1. Extern Interesting Case Group 3 7 June 2007

2. History Case 5 months-old boy Chief complaint : Dyspnea during breastfeeding 2 months PTA

3. Present history 2 months PTA, his mother noticed that her child had dyspnea during breastfeeding. He took time about 2-3 minutes each feeding and rested 30 minutes before continue next feeding. At the appointment for vaccination, He was detected that his weight didn’t gain well.

4. Present history (cont’) 3 wk PTA, He had fever, productive cough with progressive dyspnea. He was brought to a private hospital. Physical examination shown coarse crepitation both lung, pansystolic murmur grade III/VI at left parasternal border and liver was palpated 1 cm. below right costal margin

5. Present history (cont’) He was diagnosed pneumonia with congestive heart failure and was admitted. Cefotaxime 150 mg IV q 8 hr and Digoxin 0.4 ml oral bid was given for eight days. After pneumonia resolved, he was referred to Siriraj hospital.

6. Other history Developmental history : Rolling, Palmar grasp, Turn to voice and bubbling. Food history : Breast feeding, infant formula and supplementary foods. Drug and Vaccination : Last vaccination at 4 month-old. No drug allergy Delivery history : Normal labour, term AGA BW 3150 Apgar 9,9 Family history : No congenital heart disease, genetic disease in family

7. Physical examination V/S : T. 36.7C, RR 80/min, PR 177/min BP 71/53 75/47 O2 sat 99 98 90/40 80/53 99 98 BW 4.4 kg (<p3) Lt. 61 cm (p25) HC 40 cm (p25)

8. Growth Chart Weight < p3 Length p25

9. Physical examination GA : Alert,mildly pale,no jaundice, tachypnea, marked dyspnea (suprasternal, substernal and subcostal retraction), no central and peripheral cyanosis, no clubbing of finger, no paradoxical of chest movement, no dysmorphic feature HEENT : pharynx and tonsils not injected, no thyroid enlargement

10. Physical examination RS : Pectus carinatum, normal breath sound, no adventitious sound

11. Physical examination CVS : PMI at 5 th Intercostal space just lateral to midclavicular line, Apical heaving. Loud P 2 No bounding pulse - Pansystolic murmur gr III/VI at Left lower sternal border - Diastolic rumbling murmur gr II at apex

12. Physical examination Abdomen : soft, not tender, liver 2 cm Below right costal margin, smooth surface rubbery consistency. spleen not palpable NS : active, symmetrical movement, normal muscle tone, good motor power

13. Problem list 1. Dyspnea during breastfeeding for 2 months 2. Poor weight gain 3. Tachypnea, tachycardia, hepatomegaly and cardiomegaly 4.Heart murmurs 5.History of pneumonia 6. Mildly pale

14. Approach to dyspnea Cardiology cause Respiratory cause Metabolic cause Neurologic cause

15. Congestive heart failure Cardinal signs 1. Tachycardia 2. Tachypnea 3. Cardiomegaly  Pectus carinatum 4. Hepatomegaly History of dyspnea on lactation ( feeding difficulties > 20 min ) Poor weight gain  failure to thrive

16. Investigation Complete blood count Hb 9.1 g/dl Hct 31.1 % MCV 58.2 fl RDW 18.3% Wbc 12,020 /mm3 ( N 32 % L 59 % ) Platelet 523,000 /mm3 Peripheral blood smear Hypochromic microcytic anemia no anisopoikilocytosis

17. Investigation Blood chemistrry BUN 11, Cr 0.2, Na 139,K 3.8, Cl 104, HCO3 22 Ca 9.4, Mg 2.1, PO 4 57 VBG ( on oxygen 1 LPM ) pH 7.363 pCO 2 43.80 pO 2 72.5 HCO 3 25.1 O 2 sat 93.7

