Transcript Acquired Heart Disease

Acquired Heart Disease
Mohammed Alghamdi, MD, FRCPC (peds), FRCPC (card), FAAP, FACC
Assistant Professor and Consultant
Pediatric Cardiology, Cardiac Science
King Fahad Cardiac Centre
King Saud University
Acquired Heart Disease
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Rheumatic Fever
Kawasaki Disease
Infective Endocarditis
Pericarditis
Myocarditis
Cardiomyopathy
Cardiac Arrhythmias: Any case scenario of heart failure
and a heart rate more than 200 is arrhythmia until proven
other wise. The commonest arrhythmia in children is SVT.
Rheumatic Fever
• RF: most common cause of acquired heart
disease in developing countries.
– 150 in 100,000 in developing countries (more
common in developing)
– 1 in 100,000 in developed countries.
• Rheumatic heart disease (RHD) inflammatory
changes to cardiac valves and myocardium.
• RF is the commonest acquired heart disease in
developing countries while Kawasaki is the
commonest in developed countries.
Rheumatic Fever
• Precipitated by Group A Streptococcal (GAS) pharyngitis
(not other types of GAS infections)
• Acquiring the disease depends on the host susceptibility
which depends on genetic, environmental,
socioeconomic and immunological status.
• 2-4 weeks after untreated GAS Pharyngitis
• T-cell and B-cell lymphocytes produce antibodies against
some Streptococcal antigens that cross-react with
antigens on myocytes or cardiac valve tissue.
• RF no heart sequale unlike RHD. RHD will lead to
rheumatic fever which will lead to well established
disease.
• It is not due to the infection itself rather due
to cross-reactivity between myocytes and
bacterial antigens.
• Not from one infection but clinical + subclinical infection leading to acute rheumatic
fever, and if it is not recognized rheumatic
heart disease.
Rheumatic Fever
Jones criteria (1992)
Major Manifestations
Minor Manifestations
Carditis
Polyarthritis
Chorea
Erythema marginatum
Subcutaneous nodules
Arthralgia
Fever
Raised ERS or CRP
Prolonged PR interval on ECG
Evidence of antecedent GAS infection
1. Positive throat culture or rapid antigen test for GAS
2. Raised or rising streptococcal antibody titre
Rheumatic Fever
Jones criteria (1992)
• Two majors or one major and two minors plus
evidence of antecedent GAS infection. ASO titer
may be –ve; repeat in 2-4 weeks if there is a high
index of suspicion.
• Exceptions:
– Chorea & indolent carditis do NOT require evidence of
antecedent GAS infection
– Recurrent episode requires :
• Only one major OR
• Several minor manifestations
• Plus evidence GAS infection
Rheumatic Fever
• Arthritis:
– Most common ARF presentation (75% of first attacks)
– Migratory, Asymmetrical, Polyarthritis, of large joints
• Typically: extremely painful
• Highly responsive to salicylate and NSAID therapy
– Within 3 days
• Monoarthritis if history of early use of NSAID
– Premature abortion the polyarthritis manifestation.
– You can’t give aspirin if patient has already started on NSAID
Rheumatic Fever
• Cardiatis:
– Valvulitis:
• Isolated MV is the commonest followed by MV plus AV
then AV, and rarely affects the right side.
– Early disease leads to valvular regurgitation,
– Prolonged or recurrent attacks lead stenosis
– Pericarditis and myocarditis may occur
– RHD is less rare in children less than 5 ears
because it requires recurrent infection.
Rheumatic Fever
• Sydenham’s chorea
– Jerky, uncoordinated movements esp. of hands, feet,
tongue and face.
• Disappear during sleep
• More common in adolescence female
– Occur after a prolonged latent period following GAS
• 6 wks - 3 yrs following GAS infection
– Strong association with carditis
– In sydenham chorea, it’s RHD until proven otherwise.
Because it can happen 3 years post-GAS where it’s hard
to find any evidence of strep infection. Therefore,
anyone with chorea should do echo.
– Usually seen in people who have beautiful handwriting
and suddenly drops.
Rheumatic Fever
• Erythema marginatum
– Rare & difficult to detect esp. in dark-skinned
patients
– Circular patterns of bright pink macules or papules
that blanch under pressure
• Trunk and proximal extremities
• Almost never on face
• Not itchy or painful vs eczema
Rheumatic Fever
• Subcutaneous nodules
– Rare < 2% of cases,
– Highly specific manifestation of ARF but it is not
sensitive.
– Round, firm, mobile and painless nodules
– Appear 1-2 weeks after onset of other symptoms
– last 1-2 weeks (rarely > 1 month)
– Strongly associated with carditis
Rheumatic Fever
• Treatment:
– Antibiotics to eradicate of residual GAS bacteria
– Anti-inflammatory agents
• High dose aspirin or NSAIDs.
• Steroids for severe carditis
– CHF therapy as indicated
– Primary prevention by preventing the infection from
happening, secondary prevention to prevent repeated
infection (because RHD does it occur from the first
attack it needs several episodes to occur.
