Acquired Heart Disease

Mohammed Alghamdi, MD, FRCPC (peds), FRCPC (card), FAAP, FACCAssistant Professor and Consultant

Pediatric Cardiology, Cardiac ScienceKing Fahad Cardiac Centre

King Saud University

Acquired Heart Disease

• Rheumatic Fever – Most common• 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.

• It called : Rheumatic heart disease (RHD) if the inflammatory changes involve the 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 + sub-clinical infection leading to acute rheumatic fever, and if it is not recognized rheumatic heart disease.

Jones criteria (1992) حفظ مهمهMajor Manifestations Minor ManifestationsCarditis ArthralgiaPolyarthritis FeverChorea Raised ERS or CRPErythema marginatum Prolonged PR interval on ECGSubcutaneous nodules

Evidence of antecedent GAS infection1. Positive throat culture or rapid antigen test for GAS2. Raised or rising streptococcal antibody titre

Rheumatic Fever

• Two majors or one major and two minors plus evidence of antecedent GAS infection مهمه. 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

Jones criteria (1992)

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. مهمه – 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 mostly affect

the elbow– Appear 1-2 weeks after onset of other symptoms– last 1-2 weeks (rarely > 1 month)– Strongly associated with carditis– It is specific to RF but not sensitive

Rheumatic Fever

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

Secondary ProphylaxisANTIBIOTIC DOSE ROUTE FREQUENCYFirst line

Benzathine penicillin G(BPG)

1,200,000 U ≥ 20kg

600,000 U < 20kg

IMBest

route مهمه

Q4 wks ORQ3 wks if confirmed

recurrent ARF despite adherence to

4 wks injection

Second linePenicillin V 250mg Oral BID

Penicillin allergy 0.2% only stop penicillin if patient has anaphylactic shock

Erythromycin 40mg/kg/day (children)

Oral BID –QID

400mg (adults) Oral BID

Rheumatic Fever Secondary prophylaxis

Duration of Secondary ProphylaxisCATEGORY 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) محل مهمه اسئله

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 carditis

Min of 10 years after most recent episode ARF OR until age 30 years (whichever is longer)May need life long prophylaxis

Rheumatic Fever

• 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

Nb : this Dz followed mainly by cardiologist with association of rumatologist

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). 1 Conjunctivitis: bilateral, non-purulent2 Skin Rash: polymorphous skin rash3 Mucous membrane changes: red, dry, and cracked lips,

strawberry tongue4 Extremities changes: palms/soles erythema, hands/ feet

edema, Skin peeling5 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

Nb : better treat As complete to avoid complications (( coronary arteries complications ))

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

– 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:

• > 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:– 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 EndocarditisSBE 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:– 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

• 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:

• 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%)