Acquired Heart Disease in Children

Jill Narron, M.D. Clinical Assistant Professor of Pediatrics Boonshoft School of Medicine Wright State University

Acute Rheumatic Fever

Most common cause of acquired heart disease in children worldwide
Estimated that ARF and RHD affect nearly 20 million people in developing countries worldwide Leading cause of CV death in first 5 decades of life

Localized outbreaks in US in mid-1980s Caused by untreated Group A -hemolytic streptococcal pharyngitis in susceptible host
Occurs ~ 3 weeks after asymptomatic latency period History of preceding sore throat may not be evident

Epidemiology
Usually seen in school age children (age 5-15 years) and rare before age 5 in U.S. Also seen in at-risk adults
Military recruits Parents of school-age children in crowded housing

Male=Female in incidence Peak occurrence in spring in U.S. All socioeconomic groups affected

Pathogenesis: Current Theory

Group A streptococcus Susceptible Host

Tissue/Organ Inflammation

Immune Reaction

ACUTE RHEUMA TIC FEVER

The Jones Criteria

Major Migratory polyarthritis Carditis Sydenhams chorea Subcutaneous nodules E. marginatum Minor Elevated ESR Increased PR interval Fever Arthralgia Prior history of ARF

Plus.Evidence of a previous strep infection!

Cardiac Pathology
Endocarditis
Valve insufficiency initially Mitral valve most commonly affected followed by aortic valve If untreated, leads to stenosis

Cardiac Pathology
Myocarditis
Poor function Aschoff body

Pancarditis
Addition of pericarditis Pericarditis RARE without endocarditis or myocarditis

More Major Manifestations

Erythema marginatum
Subcutaneous nodules

From: www.thesynpase.net./impaedcard/issue/issue11/1231/1231.htm

Frequency of Major Manifestations

Polyarthritis in 40-70% (MOST COMMON) Carditis in 30-70% Chorea (St. Vitus Dance) in 10-30% Erythema marginatum and subcutaneous nodules in < 5% and rarely seen without carditis

Acute Rheumatic Fever

Laboratory Findings

Elevated CRP or ESR + Throat Culture Elevated WBC Hematuria Elevated ASO, or Anti-DNase B, or antiStreptokinase, or antihyaluronidase

90% 33% 80% 6% 95%

Treatment
Antibiotics to eradicate Streptococcus from the oropharynx (primary prevention) Treatment for: -Carditis -Arthritis -Sydenhams chorea Antibiotic prophylaxis against recurrence (secondary prevention)

Antibiotic Regimens
Primary Prevention
Preferred agents: -Benzathine penicillin G (IM) x 1 dose -Penicillin V (PO) x 10 days -Amoxicillin (PO) x 10 days In PCN-allergic patients: -Narrow spectrum cephalosporin (PO) x 10 days -Clindamycin (PO) x 10 days -Azithromycin (PO) x 5 days -Clarithromycin (PO) x 10 days

Treatment of Arthritis
Aspirin at 50-75 mg/kg/day for 2 weeks Gradual taper depending on patients clinical course Acute phase reactants (ESR, CRP) used to assist in tapering of salicylate therapy Steroids are only used with moderate to severe carditis

Treatment of Carditis
Mild
-ASA 90-100 mg/kg/day x 4-8 weeks -T aper based on clinical status, ESR, CRP

Moderate (Cardiomegaly, Pericarditis)

-Bedrest -Prednisone 1 mg/kg/day x 7 10 days - ASA before steroids are withdrawn

Severe (Congestive heart failure)

-Bedrest -Prednisone 4 6 weeks (dont forget to taper!!) -ASA before steroids are withdrawn -Digoxin, diuretics, afterload reducing agents, etc.

Treatment for Chorea

Usually self-limited and resolves within weeks-months

For severe symptoms: -Haloperidol -Phenobarbital

Dont forget to look for carditis in children presenting with chorea. Up to 20% will also have heart disease even though the major clinical picture is chorea!

Antibiotic Regimens
Secondary Prevention
Required after ALL cases of RF Preferred agents: -Benzathine penicillin G (IM) every 4 weeks** -Penicillin V (PO) twice daily -Sulfadiazine (PO) once daily In PCN and sulfa-allergic patients: -Macrolide or azalide (PO) If patients are compliant, prevention lowers attack rate to ~1%
** Best Regimen!**

Antibiotic Regimens
Length of prophylaxis depends on presence of carditis with initial episode & residual valve disease -NO carditis: at least 5 years or until age 21 -WITH carditis but no residual valve disease: at least 10 years or until age 21 -WITH residual valve disease: at least 10 years and at least until age 40, sometimes lifelong Endocarditis (SBE) prophylaxis is required for patients s/p valve replacement using a different antibiotic from that prescribed for RF prophylaxis

