Pediatric Acquired heart Disease Flashcards
Kawasaki Disease
Acute, self-limited febrile illness of unknown cause that predominantly affects children <5 years of age
Clinical diagnosis of KD - Warm CREAM
fever
c= conjunctivitis
R= rash
E= edema- on palms and soles
A= adenopathy
M = mucosal changes– strawberry tongue, red and fissured lips
****L = lymphadenopathy – unilateral and LARGE
pathogenesis of KD
can be caused by an initial infectious.
- abnormal immune response is provoked in a genetically susceptible host, resulting in microvasculitis, panarteritis, destructive changes and aneurysms
note- basculitis is a common but non-specific symptosm and signs
in KD, 30-50% of patients show diffusely enlarged ___ ___ in the acute phase.
-without tx, aneurysms develop within 1-3 weeks. Aneurysms may thrombose or become stenotic, resulting in risk of :
coronary artery enlargemend. higher sik for sudden death, anigina, MI and arrythmia
2 key treatments for kawasaki’s disease
- IVIG- high dose to reduce the incidence of aneurysms
- aspirin; anti-inflammatory, antipyretic and antiplatelet effects to prevent sudden thrombosis or stenosis
- – high-dose until afebrile for 48 h – low-dose for 4-6 weeks – longer for coronary dilation/aneurysm
- might include LMWH or warfarin if there is coronary dilation/aneurysm/need for longer term amangement.
natural history of the aneursyms in KD
- around 50% of aneurysms regress with treatment– but likely abnormal vascular function
- higest risk: giant aneurysms >8 mm. thrombosis, stenosis, pediatric myocardial infarction can happen
- myocardial dysfunction, valve regurgiation, aortic abnormalities.
most common cause of acquired pediatric heart disease
acute rheymatic fever. disease of childhood. more common developing country
most common bacteria cause of acute rheumatic fever
groupA strep (strep pyogenes) most common.
major criteris (jones) to dx rheumatic fever
Major Criteria
• Carditis • Chorea • Polyarthritis • Erythema marginatum • Subcutaneous nodules
minor criteria for acute rheumatic fever
Minor Criteria
• Fever • Arthralgia • Elevated acute-phase
reactants
– ESR, CRP • Prolonged PR interval
- primary RF episode = 2 major, or 1 major and 2 minor
- if a population is at high risk for ARF– consider monoarthritis as a major criteria and monoarthralgia as a minor criteria
acute rheumatic fever can cause carditis. what valve is most affected bt ARF
- mitral regurgitation )or mitral stenosis in adults)
2 . aortic regurgitation
- can cause anywhere from a murmur to critical heart failure.
treatment of ARF
- antibiotic treatment– consider penicillin
- arthritis management with NSAIDs
- carditis management (valve treatment)
- secondary prophylaxis and education
Rheumatic heart disease is a common consequence of ARF. it’s more long lasting than ARF.
2 valves affected by RHD
- chronic mitral regurgitation ( mitral stenosis in older adults)
- chronic aortic regurgitation
- more severe with recurrent episodes of ARF