Acquired Heart Diseases.

1. Acquired Heart disease
•Disease affecting cardiac tissue and functio
n which does not have its inception at birth
and usually is secondary to an extraneous a
gent.

2. Types of Acquired Heart Disease
•Ischemic / hypoxic
•Hypertensive
•infectious
•Inflammatory
•Metabolic
•Nutritional
•Traumatic

3. Ischemic / Hypoxic
•Coronary occlusion
o Atherosclerosis
o Kawasaki Disease
o Sickle cell anemia
•Hypoperfusion
o Surgical ischemic arrest
o severe hypotension
•Asphyxia

4. Hypertensive
•Systemic hypertension
•Pulmonary hypertension

5. Infectious
•Pericarditis
•Myocarditis
•Endocarditis

6. Inflammatory
•Post-pericardiotomy Syndrome
•Rheumatic fever
•Collagen vascular Diseases

7. Metabolic
•Endocrine adenopathy
o Adrenal
o Pituitary
o Pancreatic
o Thyroid
o Parathyroid
•Storage diseases
o Glycogen
o Mucopolysacchrides

8. Nutritional
•Nutritional deficiencies
o Starvation
o Vitamin
o Mineral
o Carnitine
•Nutritional Excesses
o Obesity
o Vitamin
o Mineral

9. Traumatic
•Penetrating
•Blunt

10. •Kawasaki Disease
•Pericarditis and post-pericardiotomy syndr
ome
•Myocarditis / congestive Cardiomyopathy
•Infectious Endocarditis
•Rheumatic heart disease

11. Kawasaki Disease
•Recognized in 1970’s
•Inflammatory disease of unknown etiology
•9.2/100,000 cases per year; usually <4 y/o
•Winter and spring; 3yr”epidemics”
•Asiatics and blacks > white: 9&1.5/1

12. Kawasaki’s Disease Pathophysiolo
gy
•Immunoregulatory anomalies
o Activation of T and B lymphocytes
o Production of immunoglobulins and cytokines
o wide spread immune reaction
•Generalized microvasculitis
•Myocardial and pericardial inflammation
•Coronary vasculitis

13. Kawasaki’s Disease Clinical Manif
estations
•Fever of 5 + days duration
•Physical findings
o Polymorphous rash
o Non-purulent conjunctivitis
o Erythema of oral membranes including tongue
o Indurative edema of hands and feet
o Cervical lymphadenopathy
•Acute and often severe toxic presentation
•multi-organ involvement

18. Kawasaki’s Disease Laboratory Fin
dings
•Elevated acute phase reactants
•Elevated ESR
•Elevated Platelets
•Myocardial dysfunction
•Pericardial effusion
•Coronary thickening ---> coronary dilatatio
n and aneurysm

19. Kawasaki's Disease- Cardiovascular Stages
• 20% of untreated; 2-4% with treatment
• Stage1: Week 1-2
o Microvasculitis
o Peri, myo, and endocarditis
o endocarditis and perivasculitis of coronaries
• Stage 2: Week 1.5-3
o Vasculitis of coronaries with aneurysms and thrombi
o intimal proliferation of coronaries
o peri, myo-, and endocarditis
• Stage 3: Week 4-5
o Scaring and intimal thickening of Coronaries
o Myocardial infarction
• Stage 4: >2m0
o Advanced coronary artery disease
o Myocardial Fibrosis

20. Echocardiographic Findings
•Acute phase:
o Pericardial effusion
o LV dysfunction
o Diffuse coronary artery wall thickening and dilat
ation in 30-50%
•Coronary dilatation
o <5y/o, lumen >3 mm
o Sacular or fusiform

21. Treatment
•IVGG: 2g/kg over 24 hrs
•ASA:
o 20-25 mg/kg/dose, q 6 hrs
 until afebrile 2-3 days
o 3-5 mg/kg/day
 6-8 weeks, until ESR and plt count normal
 Indefinitely if coronary artery anomalies

22. Pericarditis / post-pericardiotomy
Syndrome
•Inflammation(infection) of pericardial spac
e
•Chest pain
•Friction rub
•Pericardial effusion
•Fever
•Elevated ESR

