1. Congenital Heart Disease
Initial evaluation and stabilization
Priscilla Joe, MD
Children’s Hospital and Research Center Oakland

2. Initial evaluation History
Physical exam with 4 extremity blood pressures
Pre-ductal and post-ductal oxygen saturations
Hyperoxia test
CXR
EKG
ECHO
Exam: general appearance, growth chart, vital signs including blood pressure, signs of respiratory distress, cyanosis
Cardiac exam: inspection and palpation; 1st heart LLSB, single, mitral and tricuspid valve closure; 2nd heart LUSB; split, aortic and pulmonary valve closure; 3rd and 4th heart apex
Murmurs: Gr I- barely heard; Gr II- soft, but easily audible; Gr III- loud, recognized immediately; Gr IV-loud and associated with thrill; Gr V- audible with edge of stethoscope, thrill; Gr VI- audible without stethoscope
Pulses: weak or bounding, symmetric

3. Indications for fetal echocardiography
Fetal risk factors associated with CHD:
Trisomies, Turner’s syndrome, abnormal karyotype
Congenital malformations: duodenal atresia, TEF, omphalocele, diaphragmatic hernia, renal dysgenesis, and hydrocephalus
Fetal arrhythmias
IUGR
Nonimmune hydrops

4. Maternal metabolic disorders or infection
Diabetes mellitus
PKU
Hyperthyroidism
Lupus, collagen vascular disease
Rubella, CMV, Coxsackie, HIV

5. Maternal risk factors associated with congenital heart disease
Cardiac teratogen exposure
Lithium
Amphetamines
Alcohol
Anticonvulsants: phenytoin, valproic acid, carbamazepine,and trimethadione
Isotretinoin

6. Lungs vs heart: Differential cyanosis and the hyperoxia test
PaO2 <50 and SpO2 <85% pre-ductal despite 100% FiO2
-PPHN
-left-heart abnormalities
Post-ductal saturation higher than pre-ductal saturation
-TGA
-TAPVR above diaphragm with PDA

8. Neonatal Heart Disease
Ductal dependent lesions
Congestive heart failure
Right heart obstructive lesions
Left heart obstructive lesions
Mixing lesions
Inadequate gas exchange

9. Normal heart

10. Pulmonary Hypertension

11. Pulmonary Hypertension
Preductal SpO2
Postductal SpO2 PA Ao

12. Transposition of great arteriesAo PA

13. Transposition
Preductal
SpO2
Postductal
SpO2 Ao PA

14. TAPVR
Preductal SpO2
Postductal SpO2

15. CXR Heart size Pulmonary blood flow Cardiac position
Ebsteins, critical PS

17. Ebstein’s anomaly

18. Cyanotic with decreased pulmonary blood flow

19. Right Sided Obstructive Lesions- Blue, but comfortable
Cyanosis
No respiratory distress
Normal pulses and perfusion
Single second heart sound (no closing sound from abnormal pulm valve)
Murmur
Moderate to marked hypoxemia
CXR: normal to large sized heart, decreased PBF

21. Tetrology of Fallot

22. Tetrology of Fallot

23. Tetrology of Fallot
Infundibular septum
angled anteriorly

25. Tricuspid Atresia

26. Tricuspid Atresia

27. Cyanotic with decreased pulmonary blood flow
Tetrology of Fallot
Ebsteins Anomaly
Tricuspid Atresia with PA or PS
Pulmonary atresia with intact septum
Critical pulmonic stenosis
PPHN

28. Management right sided obstructive lesions
PGE
Supplemental O2 is OK (may slightly improve pulmonary vasodilatation)
Surgical intervention

29. Left sided obstructive lesions
Acute shock

30. Left sided obstructive lesions
Grey or ashen color (may not be blue)
Tachypnea
Poor perfusion
Decreased pulses/differential pulses
Single second heart sound
Murmur + gallop
Hepatomegaly
ABG: metabolic acidosis
CXR: cardiomegaly with increased PBF

31. Left sided obstructive lesions
Coarctation of aorta, interrupted aortic arch
Hypoplastic left heart syndrome
Aortic stenosis
Mitral stenosis
Total anomalous pulmonary venous return, below diaphragm

