Cardiovascular Flashcards
> 5 years
Male predominance
Dreaded Carditis
Chronic valvulopathy
RF/RHD
<5 years
Dreaded vasculitis, coronary aneurysm and thrombosis
Kawasaki disease
Systemic vasculitis
Inflammation of small to medium-sized arteries with resulting aneurysm
Febrile mucocutaneous illness of childhood
Fever and rash
Kawasaki disease
Leading cause of acquired heart disease in children in developing nations
Rheumatic fever/ RHD
Most important manifestation of Kawasaki
Aneurysmal involvement of coronary arteries
Peak mortality 15-45 days after onset of fever with mortality rate ranging 0.08-3.7%
Greatest risk of cardiac involvement in Kawasaki occur in
<1 year
Atypical
Etiology of Kawasaki
Infection
Superantigen-driven response (genetic predisposition -> oligoclonal conventional antigen vs polyclonal)
Increased production of cytokines and MMP
Kawasaki Phases
Early - first day of high fever, swelling of endothelium and edema of media, intact IEL
SubAcute (7-9d) - influx of neutrophils, CD8+, cytotoxic lymphocytes and IgA producing plasma (IEL fragmentation, vascular damage, dilatation, aneurysm)
Convalescent (>10d) increase cytokines, IL, MMP (fibrosis)
IV IG in Kawasaki is best given between
6th-10 days of illness because of inflammation
Kawasaki Disease criteria
Fever >5 days and
4/5 Main clinical features after exclusion
or
> 4 principal criteria at day 4 of fever
Principal features of Kawasaki
Acute: Changes in extremities (erythema of palms, soles; edema of hands)
Subacute: periungal peeling of fingers, toes in weeks 2 and 3
Polymorphous exanthem
Bilateral bulbar conjunctival injection without exudates
Changes in lips and oral cavity: erythema, lips cracking, strawberry tongue, diffuse injection of oral and pharyngeal mucosa
Cervical lymphadenopathy 1.5 cm unilateral
Patients with fever of at least 5 days and <4 principal criteria can be diagnosed with KD when
coronary artery abnormalities are detected by 2D ECHO or angiography
If patients have at least 3/5 but present with this symptom, KD is disgnosed
Coronary artery disease
High grade fever with or without other manifestations (arthritis, myocarditis)
Lips and mucocutaneous changes
Cervical adenitis
Normal CBC
Acute phase
first 10 days
Thrombocytosis Desquamation of skin and palms Beau’s lines With or without aneurysm Coronary thrombosis Mitral insufficiency
Subacute phase
11-22 days p
Cause of death in acute phase of KD
Myocarditis
Cause of death in subacute phase of KD
MI
Rupture of aneurysm
Myocarditis
May still be with Thrombocytopenia
Arthritis
Coronary and peripheral aneurysm
Convalescent phase
Cause of death: MI, IHD
Angina pectoris
MI
Chronic phase
Fever responds to this in KD
IVIG
Polymorphic exanthem on perineum mistaken for diaper rash
Dry erythematous, fissured, swollen and cracking lips
Strawbery tongue
Painful erythema and edema of extremity
Bilateral non exudative conjunctivitis
Unilateral Non-suppurative Cervical Lymphadenopathy >1.5 cm o
Fever >5 d
4/5
Kawasaki disease
Valvular regurgitation because of ischemia of papillary muscles in KD occurs in
Mitral
Aortic
Kawasaki disease Tx
IVIG (modulate cytokines, neutralization of bacterial super antigens)
Aspirin
Dipyrimadole if viral infection or hypersensitive (PDE 3 inhibitor antiplatelet)
Peaks 11-15 years
Inflammatory process mediated by immunologic reaction initiated by GABHS
Rheumatic fever