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