– Sydenham Chorea:
• Haloperidol
Rheumatic Fever Secondary prophylaxis
DOSEProphylaxis
ROUTE
FREQUENCY
Secondary
ANTIBIOTIC
First line
Benzathine penicillin G
(BPG)
1,200,000 U ≥
20kg
600,000 U < 20kg
IM
Q4 wks OR
Q3 wks if confirmed
recurrent ARF despite
adherence to
4 wks injection
250mg
Oral
BID
40mg/kg/day
(children)
Oral
BID –QID
400mg (adults)
Oral
BID
Second line
Penicillin V
Penicillin allergy 0.2%
only stop penicillin if
patient has
anaphylactic shock
Erythromycin
Rheumatic Fever
Duration of Secondary Prophylaxis
CATEGORY
DURATION OF PROPHYLAXIS
ARF with no or mild
carditis
Min of 10 years after most recent
episode ARF OR until age 21 years
(whichever is longer)
ARF with moderate
carditis
Min of 10 years after most recent
episode ARF OR until age 30 years
(whichever is longer)
with ARF with severe Min of 10 years after most recent
carditis
episode ARF OR until age 30 years
(whichever is longer)
May need life long prophylaxis
• Damage to the heart is permanent whereas
the arthritis.
Kawasaki Disease
• KD: inflammatory disease of unknown etiology
causing acute, diffuse vasculitis of medium
size blood vessels (mainly coronary arteries).
• Most common cause of acquired heart
diseases in developed countries.
– More common in Japanese population
– Higher in males than females
– Typical age: 6 months - 5 years
Kawasaki Disease
• Cardiac Involvement:
– Coronary arteries( late): if left untreated the risk of
CAD is 25%, this is reduced to 2% with treatment.
• Dilation and aneurysm formation
• Thrombus formation
• Fibrosis and stenosis
• Myocardial infarction.
– May cause myocarditis and endocarditis
• Other medium size arteries
– Axiliary, femoral, iliac, and renal arteries.
Kawasaki Disease
• Fever: at least 5 days duration
• At least 4 of the following (to call it complete
typical KD).
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Conjunctivitis: bilateral, non-purulent
Skin Rash: polymorphous skin rash
Mucous membrane changes: red, dry, and cracked
lips, strawberry tongue
Extremities changes: palms/soles erythema, hands/
feet edema, Skin peeling
Cervical lymphadenopathy: unilateral and >1.5 cm
in diameter
Kawasaki Disease
• DDX of KD:
– Scarlet fever
– EBV infection
– Adenovirus infection
– Staphylococcal scalded skin syndrome
– Drug reactions
– Stevens–Johnson syndrome.
Kawasaki Disease
• Atypical (incomplete) KD:
– Cases of KD that do not fulfill diagnostic criteria.
– More common in infants
– Children with
• Fever greater than 5 days
• Two or three classic symptoms
• Supporting laboratory abnormalities or echo evidence
of coronary involvements
– Should be treated as KD
Kawasaki Disease
• Labs abnormalities in KD:
– Elevated ESR > 50 (70%)
– Elevated C-reactive protein (50%)
– CBC:
• Neutropenia , leukocytosis (50%)
• Nonspecific anemia
• Thrombocytosis more than 500,000 is very pathagnomonic.
– Marker of KD
– Not seen until 2nd week of the disease
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Elevated liver transaminases (40%)
Low serum albumin level
Sterile pyuria (33%)
Aseptic meningitis (up to 50%).
Kawasaki Disease
• Treatments:
– IVIG 2 g/kg
• 2nd dose if persistent fever within 48 h of the initial dose
– High dose of aspirin of 30–100 mg/kg/day.
• Once afebrile: aspirin is decreased to 3–5 mg/kg/day
• Aspirin acts as an anti-inflammatory agent if give in high doses for
long time, analgesic if given in medium doses when needed and
antiplatelet if given in small doses for long time.
– Echo at base line
– Repeat echocardiogram and inflammatory markers at 6–8 weeks
• If normal coronary arteries and inflammatory markers : aspirin can
be stopped
• If coronary artery abnormalities: long-term Rx with aspirin
– Other anticoagulants warfrin if giant aneurysm of coronary arteries
Infective Endocarditis
• infection of the endocardial lining of the heart
or cardiac vessels
• Rare but with high mortality
• Usually affect abnormal cardiac structure
– Valvular disease
– Septal defects
– Presence of foreign material such as mechanical
valves and patch material after surgical repair.
Infective Endocarditis
• Etiology:
– Gram- positive bacteria:
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> 90 % 0f bacterial case
Streptococci Viridans : most common infectious agent
Staphylococcal species – especially prosthetic valves.
Enterococci : less common pediatric age group.
– Gram-negative bacteria:
• < 10% of bacterial cases
• Example: HACEK group
– Haemophilus, Actinobacillus, Cardiobacterium, Eikenella and Kingella
– Fungal – uncommon
• Neonate, immunocompromised patients, prolonged antibiotic
– 10% IE case: organisms cannot be identified.
