1. CARDIOVASCULAR CONFERENCE: Approach to a patient with cyanotic heart disease

2. General Data: Name: Baby Boy G Neonate
born of a 22 year old primigravida

3. History of the Present Illness
Initial prenatal check-up
6th month of pregnancy at local health center
CBC, urinalysis normal
UTZ (9/6/10): right ventricle appears collapsed
Single live intrauterine pregnancy, cephalic, good cardiac and somatic activity, weeks AOG, rule out hypoplastic right ventricle.
Suggests congenital anomal scan scan with detailed cardiac evaulation preferably using fetal echocardiogram
Referred to USTH

4. September 8, 2010 UTZ: 2nd and 3rd trimester
Single live intrauterine pregnancy of about weeks in breech presentation with good cardiac and somatic activity
Suggest fetal 2D echo c/o Dr. Cuaso

5. September 8, 2010
Assessment: Pregnancy weeks AOG based on 2nd trimester ultrasound, t/c hypoplastic right ventricle
Advised:
Multivitamins + FESO4 1 cap OD
Milk formula 1 glass OD
Request for CBC with blood typing, urinalysis, 50g OGCT
Request for congenital scan
Attend mother’s class every Saturday am

6. September 13, 2010 Macroscopic Exam Result Microscopic Exam Color
Dark yellow
WBC
24-26/hpf
Transparency
Slightly turbid
RBC
6-8/hpf
Reaction
Acidic
Mucus threads
Moderate
Specific gravity
1.020
Epithelial cells pH
6.0
Amorphous urates
Many
Sugar
Negative
Bacteria
Protein
negative
Cast, parasites

7. September 13, 2010 Test Result Hemoglobin 129 g/L Hematocit 0.38
RBC count
4.07 x 10/L
WBC count
10.74 x10/L
Segmenters
0.68
Lymphocytes
0.30
Eosinophils
0.02
platelets
adequate

8. September 16, 2010
OB GYN OPD
Speculum exam: cervix violaceous, smooth with moderate frothy yellowish creamy discharge
Assessment: Trichomoniasis
Advised: Metronidazole 500 mg/tab 1 tab BID
Fetal 2D Echo once with funds
50g OGCT, repeat urinalysis clean catch

9. September 24, 2010 Follow-up Unremarkable
Still for fetal 2D Echo, 50g OGCT

10. October 5, 2010 (+) terminal dysuria Urinalysis Normal OGCT results
Acidic
(++) bacteria
2-5/hpf pus cells
Normal OGCT results
Advised:
Amoxicillin 500 mg/tab 1 tab q8 for 7 days
Once with 2D Echo results, refer to pediatric surgery
(+) hyperemic conjunctiva OD- referred to Ophtha

11. October 15, 2010 USTH (October 11, 2010)
Fetal 2D- Echocardiogram: hypoplastic Left Ventricle, hypoplastic Mitral Valve, and a patent foramen ovale
FHT 142
Assessment: Pregnancy weeks, hypoplastic left heart
Advised:
Refer to pediatrics-cardiology and pediatric surgery

12. November 22, 2010 (+) persistence of dysuria
Assessment: Pregnancy weeks AOG, cephalic, Hypoplastic left ventricle, t/c UTI
Advised
Urinalysis, Hepatitis B Ag, Blood typing

13. November 25, 2010 Assessment: UTI Advised:
Amoxicillin 500mg/cap 1 cap q8 for 7 days
Increase oral fluid intake

14. November 25, 2010 Pediatric Surgery Consult
Assessment: Pregnancy 36 weeks AOG, (?) hypoplastic left ventricle
Plans: will evaluate any time after delivery

15. November 26, 2010
Blood type: AB+

16. December 10, 2010 UTZ: 2nd and 3rd trimester
There seems to be a mass in the interventricular septum
Single live intrauterine pregnancy of about weeks in cephalic presentation
BPS 8/8; SEFW 2823 grams
Cardiomegaly
Suggest referral to Dr. Cuaso

17. December 10, 2010 High Risk OB GYN clinic
Assessment: Hypoplastic left ventricle, hypoplastic mitral valve, UTI, r/o IUGR
Advised: Terraferon, Clusivol OB, Cefuroxime 500 mg/tab BID for 7 days
Repeat urinalysis after 7 days
BPS

18. December 17, 2010 UTZ: 38 weeks 6 days AOG (-) dysuria
(+) fetal movements, irregular hypogastric pains, SEFW p10-50
IE: 1 cm dilated, 60% effaced, (+) BOW, cephalic, Stn -3
Assessment: Pregnancy weeks, cephalic, not in labor, ? Mass at the interventricular septum, UTI s/p treatment

19. December 12, 2010 UTZ: 2nd and 3rd trimester Findings:
There seems to be a mass at the interventricular septum
Single live intrauterine pregnancy of about weeks in cephalic presentation
BPS 8/8; SEFW 2823 grams
Cardiomegaly
Suggest referral to Dr. Cuaso