18. Investigation CT ratio = 0.65 Increase pulmonary vasculature CXR Portable

19. EKG Left atrial enlargement Left ventricular hypertrophy

20. Etiology of Heart disease Congenital heart disease Acquired heart disease

21. Evaluation of congenital heart disease Congenital heart disease Acyanotic / Cyanotic Physical examination Pulse oximetry Increase / decrease/ normal pulmonary vascular marking Chest x ray RVH / LVH / Biventricular hypertrophy EKG Physical examination : murmur DIFFERENTIAL DIAGNOSIS DIAGNOSIS ECHOCARDIOGRAPHY

22. Approach to congenital heart disease -Large VSD -PDA -ECD -ASD (often RBBB) -PAPVR -AS -AR -CoA -MR -PS -CoA -MS -Truncus arteriosus -Common ventricle -TGA+VSD -TGA -TAPVR -HLHS -TGA + PS -Common ventricle c PS -TA -PA c Hypoplasia RV -TOF -Ebstein anomaly -PVOD 2 to VSD, PDA

23. Approach to congenital heart disease -Large VSD -PDA -ECD

24. Approach to acyanotic heart disease -Large VSD -PDA -ECD

25. Echocardiogram Gold standard for diagnosis Moderate perimembranous extended to inlet VSD 8 mm, left to right shunt, no PDA no coarctation of aorta EF 70 %

26. Large ventricular septal defect Pansystolic murmur grade III at LLSB Pansystolic murmur Diastolic rumbling murmur grade II at apex Diastolic rumbling murmur ( Relative mitral stenosis ) Loud P 2 Loud P 2

27. Definite diagnosis Congestive heart failure with moderate ventricular septal defect with failure to thrive

28. Ventricular septal defect

29. Type of VSD Type I ( outlet or subpulmonary or subarterial type) Type II (membranous type) Type III (inlet type) Type IV (muscular type) I Subpulmonary II membranous III inlet IV Muscular

30. Sign and symptoms Size of VSDSymptomSign Smallasymptomaticpansystolic murmur at LLSB ModerateDyspnea on exertion CHF (about age 6-8th weeks), loud P2, Pansytolic murmur at Largedyspnea at rest LLSB and mild diastolic rumbing murmur at apex (relative MS)

31. Clinical course Small defects, close spontaneously (during the first 2 years) esp Type II and IV The vast majority of defects, close before aged 4 years (may be in adults) Moderate or Large VSD : mostly remain

32. Complication Pulmonary vascular obstruction disease or Eisenmenger syndrome VSD c PS Recurrent pneumonia Infective endocarditis Aortic regurgitation esp Type I

33. Treatment Small VSD : F/U q 1-2 yr, check AR q 2-3 years esp Type I Moderate or large VSD : treat CHF,surgery for repairment before age at 2 nd years

34. Surgery in VSD Age <6 mo : CHF or recurrent pneumonia or FTT (HC) Age 6-24 mo : moderate or large VSD P pulmonary a. > P lt venticle 2 times Age >24 mo : Qp : Qs > 2:1 Complication : pericarditis, aortic cusp prolapse, murmur of aortic regurgitation (< 10 yr) Indication for surgery

35. Management 1. Support breathing and ventilation 2. Posture : semi-fowler position 3. Decrease physical activities : rest often and sleep adequately +/- sedation/analgesia 4. Medication : inotropic support, preload and afterload reduction 5. Diet : increase daily calories, “ no added salt diets ” 6. Correct precipitating cause 7. Surgical correction of CHD if indicated

36. Medication in CHF 1. Inotropic support Digitalis Loading dose = total digitalization dose = TDD in 24 hr TDD/2 TDD/4 TDD/4 Maintainance dose = TDD/4 devided in two given at 12 hr interval 12 HR 6-8 HR 6-8HR -Monitor : EKG & rhythm before each of the three digitalizing doses Serum digoxin when suspected digitalis toxicity Blood for serum electrolyte before & after administration