Infective Endocarditis
• Clinical Features:
– General:
• Fever
• Nonspecific manifestations
– myalgias, arthralgias, headache, and general malaise
– Cardiac:
• New onset or worsening valvular regurgitation
• Congestive heart failure
• Heart block
Infective Endocarditis
• Clinical Features:
– Extracardiac - septic embloism: ischemic changes can occur
anywhere even in the brain causing stroke.
• CNS: infraction, brain abscess
• Renal: proteinuria, hematuria, pyuria
• PHx - Stigmata of SBE:
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Janeway lesions:Non-tender, flat lesions on palms and soles.
Osler’s nodes: Tender, raised nodules on fingers and toes.
Roth’s spots: Exudative lesions of the retina.
Splinter hemorrhages: Non-blanching, linear, under nails.
Splenomegaly
Infective Endocarditis
• Labs:
– Blood cultures are the most important lab test
• Three blood cultures collected over a 24-h period from
different sites at different times. Because if only one of
the cultures is positive it could be contamination.
– CBC:
• Anemia
– Elevated ESR/CRP
– Positive rheumatoid factor
Infective Endocarditis
• Treatment:
– Supportive medical therapy
– Prolonged antibiotic therapy
• Initially empiric broad spectrum Abx which then
adjusted according to organism sensitivity. Admit for I.V
Abx with a combination of two or three drugs.
• 4-8 weeks course depending on the type of organism
and resistance.
– Removal of infected line
– Surgical intervention: may be required
Infective Endocarditis
• SBE prophylaxis:
– Only indicated at dental procedures or respiratory
procedure (not for gynecological procedures
anymore) for high risk patients
– No longer recommended for GI or GU procedures
– Prophylaxis regimens:
• Oral amoxicillin 50 mg/kg (up to 2 g) OR
• IV/IM ampicillin 50 mg/kg
• Patients allergic to penicillin
– Cephalexin 50 mg/kg PO (up to 2 g) OR
– Clindamycin 20 mg/kg (up to 600 mg) OR
– Azithromycin (or clarithromycin) 15 mg/kg (up to 500 mg)
Infective Endocarditis
SBE prophylaxis
Prosthetic cardiac valve
Previous IE
Congenital heart disease
• Unrepaired cyanotic CHD, including those with palliative
shunts and conduits
• Repaired CHD with prosthetic material or device (surgery or
cath), during the first 6 months after the procedure
• Repaired CHD with residual defects at the site or adjacent to
the site of a prosthetic patch or prosthetic device
Cardiac transplantation recipients with cardiac valvulopathy
Myocarditis
• Inflammatory infiltrate of the myocardium with
necrosis/degeneration of the myocytes
• Viral infections: most common causes in order:
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Coxsackievirus type B
Adenovirus
Parvovirus B19
Others: CMV, EBV, HIV, Hep C
• Other infectious causes: bacteria, rickettsiae, protozoa.
• Non-infectious causes:
– Rheumatologic disease: SLE, rheumatoid arthritis
– Drugs and Toxins: chemotherapy
Myocarditis
• Variable initial presentation
• Viral prodrome: fever, URTI or GI symptoms
precede the S/S of HF by 3 weeks.
• Hx: lethargy, poor feeding, irritability, respiratory
distress, exercise intolerance
• PHx:
– Signs of CHF
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Tachycardia
Tachypnia (increase work of breathing)
Gallop rhythm and murmur of mitral regurgitation
Hepatomegaly
Myocarditis
• Investigation:
– CXR:
• Cardiomegaly
• Pulmonary congestion
– ECG:
• Sinus tachycardia
• Low voltage QRS
• Non-specific T wave changes
– Echo:
• Dilated LV with reduced systolic function
• MR
• Pericardial effusion
Myocarditis
• Investigation:
– Myocardial biopsy:
• Historically used to be the gold standard test
• Currently not routinely performed
– Invasive
– low sensitivity of the procedure (3–63%)
» patchy involvement of the myocardium
• Treatment:
– Cardio-respiratory Support:
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Inotropes, Ventilation, ECMO
Anti-CHF therapy
IVIG and Steroid – not supporting evidence for their benefit
Antiviral agents and interferon – need further studies.
Cardiomyopathy
• Cardiomyopathy: myocardial disease resulting in thickening
of myocardial fibers or fibrosis.
• Pediatric cardiomyopathy: almost exclusively non- ischemic
• Types:
– Dilated cardiomyopathy (58%) the commonest, must rule out
arrhythmia.
– Hypertrophic cardiomyopathy (30%) syncope while exercising
might lead to sudden cardiac death in athletics because of
ventricular tachycardia progressing to ventricular fibrillation.
– Restrictive cardiomyopathy (5%)
– Arrhythmogenic Right Ventricular Cardiomyopathy ARVC (5%)