CARDIOVASCULAR CONFERENCE: Approach to a patient with cyanotic heart disease

1. CARDIOVASCULAR CONFERENCE: Approach to a patient with cyanotic heart disease

2. General Data: • Name: Baby Boy G • Neonate • born of a 22 year old primigravida

3. 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

4. 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

5. 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

6. Maternal History • (-)exposure to radiation • (-) symptoms of viral exanthems • (-) use ofillicit 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

7. Family History

8. Family History • No diabetes, hypertension, allergies • Denies hereditary illnesses

9. 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

10. 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

11. Physical Examination on Admission • (-) Palpable neck masses, intact clavicle, no crepitations • (-) Chest deformities, symmetrical chest expansion, (-) retractions, clear and equal breath sounds • Adynamicprecordium, 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

12. APPROACH TO DIAGNOSIS OF A PATIENT PRESENTING WITH CYANOSIS AT BIRTH

13. 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

14. Classification of Congenital Heart Diseases A) Acyanotic B) Cyanotic

15. 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

16. Subgroups of Acyanotic Diseases • Shunt anomalies • Valvular defects • Obstructive lesions • Inflow anomalies • Primary myocardial diseases

17. Shunt Anomalies • L  R shunt • Increased pulmonary blood flow • Increased pulmonary vascular arterial markings on chest Xray • ASD, VSD, PDA

18. Obstructive Lesion • Discrepancy in amplitude of the peripheral pulses • Coarctation of the Aorta

19. Inflow Anomalies • Increased pulmonary venous markings on chest Xray • No murmur • Cor Triatriatum, Pulmonary vein stenosis

20. Valvular Defects • Stenosis or regurgitant • Characteristic murmur • AS, AR, PS, PR, MS, MR, TS, TR

21. Primary Myocardial Diseases • No murmur • Disparity between cardiac size and pulmonary vascular markings • Glycogen storage disease • Cardiomyopathy

22. Hemodynamic Consequences A) Volume (Diastolic) overload B) Pressure (Systolic) overload

23. ASD Hemodynamic Consequence Diastolic overload of RV

24. VSD • Hemodynamic Consequence • MODERATE SIZE • Volume overload of LV • LARGE SIZE • Volume overload of LV • Pressure overload of RV

25. 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

26. Do you suspect that patient is Cyanotic? • When in doubt • Clubbing • CBC • Hyperoxia test

27. Hyperoxia Test • Hyperoxia test is considered positive for intracardiac shunting if PO2 < 150 mmHg (torr) after 10 minutes of 100% fiO2

28. PVA / IVS • Hemodynamic Consequence • Pressure overload of RV

29. PVA / VSD • Hemodynamic Consequence • Pressure overload of RV

30. PDA Dependent Pulmonary Circulation • Pulmonary valve atresia (PVA) with intact interventricular septum • Other lesions with accompanying PVA

31. Approach to diagnosis

32. Chest x-ray

33. Causes of Cyanosis

34. Pulmonary Vascular MarkingsDecreased: Cyanotic

35. Second Heart Sound (S2)

36. Cardiac Work-Up • EKG • Chest Xray • 2D echocardiography (TTE, TEE, ICE, IVUS) • Cardiac catheterization • CT angiography, cardiac MRI

37. PLACE THE: • ECG • 2-D ECHO

38. Modalities of Management • Pharmacologic • Catheter based therapy • Surgical

39. Pharmacologic • digoxin, diuretics, inotropes (pressor), vasodilators • Prostaglandin

40. Catheter Based Therapy (DI KO PA ALAM ITO, EXAMPLES LANG TO) • Balloon atrioseptostomy (Rashkind) • Balloon valvuloplasty • Balloon angioplasty • Delivery of occlusion devices • Radio frequency ablation

41. Surgical (DI KO PA ALAM ITO, EXAMPLES LANG TO) • Shunts like Modified Blalock-Taussig • PA band • Complete repair • Glenn, Fontan • Norwood • Jatene, Mustard, Senning