surgery
Interventional

26. VSD: Long-term follow up
Perimembranous/Subaortic VSD: May cause aortic valve prolapse, and need yearly echo to monitor
VSDs do not require SBE prophylaxis
P/O VSDs need SBE prophylaxis for 6 months after surgery, or forever if small residuals persist

27. PDA: Embryology
                                                         

28. PDA: Pathophysiology Fetal life: Functional closure in 72 hours
Extremely important structure, as it shunts blood away from pulmonary arteries to descending aorta
Functional closure in 72 hours
Anatomic closure in 2 months
Strongest stimulus for closure:
PaO2>50

29. PDA: Symptoms Premature neonate: Older population:
Large PDA shunts blood into lung bed Increasing O2 requirement
Older population:
Silent PDA
Tiny/small PDA-Asymptomatic
Moderate/Large PDA-Behave like large shunts

30. PDA: Investigations ECG: LAE/LVH
CXR: Cardiomegaly / LV apex / Increased vascularity
ECHO: Diagnostic
Cath: Therapeutic

31. PDA: X-Ray Chest

32. PDA: Echocardiography

33. PDA: Treatment Medical: Surgical: Cath: Indomethacin
Lasix (controversial)
Management of CHF
Surgical:
Ligation
Division
Cath:
Coil embolization
Grifka bag
Occluders

34. Atrioventricular septal defects Embryology

35. AVSD-Anatomy

36. AVSD-Pathophysiology
Left-to-right shunt at atrial and ventricular level produces volume overload
AV valve insufficiency worsens process
Early CHF and Eisenmenger
Some R-L shunting may cause desaturations

37. AVSD-Signs & Symptoms Behave like large L-R shunts
Feeding difficulty, sweating on forehead, failure to gain weight
Tachycardia/Hyperdynamic precordium
Widely split S2
Murmur:
ESM at pulmonic area
MDM at mital area
PSM at mitral area in case of associated MR

38. AVSD-ECG

39. AVSD-CXR

40. AVSD-Echocardiogram

41. AVSD-Cardiac catheterization
Not frequently done these
days, unless the diagnosis
has been missed, and
pulmonary vascular
resistance needs to be
checked

42. AVSD-Mangement Management of CHF
Definitive surgical repair at around 4-6 months of life
Long-term issues:
Mitral insufficiency
Subaortic stenosis

43. Pulmonary valve stenosis
Deformation or incomplete separation of valve leaflets
Valve may be bicuspid or tricuspid
Occurring in isolation, or with VSD/ASD
Common cardiac lesion with Noonan syndrome

44. PS-Anatomy

45. PS-Pathophysiology Presentation depends on degree of stenosis
If the opening is tiny, R-L shunt at atrial level and ductal-dependent pulmonary circulation
With decreasing grade of stenosis, severe to almost no RV failure

46. PS-Signs/Symptoms
Critical PS-Pulmonary circulation is ductal-dependent. Neonate is cyanotic, and may be in extremis. + murmur
Severe PS-Loud heart murmur, RV failure
Moderate PS-Heart murmur, exercise intolerance
Mild PS-Heart murmur, asymptomatic
Relationship of heart murmur to drop in pulmonary vascular resistance

47. PS-Signs/Symtpoms S1 normal S2-Pulmonary component inaudible
Ejection systolic click
Ejection systolic murmur at the upper sternal border, radiating to the axillae and the back

48. PS-ECG

49. PS-CXR Normal cardiothoracic ratio Obliteration of ant. mediastinum
Prominent pulmonary notch

50. PS-Echocardiogram

51. PS-Cardiac Catheterization
Detects pressure gradients across valve
Mild: mm Hg
Moderate: mm Hg
Severe: >60 mm Hg
Catheterization is usually done only when balloon valvuloplasty is being contemplated for treatment

52. PS-Management
Critical PS: PGE1, till definitive procedure- cath. vs. surgical valvotomy
Severe/Moderate PS: Balloon
Mild PS: Follow-up (No SBE reqd.)
Decision to take child up for catheterization depends on morphology of the valve. Thickened & deformed valves are not amenable to cath. relief

53. Aortic stenosis ~ 5% of CHD (not including bicuspid)
Due to fusion of valve leaflets
Stenosis may be subvalvar (viz. subaortic membrane), or supravalvar (as with Williams syndrome)

54. AS-Anatomy

55. AS-Pathophysiology Manifestation depends on degree of obstruction
Critical AS causes cardiogenic shock
Severe AS might present with syncope on exertion
Mild AS might be asymptomatic

56. AS-Signs S1: normal S2: aortic component is soft
Ejection systolic click in aortic area with mild-moderate valvar AS
Ejection systolic murmur in aortic area, intensity depends on degree of obstruction

57. AS-ECG

58. AS-Echocardiogram

59. AS-Management
Critical AS: PGE1 to stabilize the child, followed by emergent surgical valvotomy vs. balloon valvuloplasty
Severe AS: Gradient>60 mm Hg-intervene
Moderate AS: Gradient>30 mm Hg-is AI present
Mild AS: < 30 mmHg-follow
Morphology of the valve, and presence of aortic insufficiency add to the decision-making

60. AS-Long-term follow up
Mild AS: F/U echocardiogram to check for gradient, watch for syncope
S/P procedure: AI, poorly tolerated
Ross procedure
Homograft replacement
Prosthetic valve
SBE prophylaxis

61. Coarctation of the aorta
Usually discrete opposite the PDA
Sometimes tubular narrowing of the arch (usually associated with AS, large VSD, or with Shone complex)
Common defect with Turner syndrome

62. CoA-Anatomy

63. CoA-Pathophysiology
?Constriction of ductal tissue in juxtaductal segment
With closure of PDA, obstruction to flow to the abdominal aorta
With critical/severe CoA, LV failure
With other types, development of collaterals HTN later on in life

64. CoA-Signs/Symtpoms Neonate:Left ventricular failure Non-critical CoA
Asymptomatic, Claudication
HTN, with arm-leg discrepancy of BP
Continuous murmur from collaterals
Murmurs of associated defects, viz. bicuspid aortic valve or VSD

65. CoA-ECG

66. CoA-CXR

67. CoA-Echocardiogram

68. CoA-Cardiac Catheterization

69. CoA-Management
Neonatal: Support with PGE1, followed by surgical relief of coarctation
Infancy: Controversial. Some opt for surgery, others for balloon angioplasty
2-7 years: Balloon angioplasty
> 7 years: Balloon angioplasty + stent placement

70. CoA-Follow up HTN: Recurrent coarctation:
May be transient in the post-surgical phase
Older children may have sustained HTN (? Permanent change in aortic elasticity)
Recurrent coarctation:
Upto 50% of neonatal repairs, addressed by balloon angioplasty