23. Pericarditis
•Viral
•Purulent
•Tuberculous
•Rheumatic
•Kawasaki
•Uremic

24. Post - pericardiotomy Syndrome
•30%, if pericardium opened
•1-2 weeks post surgery
•Etiology??
o Viral Autoimmune
•Symptoms:
o Fever
o Chest pain
o Friction rub
o Pericardial effusion

25. Post - pericardiotomy syndrome
•Treatment:
o ASA: 50-75 mg/kg/day; 4-6 weeks
o Steroids: 2mg/kg/day; taper over 3-4 weeks
o Diuretics (cautiously)

26. Cardiac tamponade
•Pathophysiology
o Increase in pericardial fluid which elevates fillin
g pressures, impedes ventricular filling and decr
eases cardiac output
o Rapid small volume increase versus large chroni
c volume

27. Cardiac Tamponade
•Physical findings
o Decreased heart sounds
o Distended jugular veins
o Pulsus paradoxus
 >10 mmHg decrease in SBP with inspiration
 Increased pooling of blood in pulmonary bed due to
decreased LV filling

28. Cardiac tamponade
•ECG:
o Low voltage
o ST - T wave changes
o Electrical alternans
•CXR
o “Water - bottle” heart, if large volume
o Normal, if acute
•ECHO
o space between heart and pericardium
o Swinging heart
o Inspiratory variation in Doppler flows

30. Myocarditis / Congestive Cardiom
yopathy
•Infection of myocardium with lymphocytic i
nfiltration
•Degenerative process affecting myocytes
•Impairment of myocardial function

31. Myocarditis - Etiology
•Viral - Coxackievirus, ECHO, adeno, etc.
•Bacterial - Tuberculosis, strep, etc.
•Fungal - unusual
•Protozoan - Chaga’s disease (T.cruzi), malar
ia, toxoplasmosis
•Rickettsial
•Spirochetal
•metazoal - trichinosis, echinococcosis, etc.

32. Congestive Cardiomyopathy - Etiol
ogy
•Infectious - viral
•familial - duchenne’s
•Metabolic - glycogen storage
•Ischemic - Kawasaki
•Toxic - anthracyclines
•Nutritional - carnitine

33. Clinical Manifestation
•General malaise or viral syndrome
•low cardiac output state (Shock)
•Gallop rhythm (mitral insufficiency)
•ST - T wave changes
•ECHO:
o Reduced shortening fraction
o Segmental wall motion anomalies
o Valvar insufficiency

34. Course
Myocarditis
70-80% 20-30%
Mild-Mod CHF Severe CHF
60-70% 10-20% 10%
Recovery Dil.Cardiomyo Death/Trans

35. Diagnosis
•Clinical findings
•Identification of etiologic agent
•Endomyocardial biopsy
o Lymphocytic infiltrate
o Etiologic agent
o Necrosis
o “Staging”

36. Treatment
•Symptomatic
o inotropes
o Diuretics
o afterload reduction
•Correction of etiology
•Immunosupression
o Steroids
o Anti-virals
o Cyclosporin
o Interferon
•Transplantation

38. Infective Endocarditis
•Microbial infection of endocardial surface of heart
- valves or wall
• “Acute” (virulent) / “subacute” (prolonged)
•1:1800 to 1:4500 ped cases: admissions
•Any age; greater in 5th decade
•Pre-v. post-antibiotic era - no change
•Factors:
o Better diagnosis
o Drug abuse
o Treatment modalities

39. Etiology
•Alpha hemolytic strep: most common (>60
%); prolonged
•Staph aureus: 2nd most common (20%); vir
ulent
•Beta hemolytic strep: uncommon
•Coagulase negative Staph: increasing
•Candida

40. Risk Factors
• High Risk
o Prosthetic valves
o Surgical shunts
o Indwelling catheters
o Previous SBE
• Moderate Risk
o PDA
o VSD
o ASD (not secondum)
o Bicuspid aortic valve
o RHD
o MVP with MR

