General Data:  Name: Baby Boy G  Neonate

History of the Present Illness  Baby Boy Guadiz is born to 22-year old primigravid 2 nd 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, 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 weeks AOG, the mother had trichomoniasis for which she was given metronidazole tablet for 7 days. At 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 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 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

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 (L  R or R  L)  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 doubt A) Clubbing B) CBC C) Hyperoxia test

Hyperoxia Test  Hyperoxia test is considered positive for intracardiac shunting if PO 2 < 150 mmHg (torr) after 10 minutes of 100% fiO 2

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 XrayIncreased or decreased pulmonary vascular arterial markings B) EKGRVH, LVH, CVH C) Character of second heart sound S2 single, loud S2 single, normal Split S2

Causes of Cyanosis NoncardiacCardiac 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 Markings Decreased: Cyanotic TOFTricuspid Atresia Complex heart with PSPVA / IVS

Second Heart Sound (S2) Single LoudSingle NormalSplit S2 TGATOFTAPVR without obstruction Aortic / Mitral atresia Tricuspid atresia Truncus Arteriosus PVA

Cardiac Work-Up A) EKG B) Chest Xray C) 2D echocardiography (TTE, TEE, ICE, IVUS) D) Cardiac catheterization E) CT angiography, cardiac MRI

 PLACE THE:  ECG  2-D ECHO

Modalities of Management A) Pharmacologic B) Catheter based therapy C) 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 valvuloplasty C) Balloon angioplasty D) Delivery of occlusion devices E) Radio frequency ablation

Surgical (DI KO PA ALAM ITO, EXAMPLES LANG TO) A) Shunts like Modified Blalock-Taussig B) PA band C) Complete repair D) Glenn, Fontan E) Norwood F) Jatene, Mustard, Senning