8. Rheumatic fever:
Acute rheumatic fever: inflammatory disease with
devastating sequelae
Link to pharyngeal infection with group A beta hemolytic
streptocci
Continues to be a problem worldwide:
- sporadic outbreaks in developed countries
- frequent occurrences in developing countries
Still gaining understanding of etiology
- link between genetic predisposition and clinical
manifestations
Best prevention still correct use of antibiotics
11. Pathogenesis
Group A strep pharyngeal infection precedes clinical
manifestations of ARF by 2 - 6 weeks
Antibodies made against group A strep cross-react
with human tissue
heart valve and brain share common antigenic
sequences
with GAS bacteria
theory of molecular mimicry
Host immune responses may play a role in
determining who gets ARF following infection
Virulent strains: rheumatogenic serotypes
13. Arthritis
Most common feature: present in 80% of
patients
Painful, migratory, short duration, excellent
response of salicylates
Usually >5 joints affected and large joints
preferred
Knees, ankles, wrists, elbows, shoulders
Small joints and cervical spine less
commonly involved
14. Subcutaneous Nodules
Usually 0.5 - 2 cm long
Firm, non-tender, isolated or in clusters
Most common: along extensor surfaces of
joint
Knees, elbows, wrists
Also: on bony prominences, tendons, dorsi
of feet, occiput or cervical spine
Last a few days only
Occur in 9 - 20% of cases
Often associated with carditis
15. Erythema Marginatum
Present in 7% of patients
Highly specific to ARF
Cutaneous lesion:
Reddish pink border
Pale center
Round or irregular shape
Often on trunk, abdomen, inner arms, or
thighs
Highly suggestive of carditis
16. Sydenham’s Chorea
Extrapyramidal disorder
Fast, clonic, involuntary movements
(especially face and
limbs)
Muscular hypotonus
Emotional lability
First sign: difficulty walking, talking, writing
Usually a late manifestation: months after
infection
Often the only manifestation of ARF
17. Carditis
Most serious manifestation
May lead to death in acute phase or at later stage
Any cardiac tissue may be affected
Valvular lesion most common: mitral and aortic
Seldom see isolated pericarditis or myocarditis
Mitral and aortic regurgitation most common
Apical systolic and basal diastolic murmurs
Pericarditis usually asymptomatic
Occasionally causes chest pain, friction rubs or distant
heart sounds
36. Diagnosis: Jones Criteria
•
•
•
•
•
Major criteria
Arthritis
Carditis
Sydenham’s chorea
Erythema
marginatum
Subcutaneous
nodues
Minor criteria
•Fever
•Arthralgia
•Elevated c-reactive
protein or
•Erythrocyte
sedimentation rate
•Prolonged PR
interval on EKG
37. CONGENITAL HEART
DEFECTS
Faulty embryogenesis (week 3-8)
Usually MONO-morphic (i.e., SINGLE
lesion) (ASD, VSD, hypo-RV, hypo-LV)
May not be evident until adult life
(Coarctation, ASD)
Overall incidence 1% of USA births
INCREASED simple early detection via
non invasive methods, e.g., US, MRI,
CT, etc.
38. Incidence per Million Live
Births
%
4482
42
1043
10
Pulmonary stenosis
836
8
Patent ductus arteriosus
781
7
Tetralogy of Fallot
577
5
Coarctation of aorta
492
5
Atrioventricular septal defect
Aortic stenosis
396
4
388
4
Transposition of great arteries
Truncus arteriosus
Total anomalous pulmonary venous connection
Tricuspid atresia
388
4
136
1
120
1
Malformation
Ventricular septal defect
Atrial septal defect
39. CONGENITAL HEART
DEFECTS
LR SHUNTS: all “D’s” in their names
NO cyanosis
Pulmonary hypertension
SIGNIFICANT pulmonary hypertension is
IRREVERSIBLE
RL SHUNTS: all “T’s” in their names
CYANOSIS
VENOUS EMBOLI become SYSTEMIC
OBSTRUCTIONS
42. ASD
NOT patent foramen ovale
Usually asymptomatic until
adulthood
SECUNDUM (90%): Defective fossa
ovalis
PRIMUM (5%): Next to AV valves,
mitral cleft
SINUS VENOSUS (5%): Next to
SVC with anomalous pulmonary
veins draining to SVC or RA
43. VSD
By far, most common CHD defect
Only 30% are isolated
Often with TETRALOGY of FALLOT
90% involve the membranous septum
If muscular septum is involved, likely to
have multiple holes
SMALL ones often close spontaneously
LARGE ones progress to pulmonary
hypertension
44.
45. PDA
90% isolated
HARSH, machinery-like murmur
LR, possibly RL as pulmonary
hypertension approaches systemic
pressure
Closing the defect may be life
saving
Keeping it open may be life saving
(Prostaglandin E). Why?
48. RL SHUNTS
TETRALOGY of FALLOT most COMMON
1) VSD, large
2) OBSTRUCTION to RV flow
3) Aorta OVERRIDES the VSD
4) RVH
SURVIVAL DEPENDS on SEVERITY of
SUBPULMONIC STENOSIS
Can be a “PINK” tetrology if pulmonic
obstruction is small, but the greater the obstruction,
the greater is the RL shunt
49.
50. TGA (TRANSPOSITION
of GREAT ARTERIES)
NEEDS a SHUNT for
survival
PDA or PFO (65%),
“unstable” shunt
VSD (35%), “stable”
shunt
RV>LV in thickness
Fatal in first few
months
Surgical “switching”
53. Total Anomalous Pulmonary
Venous Connection (TAPVC)
PULMONARY VEINS do NOT go
into LA, but into L. innominate
v. or coronary sinus
Needs a PFO or a VSD
HYPOPLASTIC LA
Notes de l'éditeur
A GREAT classical sydenham chorea (St. Vitus “Dance”) can be seen at www.youtube.com/watch?v=RnxqqW_nH0k
Do the NAMES of these congenital heart conditions adequately describe the pathology? Ans: YES
Why have I highlighted the “D”s and the “T”s? Ans: D = L shunt, T= RL shunt (cyanosis, or “blue” babies).
LEFT to RIGHT SHUNTS, NON-cyanotic
All the RL congenital shunts are CYANOTIC, and have T’s in their names.
CLASSICAL “TETROLOGY” of FALLOT:
1) VSD, large
2) OBSTRUCTION to RV flow
3) Aorta OVERRIDES the VSD
4) RVH
