CARDIOVASCULAR CONFERENCE: APPROACH TO A PATIENT WITH CYANOTIC HEART DISEASE

General Data:

Name: Baby Boy G Neonate

History of the Present Illness Baby Boy Guadiz is born to 22-year

old primigravid 2nd year nursing student mother, married to a 23-year old unemployed partner. Initial pre-natal check up of the mother was at 6 month at a local health center. CBC and urinalysis results done revealed normal results.

History of the Present Illness UTZ done showed Single Live

Intrauterine pregnancy, cephalic, good cardiac and somatic activity, 24-25 weeks AOG, to rule out hypoplastic Right Ventricle. For further evaluation, the mother consulted at our institution and was advised fetal 2D echo.

History of the Present Illness The fetal 2D echo revealed pertinent

findings of hypoplastic Left Ventricle, hypoplastiv Mitral Valve, and a patent foramen ovale. At 26-27 weeks AOG, the mother had trichomoniasis for which she was given metronidazole tablet for 7 days. At 37-38 weeks, the mother developed UTI. Cefuroxime 500mg BID was given for 7 days that provided symptomatic relief.

History of the Present Illness The mother denied any exposure to

viral exanthems and radiation. No illicit drugs and abortifacients use. She is a non-smoker; however, was a previous alcoholic beverage drinker. Hep B screening was non-reactive and OGCT was normal. No history of hypertension, allergy, thyroid disease, diabetes, asthma, or blood dyscrasia.

History of the Present Illness Family history is negative for diabetes

mellitus, hypertension, and cardiovascular disease. The mother came in our institution for follow up but was 3cm dilatation, 70% effacement intact BOW, there was progression of labor alongside with spontaneous rupture of BOW. Clear, non-foul smelling amniotic fluid was observed. Repeat fetal 2D echo was not done due to lack of funds.

History of the Present Illness Patient was born live, term,

singleton, male, delivered via normal spontaneous delivery, BW 2.75 kg, BL 48 cm, AS 6 and 7, MT 38-39 weeks AOG, AGA.

Physical Examination on Admission: HR 134, RR 58, T 37.2˚C BW 2.75 kg, BL 48 cm, HC 33 cm, CC 31 cm,

AC 29 cm, AS 6 and 7, MT 38-39 weeks, AGA 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,

S1 and S2 normal, (-) murmurs 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