20. December 20, 2010 For follow up Supposedly for repeat Fetal 2D Echo
3 cm dilated, 70% effaced intact BOW, there was progression of labor alongside with spontaneous rupture of BOW.
Clear, non-foul smelling amniotic fluid

21. Maternal History (-) exposure to radiation
(-) symptoms of viral exanthems
(-) use of illicit drugs and abortifacients
Non-smoker
Non drinker of alcoholic beverages
(-) hypertension, allergy, thyroid disease, diabetes, asthma, liver disease, or blood dyscrasia
Hep B screening non-reactive
OGCT normal

22. Family History Name Age Relation Educational Attainment Occupation
Health
MPG 22
Mother
2nd year nursing student
Student
Healthy LG 23
Father
High school graduate
Unemployed

23. Family History
No diabetes, hypertension, cardiac diseases, cancer, tuberculosis, allergies
Denies hereditary illnesses

24. Physical Examination General Data
live, term, singleton, male, delivered via normal spontaneous delivery
BW 2.75 kg, BL 48 cm
AS 6 and 7 at 5 minutes, MT weeks
AGA

25. Physical Examination on Admission
HR 134 bpm, RR 58 cpm, T 37.2˚C
Blue, pale, (+) circumoral cyanosis
(-) Rash, (-) birth marks, (+) palmar and plantar cyanosis
(+) Molding, (+) caput succedaneum (-) cephalhematoma
(+) ROR OU, (-) eye discharge, normal set ears, (-) preauricular pits, patent nares, (-) Epstein’s pearls

26. Physical Examination on Admission
(-) Palpable neck masses, intact clavicle, no crepitations
(-) Chest deformities, symmetrical chest expansion, (-) retractions, clear and equal breath sounds, good respiratory effort
Adynamic precordium, regular heart rate and rhythm, grade 1 holosystolic murmur at left parasternal area
Globular abdomen, (+) umbilical stump with 2 arteries and 1 vein, (-) organomegaly, (-) palpable masses

27. Grossly male, bilaterally descended testes, good rugae, patent anus
Femoral pulses full and equal, good flexion of extremities, (-) Barlow, (-) Ortolani
Straight spine, (-) sacral dimpling, (-) tuft of hair
(+) Moro, grasp, rooting, plantar, and sucking reflexes

28. APPROACH TO DIAGNOSIS OF A PATIENT PRESENTING WITH CYANOSIS AT BIRTH

29. Indicators that heart disease may exist
Cyanosis
Cardiomegaly (Radiologic or Pericardial bulge)
Pathologic heart murmur
Tachypnea or overt respiratory distress (dyspnea)
Sweating especially during feeding
Increased or decreased pulses
Failure to thrive

30. Classification of Congenital Heart Diseases
A) Acyanotic B) Cyanotic

31. Major Considerations Is there a shunt (LR or RL)
Is there obstruction to inflow or outflow
Abnormal heart valves
Abnormal connections of great vessels
Combination

32. Subgroups of Acyanotic Diseases
Shunt anomalies
Valvular defects
Obstructive lesions
Inflow anomalies
Primary myocardial diseases

33. Shunt Anomalies L  R shunt Increased pulmonary blood flow
Increased pulmonary vascular arterial markings on chest Xray
ASD, VSD, PDA

34. Obstructive Lesion Discrepancy in amplitude of the peripheral pulses
Coarctation of the Aorta

35. Inflow Anomalies Increased pulmonary venous markings on chest Xray
No murmur
Cor Triatriatum, Pulmonary vein stenosis

36. Valvular Defects Stenosis or regurgitant Characteristic murmur
AS, AR, PS, PR, MS, MR, TS, TR

37. Primary Myocardial Diseases
No murmur
Disparity between cardiac size and pulmonary vascular markings
Glycogen storage disease
Cardiomyopathy

38. Hemodynamic Consequences
A) Volume (Diastolic) overload B) Pressure (Systolic) overload

39. ASD
Hemodynamic Consequence
Diastolic overload of RV

40. VSD MODERATE SIZE LARGE SIZE Volume overload of LV
Hemodynamic Consequence
MODERATE SIZE
Volume overload of LV
LARGE SIZE
Pressure overload of RV

41. Cyanotic Heart Disease
Cyanotic heart disease exist when one defect or association of defects allow the mixture of saturated and de-saturated blood to reach the systemic circulation

42. Do you suspect that patient is Cyanotic?
When in doubt
Clubbing
CBC
Hyperoxia test

43. Hyperoxia Test
Hyperoxia test is considered positive for intracardiac shunting if PO2 < 150 mmHg (torr) after 10 minutes of 100% fiO2