32. Hypoplastic left heart syndrome

33. Aortic stenosis

34. Hypoplastic Left Heart Syndrome
PDA supplies:
body
lungs
head
coronaries

35. Coartation of aorta

36. HLHS Treatment Fetal diagnosis is vital to prevent end organ failure
PGE
Balance perfusion to body/coronaries/head vs lungs
Avoid oxygen, hyperventilation, pressors to limit PBF
Control ventilation; paralyze and hypoventilate
Blend in nitrogen to raise PVR and limit PBF
Surgical intervention

37. Cyanotic with increased pulmonary blood flow
Inadequate mixing
Normal pulmonary vessels should not be seen beyond the middle 1/3 of lung

38. Inadequate Mixing Lesions
Cyanosis, often profound
Mild tachypnea
Normal pulses
Single heart sound
Murmur
ABG: marked hypoxemia, + acidosis
CXR: cardiomegaly, normal or increased PBF

40. d - Transposition of the Great Vessels

41. Transposition of Great Arteries
Mixing at PFO
and PDA

42. Truncus arteriosus

44. Truncus arteriosus

45. Cyanotic with increased pulmonary blood flow
d-Transposition of the great vessels
Truncus arteriosus
Total anomalous pulmonary venous return, above diaphragm
Single ventricle
Endocardial cushion defect
Normal pulmonary vessels should not be seen beyond the middle 1/3 of lung

46. Treatment of mixing lesions: TGA
PGE
Avoid too much PBF, may worsen patient
Balloon septostomy
Supplemental O2 may be helpful
Surgical repair

47. Lesions with poor gas exchange

48. Lesions with poor gas exchange
Cyanosis
Marked tachypnea (difficult to differentiate from GBS pneumonia/MAS
Perfusion fair, pulses normal
Second heart sound may be single
May or may not have a murmur
CXR: normal heart size, pulmonary congestion

49. Total anomalous pulmonary venous return

50. Supracardiac TAPVR

51. Management TAPVR Ventilation with PEEP Diuretics
PGE may worsen patient
iNO will worsen patient
Surgical intervention

52. Initial stabilization
Airway management: use of neuromuscular blockade
Titrate Fi02 to keep Sp02 80%-85%.
Use of PGE1 (0.02 to 0.05 mcg/kg/min)

53. Prostaglandin E1
Failure to respond: diagnosis incorrect, older infant with unresponsive ductus, ductus absent, obstructed pulmonary venous return
Clinical deterioration after PGE1: obstructed blood flow out of pulmonary veins or left atrium; HLHS with restrictive FO, TGA with intact ventricular septum and restrictive FO, obstructed TAPVR, mitral atresia with restrictive FO)

54. PGE 1 - side effects
Common: Apnea, fever, leukocytosis, cutaneous flushing, and bradycardia.
Uncommon: seizures, hypoventilation, hypotension, tachycardia, cardiac arrest, sepsis, diarrhea, DIC, fever
Rare: urticaria, bronchospasm, hemorrhage*, hypoglycemia, and hypocalcemia
*inhibits platelet aggregation

55. Stabilization for transport
Reliable vascular access
Intubation if on PGE1
Oxygen delivery, Sp02
Monitor HR, tissue perfusion, blood pressure, and acid-base status
Calcium and glucose status

58. EKG : QRS axis Tricuspid atresia with PS or PA : superior
Critical PS or PA : 0 to 90 degree quadrant
TOF and TOF with PA: degree quadrant
Superior axis: tricuspid atresia, primum ASD, complete endocardial cushion defect.

59. Acyanotic with increased pulmonary blood flow
VSD
ASD
PDA
Endocardial cushion defect

60. Ventriculo septal defect

61. Cardiac malpositions and heterotaxy

62. Dextrocardia
Heterotaxy syndrome: abnormal cardiac and visceral situs, abnormal bronchial anatomy, associated malrotation
Right sided isomerism: two right lungs (tri-lobed lungs) and asplenia
Left sided isomerism: two left lungs (bi-lobed lungs) and polysplenia