cardiovascular conference: approach to a patient with cyanotic heart disease
DESCRIPTION
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 6 th month of pregnancy at local health center CBC, urinalysis normal - PowerPoint PPT PresentationTRANSCRIPT
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CARDIOVASCULAR CONFERENCE: Approach to a patient with
cyanotic heart disease
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General Data:
• Name: Baby Boy G• Neonate• born of a 22 year old primigravida
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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
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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
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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
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September 13, 2010Macroscopic 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
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September 13, 2010Test 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
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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
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September 24, 2010
• Follow-up• Unremarkable • Still for fetal 2D Echo, 50g OGCT
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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
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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
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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
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November 25, 2010
• Assessment: UTI• Advised:
– Amoxicillin 500mg/cap 1 cap q8 for 7 days– Increase oral fluid intake
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November 25, 2010
• Pediatric Surgery Consult• Assessment: Pregnancy 36 weeks AOG, (?)
hypoplastic left ventricle• Plans: will evaluate any time after delivery
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November 26, 2010
• Blood type: AB+
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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
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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
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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
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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
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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
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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
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Family HistoryName Age Relation Educational
AttainmentOccupation Health
MPG 22 Mother 2nd year nursing student
Student Healthy
LG 23 Father High school graduate
Unemployed Healthy
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Family History
• No diabetes, hypertension, cardiac diseases, cancer, tuberculosis, allergies
• Denies hereditary illnesses
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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
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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
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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
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• 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
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APPROACH TO DIAGNOSIS OF A PATIENT PRESENTING WITH CYANOSIS AT BIRTH
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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
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Classification of Congenital Heart Diseases
A) Acyanotic
B) Cyanotic
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Major Considerations
• Is there a shunt (LR or RL)• Is there obstruction to inflow or outflow• Abnormal heart valves• Abnormal connections of great vessels• Combination
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Subgroups of Acyanotic Diseases
• Shunt anomalies• Valvular defects• Obstructive lesions• Inflow anomalies• Primary myocardial diseases
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Shunt Anomalies
• L R shunt• Increased pulmonary blood flow• Increased pulmonary vascular arterial
markings on chest Xray
• ASD, VSD, PDA
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Obstructive Lesion
• Discrepancy in amplitude of the peripheral pulses
• Coarctation of the Aorta
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Inflow Anomalies
• Increased pulmonary venous markings on chest Xray
• No murmur
• Cor Triatriatum, Pulmonary vein stenosis
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Valvular Defects
• Stenosis or regurgitant• Characteristic murmur
• AS, AR, PS, PR, MS, MR, TS, TR
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Primary Myocardial Diseases
• No murmur• Disparity between cardiac size and pulmonary
vascular markings
• Glycogen storage disease• Cardiomyopathy
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Hemodynamic Consequences
A) Volume (Diastolic) overload
B) Pressure (Systolic) overload
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ASD
Hemodynamic Consequence
Diastolic overload of RV
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VSD• Hemodynamic
Consequence• MODERATE SIZE
– Volume overload of LV
• LARGE SIZE– Volume overload of
LV– Pressure overload of
RV
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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
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Do you suspect that patient is Cyanotic?
• When in doubtA) ClubbingB) CBCC) Hyperoxia test
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Hyperoxia Test
• Hyperoxia test is considered positive for intracardiac shunting if PO2 < 150 mmHg (torr) after 10 minutes of 100% fiO2
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PVA / IVS• Hemodynamic
Consequence
• Pressure overload of RV
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PVA / VSD• Hemodynamic
Consequence
• Pressure overload of RV
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PDA Dependent Pulmonary Circulation
• Pulmonary valve atresia (PVA) with intact interventricular septum
• Other lesions with accompanying PVA
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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, loudS2 single, normalSplit S2
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Chest x-ray
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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
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Pulmonary Vascular MarkingsDecreased: Cyanotic
TOF Tricuspid Atresia
Complex heart with PS PVA / IVS
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Second Heart Sound (S2)
Single Loud Single Normal Split S2
TGA TOF TAPVR without obstruction
Aortic / Mitral atresia
Tricuspid atresia
Truncus Arteriosus
PVA
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Cardiac Work-Up
A) EKGB) Chest XrayC) 2D echocardiography
(TTE, TEE, ICE, IVUS)D) Cardiac catheterizationE) CT angiography, cardiac MRI
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• PLACE THE:– ECG– 2-D ECHO
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Modalities of Management
A) PharmacologicB) Catheter based therapyC) Surgical
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Pharmacologic
A) digoxin, diuretics, inotropes (pressor), vasodilators
B) Prostaglandin
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Catheter Based Therapy (DI KO PA ALAM ITO, EXAMPLES LANG TO)
A) Balloon atrio septostomy (Rashkind)B) Balloon valvuloplastyC) Balloon angioplastyD) Delivery of occlusion devicesE) Radio frequency ablation
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Surgical (DI KO PA ALAM ITO, EXAMPLES LANG TO)
A) Shunts like Modified Blalock-TaussigB) PA bandC) Complete repairD) Glenn, FontanE) NorwoodF) Jatene, Mustard, Senning
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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
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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
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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
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15L ECG
• Normal axis• Sinus tachycardia• LVH
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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
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CXR
• Lung fields are clear• Prominent cardiac silhouette• Suspicious prominence of pulmonary
vascularity• Normal hemidiagphragms and sinuses• Unremarkable visualized osseous structures
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Lab resultsResult 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 +