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 – UTZ: single live intrauterine pregnancy, cephalic, good cardiac and somatic activity, 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 weeks AOG Metronidazole 500mg/tab for 7 days – (+) UTI 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 History NameAgeRelationEducational Attainment OccupationHealth MPG22Mother2 nd year nursing student StudentHealthy LG23FatherHigh school graduate UnemployedHealthy

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 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 (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

Chest x-ray

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