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: single live intrauterine pregnancy, cephalic,
good cardiac and somatic activity, 24-25 weeks AOG, rule out hypoplastic right ventricle.
– Referred to USTH
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
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
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
Family HistoryName Age Relation Educational
AttainmentOccupation Health
MPG 22 Mother 2nd year nursing student
Student Healthy
LG 23 Father High school graduate
Unemployed Healthy
Family History
• No diabetes, hypertension, 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 AOG– AGA
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
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
APPROACH TO DIAGNOSIS OF A PATIENT PRESENTING WITH CYANOSIS AT BIRTH
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
Classification of Congenital Heart Diseases
A) Acyanotic
B) Cyanotic
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
Subgroups of Acyanotic Diseases
• 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 doubtA) ClubbingB) CBCC) 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 heart sound
S2 single, loudS2 single, normalSplit S2
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 MarkingsDecreased: 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) EKGB) Chest XrayC) 2D echocardiography
(TTE, TEE, ICE, IVUS)D) Cardiac catheterizationE) CT angiography, cardiac MRI
• PLACE THE:– ECG– 2-D ECHO
Modalities of Management
A) PharmacologicB) Catheter based therapyC) Surgical
Pharmacologic
A) digoxin, diuretics, inotropes (pressor), vasodilators
B) Prostaglandin
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
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