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: single live intrauterine pregnancy, cephalic,
good cardiac and somatic activity, 24-25 weeks AOG, rule out hypoplastic right ventricle.
– Referred to USTH
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HPI
• USTH (October 2010)– Fetal 2D- Echocardiogram: hypoplastic Left
Ventricle, hypoplastic Mitral Valve, and a patent foramen ovale
– (+) Trichomoniasis• 26-27 weeks AOG• Metronidazole 500mg/tab for 7 days
– (+) UTI• 37-38 weeks AOG• Cefuroxime 500mg BID for 7 days
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HPI
• The mother came in our institution for follow up
• 3 cm dilated, 70% effaced intact BOW, there was progression of labor alongside with spontaneous rupture of BOW.
• Clear, non-foul smelling amniotic fluid• Repeat fetal 2D echo was not done due to lack
of funds
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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, 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 AOG– AGA
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Physical Examination on Admission
• HR 134 bpm, RR 58 cpm, T 37.2˚C • Blue, pale; some flexion of extremities, good
respiratory effort, cyanotic • (-) Rash, (-) birth marks,• (+) 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 • Adynamic precordium, regular heart rate and rhythm, grade 1
holosystolic murmur • 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, (-) 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