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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 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

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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 • 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 (9/6/10): right ventricle appears collapsed • Single live intrauterine pregnancy, cephalic, good cardiac and somatic activity, 24-25 weeks AOG, rule out hypoplastic right ventricle. • Suggests congenital anomal scan scan with detailed cardiac evaulation preferably using fetal echocardiogram • Referred to USTH

  4. September 8, 2010 • UTZ: 2nd and 3rd trimester • Single live intrauterine pregnancy of about 24-25 weeks in breech presentation with good cardiac and somatic activity • Suggest fetal 2D echo c/o Dr. Cuaso

  5. September 8, 2010 • Assessment: Pregnancy 24-25 weeks AOG based on 2nd trimester ultrasound, t/c hypoplastic right ventricle • Advised: • Multivitamins + FESO4 1 cap OD • Milk formula 1 glass OD • Request for CBC with blood typing, urinalysis, 50g OGCT • Request for congenital scan • Attend mother’s class every Saturday 10-11 am

  6. September 13, 2010

  7. September 13, 2010

  8. September 16, 2010 • OB GYN OPD • Speculum exam: cervix violaceous, smooth with moderate frothy yellowish creamy discharge • Assessment: Trichomoniasis • Advised: Metronidazole 500 mg/tab 1 tab BID • Fetal 2D Echo once with funds • 50g OGCT, repeat urinalysis clean catch

  9. September 24, 2010 • Follow-up • Unremarkable • Still for fetal 2D Echo, 50g OGCT

  10. October 5, 2010 • (+) terminal dysuria • Urinalysis • Acidic • (++) bacteria • 2-5/hpf pus cells • Normal OGCT results • Advised: • Amoxicillin 500 mg/tab 1 tab q8 for 7 days • Once with 2D Echo results, refer to pediatric surgery • (+) hyperemic conjunctiva OD- referred to Ophtha

  11. October 15, 2010 • USTH (October 11, 2010) • Fetal 2D- Echocardiogram: hypoplastic Left Ventricle, hypoplastic Mitral Valve, and a patent foramen ovale • FHT 142 • Assessment: Pregnancy 29-30 weeks, hypoplastic left heart • Advised: • Refer to pediatrics-cardiology and pediatric surgery

  12. November 22, 2010 • (+) persistence of dysuria • Assessment: Pregnancy 35-36 weeks AOG, cephalic, Hypoplastic left ventricle, t/c UTI • Advised • Urinalysis, Hepatitis B Ag, Blood typing

  13. November 25, 2010 • Assessment: UTI • Advised: • Amoxicillin 500mg/cap 1 cap q8 for 7 days • Increase oral fluid intake

  14. November 25, 2010 • Pediatric Surgery Consult • Assessment: Pregnancy 36 weeks AOG, (?) hypoplastic left ventricle • Plans: will evaluate any time after delivery

  15. November 26, 2010 • Blood type: AB+

  16. December 10, 2010 • UTZ: 2nd and 3rd trimester • There seems to be a mass in the interventricular septum • Single live intrauterine pregnancy of about 35-36 weeks in cephalic presentation • BPS 8/8; SEFW 2823 grams • Cardiomegaly • Suggest referral to Dr. Cuaso

  17. December 10, 2010 • High Risk OB GYN clinic • Assessment: Hypoplastic left ventricle, hypoplastic mitral valve, UTI, r/o IUGR • Advised: Terraferon, Clusivol OB, Cefuroxime 500 mg/tab BID for 7 days • Repeat urinalysis after 7 days • BPS

  18. December 17, 2010 • UTZ: 38 weeks 6 days AOG • (-) dysuria • (+) fetal movements, irregular hypogastric pains, SEFW p10-50 • IE: 1 cm dilated, 60% effaced, (+) BOW, cephalic, Stn-3 • Assessment: Pregnancy 38-39 weeks, cephalic, not in labor, ? Mass at the interventricular septum, UTI s/p treatment

  19. December 12, 2010 • UTZ: 2nd and 3rd trimester • Findings: • There seems to be a mass at the interventricular septum • Single live intrauterine pregnancy of about 35-36 weeks in cephalic presentation • BPS 8/8; SEFW 2823 grams • Cardiomegaly • Suggest referral to Dr. Cuaso

  20. December 20, 2010 • For follow up • Supposedly for repeat Fetal 2D Echo • 3 cm dilated, 70% effaced intact BOW, there was progression of labor alongside with spontaneous rupture of BOW. • Clear, non-foul smelling amniotic fluid

  21. 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

  22. Family History

  23. Family History • No diabetes, hypertension, cardiac diseases, cancer, tuberculosis, allergies • Denies hereditary illnesses

  24. 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 • AGA

  25. Physical Examination on Admission • HR 134 bpm, RR 58 cpm, T 37.2˚C • Blue, pale, (+) circumoral cyanosis • (-) Rash, (-) birth marks, (+) palmar and plantar cyanosis • (+) Molding, (+) caput succedaneum (-) cephalhematoma • (+) ROR OU, (-) eye discharge, normal set ears, (-) preauricular pits, patent nares, (-) Epstein’s pearls

  26. Physical Examination on Admission • (-) Palpable neck masses, intact clavicle, no crepitations • (-) Chest deformities, symmetrical chest expansion, (-) retractions, clear and equal breath sounds, good respiratory effort • Adynamicprecordium, regular heart rate and rhythm, grade 1 holosystolic murmur at left parasternal area • Globular abdomen, (+) umbilical stump with 2 arteries and 1 vein, (-) organomegaly, (-) palpable masses

  27. Grossly male, bilaterally descended testes, good rugae, patent anus • Femoral pulses full and equal, good flexion of extremities, (-) Barlow, (-) Ortolani • Straight spine, (-) sacral dimpling, (-) tuft of hair • (+) Moro, grasp, rooting, plantar, and sucking reflexes

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

  29. 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

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

  31. 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

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

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

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

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

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

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

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

  39. ASD Hemodynamic Consequence Diastolic overload of RV

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

  41. 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

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

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

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

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

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

  47. Approach to diagnosis

  48. Chest x-ray

  49. Causes of Cyanosis

  50. Pulmonary Vascular MarkingsDecreased: Cyanotic

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