44. PVA / IVS
Hemodynamic Consequence
Pressure overload of RV

45. PVA / VSD
Hemodynamic Consequence
Pressure overload of RV

46. PDA Dependent Pulmonary Circulation
Pulmonary valve atresia (PVA) with intact interventricular septum
Other lesions with accompanying PVA

47. Approach to diagnosis
A) Chest Xray
Increased or decreased pulmonary vascular arterial markings
B) EKG
RVH, LVH, CVH
C) Character of second heart sound
S2 single, loud
S2 single, normal
Split S2

48. Chest x-ray

49. Causes of Cyanosis Noncardiac Cardiac
Pulmonary disorders (structural abnormalities of the lung, ventilation-perfusion mismatching, congenital or acquired airway obstruction, pneumothorax, hypoventilation)
Abnormal forms of hemoglobin (methemoglobin)
Poor peripheral perfusion (sepsis, hypoglycemia, dehydration, hypoadrenalism)
primary or persistent pulmonary hypertension
Increased pulmonary vascularity
D-TGA
TAPVR without obstruction
PTA
Single ventricle
DORV w/o PS
PPHN
Decreased pulmonary vascularity
TOF
Ebstein’s anomaly PS PA
TA with PS
DORV with PS

50. Pulmonary Vascular Markings Decreased: Cyanotic
TOF
Tricuspid Atresia
Complex heart with PS
PVA / IVS

51. Second Heart Sound (S2) Single Loud Single Normal Split S2 TGA TOF
TAPVR without obstruction
Aortic / Mitral atresia
Tricuspid atresia
Truncus Arteriosus
PVA

52. Cardiac Work-Up EKG Chest Xray 2D echocardiography
(TTE, TEE, ICE, IVUS)
Cardiac catheterization
CT angiography, cardiac MRI

53. PLACE THE:
ECG
2-D ECHO

54. Modalities of Management
Pharmacologic
Catheter based therapy
Surgical

55. Pharmacologic digoxin, diuretics, inotropes (pressor), vasodilators
Prostaglandin

56. Catheter Based Therapy (DI KO PA ALAM ITO, EXAMPLES LANG TO)
Balloon atrio septostomy (Rashkind)
Balloon valvuloplasty
Balloon angioplasty
Delivery of occlusion devices
Radio frequency ablation

57. Surgical (DI KO PA ALAM ITO, EXAMPLES LANG TO)
Shunts like Modified Blalock-Taussig
PA band
Complete repair
Glenn, Fontan
Norwood
Jatene, Mustard, Senning

58. Course in the Wards 1:31 AM (12/21/10)
May feed 10-15mL FBM q3 with strict aspiration precautions
Keep O2 sat >62%
Refer to pedia cardio
Prewarmed radiant warmer
Labs: CBC with PC, CXR, 2D echo, 15L ECG
Routine newborn care
Erythromycin strip 1cm OU
Vit K 1mg/IM
Hepa B vaccine 0.5mg/IM at lateral thigh
Cord care with 70% ethanol

60. Course in the Wards 7:30 AM (12/21/10) 1:00PM (12/21/10)
Opted to withhold any further aggressive treatment
1:00PM (12/21/10)
Referral to pedia cardio answered
7:00 AM (12/22/10)
Feeding: 20-30mL FBM q3
9:00 AM (12/23/10)
Decision to take home baby

61. Course in the Wards 12:00 NN (12/23/10) Discharge instructions
Daily cord care with 70% ETOH q6
Daily bath with mild soap and lukewarm water
Daily sun exposure 7 to 9 AM for 15 min
Exclusive breastfeeding q2-q8 15 to 30min for each breast
Discharge medications
Multivitamins 0.5mL/day
Follow up at Pedia High Risk and cardio clinic
For hearing screening as out patient

62. 15L ECG
Normal axis
Sinus tachycardia
LVH

63. 2D echo PDA Pulmonary valve atresia Intact ventricular septum
Hypertrophied right ventricle
Probably tripartite chamber
R->L shunt across formen ovale
Pulmonic annulus 5.6cm, MPA 5.22mm, RPA 5.0mm, LPA 6.0mm
Normal aortic arch, coronary arteries, pulmonary veins

64. CXR Lung fields are clear Prominent cardiac silhouette
Suspicious prominence of pulmonary vascularity
Normal hemidiagphragms and sinuses
Unremarkable visualized osseous structures

65. Lab results Result Hemoglobin 171 g/L Neutrophils 0.62 RBC
4.74 x 10^12/L
Metamyelocytes -
Hematocrit
0.51
Bands
MCV
U^3
Segmented
MCH
36.10 pg
Lymphocytes
0.35
MCHC
33.60 g/dL
Monocytes
0.02
RDW
16.90
Eosinophils
0.01
MPV
8.30fL
Basophils
Platelet
227 x 10^9/L
Note
1 nRBC/100 WBC
WBC
25.20 x 10^9/L
Blood type
B +