37. The dosage of digitalis ( oral ) TDD (mg/kg)Maintainance (mg/kg/day) Term infant0.030.008-0.01 Preterm infant0.02-0.250.005 Age< 6 yr0.03-0.040.008-0.01 Age> 6 yr0.02 (max 1 mg)0.125-0.25mg/day

38. Medication in CHF 1. Inotropic support A and B -adrenagic agonists IV Dopamine 2–30µg/kg/min Dobutamine 2–20µg/kg/min Isoproterenol 0.01–0.5µg/kg/min Epinephrine 0.05–1.0µg/kg/min Norepinephrine 0.1–2.0µg/kg/min

39. Medication in CHF 2. Preload-reducing agents Furosemide (Lasix) – IV 1-2 mg/dose prn – PO 1-4 mg/kg/day, divided qd–qid Bumetanide (Bumex) – IV 0.01-0.1mg/kg/dose – PO 0.05-0.1 mg/kg/day, divided q 6– 8h Chlorothiazide (Diuril) – PO 20-50 mg/kg/day, divided bid or tid Spironolactone (Aldactone) – PO 1-3 mg/kg/day, divided bid or tid Monitor serum electrolyte in long term therapy BW, urine input / output

40. 3. Afterload-reducing agents Hydralazine (Apresoline) IV or IM 0.1-0.5 mg/kg/dose (max 20mg ) PO 0.25-1 mg/kg/dose q 6-8h (max 200 mg/day) Nitroglycerin 0.25-5 µg/kg/min Nitroprusside (Nipride) IV 0.5-8 µg/kg/min Captopril (Capoten) PO Infants 0.1-0.5mg/kg/dose q8-12h (max 4 mg/kg/day) Prematures: start at 0.01mg/kg/dose Children 0.1-2 mg/kg/day q 8-12 h Enalapril (Vasotec) PO 0.08-0.5mg/kg/dose q12-24h (max1mg/kg/day) monitor : BP ( keep BP post Rx >/= BP pre Rx ) Medication in CHF

41. Others Phosphodiesterase inhibitors IV Amrinone 3-10 µg/kg/min Milrinone 0.25 - 1µg/kg/min Chronic treatment with B-blocker

42. Treatment in this patient Lasix ( 1 MKDose) 4.5 mg po q 8 hr Aldactone ( 2 MKDay ) 2 ml IV q 12 hr Lanoxin ( TDD 0.04) 0.04 ml po bid x one day then Dobutamine IV 1 cc/hr ( 1cc/hr = 5ug/kg/day ) Captopril ( 0.1 MKDose ) 0.4 ml po q 8 hr step to 3 MKDose

43. Monitoring in heart failure Clinical Dyspnea, tachypnea Physical exam Perfusion, RR, Sleeping pulse, liver size and consistency Input / output per day and body weight

44. Progression in this patient Day 2 : PRC has been given due to Hct 31 % Off lanoxin Captropril has been stepped up to 0.2 MKDay Day 3 : Captropril has been stepped up to 0.3 MKDay Lanoxin oral has been given Lasix has been given because he looked more dyspnea and weight gain Day 4 : Off Dobutamine Day 5 : Change lasix to oral form After treated for 6 days less tachypnea, less dyspnea, liver is soft

45. Plan of management Try medical treatment : Lasix 1 MKDose oral q 12 hr Aldactone 2 mg/kg/day Captopril 0.3 mg/kg/dose Lanoxin TDD 0.04 Ferrous sulfate drops 2-3 mg/kg/day Follow up 3 months at cardiology clinic Surgery if failure to thrive or clinical does not improve

46. Take home message – Cardinal sign of CHF : Tachypnea, Tachycardia Hepatomegaly, Cardiomegaly – CHD is vary in clinical presentation – VSD must be referred to treat before 2 yrs – The severity of disease does not depend on loudness of heart murmur

47. Thank you for your attention Special thanks Dr. Kritvikrom Dr.Niran Dr. Jarupim