Transcript Approach to a patient with cyanotic heart disease

CARDIOVASCULAR CONFERENCE:
Approach to a patient with
cyanotic heart disease
General Data:
• Name: Baby Boy G
• Neonate
• born of a 22 year old primigravida
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, 24-25 weeks AOG, rule out hypoplastic right
ventricle.
• Suggests congenital anomal scan scan with detailed cardiac
evaulation preferably using fetal echocardiogram
– Referred to USTH
September 8, 2010
• UTZ: 2nd and 3rd trimester
– Single live intrauterine pregnancy of about 24-25
weeks in breech presentation with good cardiac
and somatic activity
– Suggest fetal 2D echo c/o Dr. Cuaso
September 8, 2010
• Assessment: Pregnancy 24-25 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 10-11 am
September 13, 2010
Macroscopic Exam
Result
Microscopic Exam
Result
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
Moderate
pH
6.0
Amorphous urates
Many
Sugar
Negative
Bacteria
Protein
negative
Cast, parasites
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
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
September 24, 2010
• Follow-up
• Unremarkable
• Still for fetal 2D Echo, 50g OGCT
October 5, 2010
• (+) terminal dysuria
• Urinalysis
– 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
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 29-30 weeks, hypoplastic
left heart
• Advised:
– Refer to pediatrics-cardiology and pediatric surgery
November 22, 2010
• (+) persistence of dysuria
• Assessment: Pregnancy 35-36 weeks AOG,
cephalic, Hypoplastic left ventricle, t/c UTI
• Advised
– Urinalysis, Hepatitis B Ag, Blood typing
November 25, 2010
• Assessment: UTI
• Advised:
– Amoxicillin 500mg/cap 1 cap q8 for 7 days
– Increase oral fluid intake
November 25, 2010
• Pediatric Surgery Consult
• Assessment: Pregnancy 36 weeks AOG, (?)
hypoplastic left ventricle
• Plans: will evaluate any time after delivery
November 26, 2010
• Blood type: AB+
December 10, 2010
• UTZ: 2nd and 3rd trimester
– There seems to be a mass in the interventricular
septum
– Single live intrauterine pregnancy of about 35-36
weeks in cephalic presentation
– BPS 8/8; SEFW 2823 grams
– Cardiomegaly
• Suggest referral to Dr. Cuaso
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
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 38-39 weeks, cephalic,
not in labor, ? Mass at the interventricular
septum, UTI s/p treatment
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 35-36
weeks in cephalic presentation
– BPS 8/8; SEFW 2823 grams
– Cardiomegaly
– Suggest referral to Dr. Cuaso
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
Maternal History
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(-) 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
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
Healthy
Family History
• No diabetes, hypertension, cardiac diseases,
cancer, tuberculosis, allergies
• Denies hereditary illnesses
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 38-39 weeks
– AGA
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
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
• 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
APPROACH TO DIAGNOSIS OF A
PATIENT PRESENTING WITH
CYANOSIS AT BIRTH
Indicators that heart disease
may exist
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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
Classification of
Congenital Heart Diseases
A) Acyanotic
B) Cyanotic
Major Considerations
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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
Subgroups of Acyanotic Diseases
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Shunt anomalies
Valvular defects
Obstructive lesions
Inflow anomalies
Primary myocardial diseases
Shunt Anomalies
• L  R shunt
• Increased pulmonary blood flow
• Increased pulmonary vascular arterial
markings on chest Xray
• ASD, VSD, PDA
Obstructive Lesion
• Discrepancy in amplitude of the peripheral
pulses
• Coarctation of the Aorta
Inflow Anomalies
• Increased pulmonary venous markings on
chest Xray
• No murmur
• Cor Triatriatum, Pulmonary vein stenosis
Valvular Defects
• Stenosis or regurgitant
• Characteristic murmur
• AS, AR, PS, PR, MS, MR, TS, TR
Primary Myocardial Diseases
• No murmur
• Disparity between cardiac size and pulmonary
vascular markings
• Glycogen storage disease
• Cardiomyopathy
Hemodynamic Consequences
A) Volume (Diastolic) overload
B) Pressure (Systolic) overload
ASD
Hemodynamic Consequence
Diastolic overload of RV
VSD
• Hemodynamic
Consequence
• MODERATE SIZE
– Volume overload of
LV
• LARGE SIZE
– Volume overload of
LV
– Pressure overload of
RV
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
Do you suspect that patient is Cyanotic?
•
When in doubt
A) Clubbing
B) CBC
C) Hyperoxia test
Hyperoxia Test
• Hyperoxia test is considered positive for
intracardiac shunting if PO2 < 150 mmHg (torr)
after 10 minutes of 100% fiO2
PVA / IVS
• Hemodynamic
Consequence
• Pressure overload
of RV
PVA / VSD
• Hemodynamic
Consequence
• Pressure overload
of RV
PDA Dependent Pulmonary Circulation
• Pulmonary valve atresia (PVA) with intact
interventricular septum
• Other lesions with accompanying PVA
Approach to diagnosis
A) Chest Xray
Increased or decreased
pulmonary vascular
arterial markings
B) EKG
RVH, LVH, CVH
C) Character of second
S2 single, loud
S2 single, normal
Split S2
heart sound
Chest x-ray
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
Pulmonary Vascular Markings
Decreased: Cyanotic
TOF
Tricuspid Atresia
Complex heart with PS
PVA / IVS
Second Heart Sound (S2)
Single Loud
Single Normal
Split S2
TGA
TOF
TAPVR without
obstruction
Aortic / Mitral
atresia
Tricuspid
atresia
Truncus
Arteriosus
PVA
Cardiac Work-Up
A) EKG
B) Chest Xray
C) 2D echocardiography
(TTE, TEE, ICE, IVUS)
D) Cardiac catheterization
E) CT angiography, cardiac MRI
• PLACE THE:
– ECG
– 2-D ECHO
Modalities of Management
A) Pharmacologic
B) Catheter based therapy
C) Surgical
Pharmacologic
A) digoxin, diuretics, inotropes (pressor),
vasodilators
B) Prostaglandin
Catheter Based Therapy (DI KO PA ALAM ITO,
EXAMPLES LANG TO)
A)
B)
C)
D)
E)
Balloon atrio septostomy (Rashkind)
Balloon valvuloplasty
Balloon angioplasty
Delivery of occlusion devices
Radio frequency ablation
Surgical (DI KO PA ALAM ITO,
EXAMPLES LANG TO)
A)
B)
C)
D)
E)
F)
Shunts like Modified Blalock-Taussig
PA band
Complete repair
Glenn, Fontan
Norwood
Jatene, Mustard, Senning
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
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Erythromycin strip 1cm OU
Vit K 1mg/IM
Hepa B vaccine 0.5mg/IM at lateral thigh
Cord care with 70% ethanol
Course in the Wards
• 7:30 AM (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
Course in the Wards
• 12:00 NN (12/23/10)
• Discharge instructions
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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
15L ECG
• Normal axis
• Sinus tachycardia
• LVH
2D echo
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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
CXR
• Lung fields are clear
• Prominent cardiac silhouette
• Suspicious prominence of pulmonary
vascularity
• Normal hemidiagphragms and sinuses
• Unremarkable visualized osseous structures
Lab results
Result
Result
Hemoglobin
171 g/L
Neutrophils
0.62
RBC
4.74 x 10^12/L
Metamyelocytes
-
Hematocrit
0.51
Bands
-
MCV
107.50 U^3
Segmented
0.62
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+