41. Clinical Manifestations
•Fever
o High (Staph)
o Low (Strep)
• “Viral syndrome”
•New murmur
•CHF
•Petechiae
•Inc ESR, anemia, hematuria

44. Diagnosis
•Blood Culture
o Positive off antibiotics
o 5-8% negative cultures
o 2-3 sets over 24 hrs; (as much as possible)
•ECHO:
o Vegitations
o Valve insufficiency

45. Treatment
•Specific anti-microbial
•4-6 weeks IV
•2 weeks w/wo P.O.
•Surgery, esp. prosthetic valves

46. Prophylaxis
•AHA guidelines
o Amoxicillin - oral, upper resp procedures
o Clindamycin (penicillin allergic)
o Amp and gent or vancomycin - GU or GI

48. Rheumatic Fever
•Most common cause of acquired heart dise
ase in children (5-15 y peak of 8 y)
•USA: 0.5-3.0/100,000 (1900: 100-200/100,
000)
•Post- infectious connective tissue response
in susceptible host
•Group A beta- hemolytic streptococcus infe
ction of the pharynx
•F/H of RHD and low socioecnomic status.

50. Pathophysiology
•1960 Kaplan and coworkers- show an antig
enic “similarity” between strep cell walls an
d myocardium.
•An autoimmune response to strep group A
with cross reaction to myocardium.

52. Jones Criteria
•Major
o Carditis: 40-50%
o Arthritis: 60-85%
o Chorea; 15%
o Erythema marginatum: 10%
o Subcutaneous nodules; 2-10%
•Minor
o Clinical: Arthralgia, fever and H/O RF or RHD
o Laboratory:Elevated ESR, C-reactive protein and Prolon
ged PR interval
•Must have evidence for strep infection (Inc ASO, +
ve culture or recent scarlet fever).

53. Arthritis
•Most common manifestation
•Monoarticular, usually large joints
•Migratory or Fleeting
•Good response to ASA
•No residual effect

54. Carditis
•1-2 weeks after Strep; may be delayed
•Inflammation of:
o Endocardium: Valves
o Myocardium (Tachycardia,cardiomegally and H
eart failure).
o Pericardium: Rub or PE ( rare)
•Prior attack predisposes to recurrence
•The only feature which cause permanent d
amage.

55. Valvular Involvement
•Mitral
o Insufficiency; mild to severe (Carey-Coombs)
o Congestive heart failure
o Stenosis, late
•Aortic
o Insufficiency
o Less common but more severe

56. Chorea
•Sydenham’s chorea or St. Vitus’ dance
•Prepubertal girls (8-12y)
•First emotional lability and personality cha
nges
•Followed by loss of motor coordination - ch
aracteristic spontaneous, purposeless mov
ement and motor weakness
•It is often an isolated manifestation.

57. Erythema Marginatum
•Nonpruritic serpiginous or annular erythem
atous rashes.
•Most prominent on the trunk and inner pro
ximal portions of the extremities.

59. Subcutaneous Nodules
•Hard, painless, nonpruritic, freely movable,
swelling, 0.2-2.0 cm in diameter.
•Symmetrical, single or clusters
•On the extensor surfaces of both large and
small joints, scalp or along the spine.

61. Investigations
•Elevated ESR
•ASO > 333 Todd units
•Throat culture
•Leukocytosis
•Hypochromic microcytic anemia
•ECG: first degree heart block, arrhythmia.
•CXR: progressive cardiac enlargement.
•ECHO.

62. Treatment
•Cardiac supportive
o Bed rest 1-2 W
o Immobilise inflammed joints
o ASA 100 mg/kg/d (level 20 mg/100 ml) - side eff
ects (after diagnosis of RF is made)
o Benz Penicillin G 0.6-1.2 million U for eradicatio
n
o Steroids Prednisone (severe carditis) 2 mg/kg /d
2-4 W ???
o Treatment of CHF- Digoxin toxisity

63. Prevention
•Any pt with documented H/O RF
•Prophylaxis after attack: until 21-25y of age
•Benzathine Penicillin 1.2 million U IM q 28
d. or Erythromycin 250 mg BID for penicillin
allergics