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CARDIOVASCULAR CONFERENCE: Approach to a patient with cyanotic heart disease

CARDIOVASCULAR CONFERENCE: Approach to a patient with cyanotic heart disease. General Data:. Name: Baby Boy G Neonate. History of the Present Illness.

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CARDIOVASCULAR CONFERENCE: Approach to a patient with cyanotic heart disease

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

  2. General Data: • Name: Baby Boy G • Neonate

  3. 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.

  4. 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.

  5. 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.

  6. 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.

  7. 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.

  8. 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.

  9. 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

  10. 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, 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

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

  12. 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

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

  14. 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

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

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

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

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

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

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

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

  22. ASD Hemodynamic Consequence Diastolic overload of RV

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

  24. 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

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

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

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

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

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

  30. Approach to diagnosis

  31. Chest x-ray

  32. Causes of Cyanosis

  33. Pulmonary Vascular MarkingsDecreased: Cyanotic

  34. Second Heart Sound (S2)

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

  36. PLACE THE: • ECG • 2-D ECHO

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

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

  39. 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

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

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