Infective Endocarditis
Defined as microbial infection of endothelial surface of heart Native or prosthetic valves most frequently involved Septal defects also involved along with intravascular foreign devices (patches, surgical shunts, IV catheters) Previously occurred with underlying rheumatic heart disease

Epidemiology
Increased incidence now likely related to:
Increased survival of CHD patients Aggressive management of ICU patients

17% of cases are in children with no previously known heart disease 43% of pediatric cases are in postoperative patients Most common underlying conditions: Tetralogy of Fallot, VSD, Aortic stenosis

Pathogenesis
Pre-existing congenital or acquired lesion USUAL Indwelling catheters also possible Vegetations usually occur where there is pressure gradient causing turbulent blood flow Damage to endothelium -> formation of nonbacterial thrombotic endocarditis (NBTE) -> occurrence of transient bacteremia -> adherence of bacteria to NTBE -> proliferation of bacteria within vegetation

Infective Endocarditis
Etiologic Agents in Children
Alpha-hemolytic strep* Staphylococcus aureus* Beta-hemolytic Strep Coagulase negative Staph Candida Most Common Second Most Common

Uncommon Uncommon (increasing) Uncommon RARE RARE RARE

Enterococci Pneumococci Gram negatives

*Propensity to adhere to valve tissue

Clinical Findings
Fever Nonspecific symptoms (HA, myalgia, arthralgia, malaise) Murmur (new or changing) Heart failure Petechiae Embolic phenomena Splenomegaly Neurologic findings Osler nodes, Janeway lesions, Roth spots, Splinter hemorrhages
Neonates may have few specific symptoms

++++ +++ ++ ++ ++ ++ ++ ++ +

Clinical Manifestations
Osler nodes
Splinter hemorrhage

Janeway lesions Conjunctival petechiae

From: NEJM 345(10), 739:2001

Clinical Manifestations

From: www.nlm.nih.gov/medlineplus/ency/imagepages/18132.htm

www.emedicine.com/ped/images/727800bavendo1.jpg

Lab Findings
Positive blood culture (off abx) ++++ Elevated acute phase reactants++++ Anemia +++ Hematuria +++ (+) Rheumatoid factor ++ Leukocytosis ++

Therapy
Bactericidal IV antibiotics (typically 4-6 weeks; sometimes longer with prosthetic valves) Surgical intervention more likely with:
Significant embolic events Persistent infection Progressive cardiac failure Prosthetic valve

Fungal endocarditis poor prognosis

Prevention (SBE Prophylaxis)

Most recent AHA recommendations published in May 2007 Conclusion that bacteremia from routine daily activities MORE LIKELY to cause IE than dental procedure Only cardiac conditions at risk for adverse outcome from IE should receive antibiotic prophylaxis Good oral and dental hygiene stressed

At Risk Conditions
Prosthetic cardiac valve Previous endocarditis Cardiac transplant recipients with valve disease CHD in following categories:
Unrepaired CYANOTIC CHD including those with palliative shunts/conduits Repaired CHD with prosthetic material or device within first 6 months after procedure Repaired CHD with residual defect at site or adjacent to site of prosthetic patch or device

Indicated Procedures
Dental work involving manipulation of gingival tissue or periapical region of teeth or perforation of oral mucosa Procedures on respiratory tract involving perforation of mucosa Procedures on infected skin, skin structures, or musculoskeletal tissue NO LONGER recommended for GI or GU procedures

Prevention (SBE Prophylaxis)

Antibiotic Regimens

Typically Amoxicillin 50 mg/kg (max dose 2 grams) one hour prior to procedure See AHA Card for complete details

Kawasaki Disease
Initially described by Dr. Kawasaki in Japan in 1967 First identified in U.S. in 1974 in Hawaii and now known to occur worldwide Has surpassed rheumatic fever as leading cause of acquired heart disease in U.S. children

Epidemiology
Highest rate in Asian population Age: mean 18-24 months; ~75% < 5 yo Male/female ratio=1.5:1 More common in winter and early spring No etiologic agent identified to date

Making the Diagnosis

Classic - Fever of 5 days duration and at least 4 of 5 principal criteria Fever and fewer than 4 other criteria are diagnostic when coronary artery abnormality identified In presence of 4 clinical criteria, AHA now states that diagnosis can be made on day 4 of fever Exclusion of other diagnoses

Principal Criteria
Conjunctivitis & inflamed oral mucosae

Principal Criteria
Strawberry tongue

Principal Criteria
Rash

Principal Criteria
Palm erythema & swelling

Principal Criteria
Periungual desquamation

Principal Criteria
Cervical lymphadenopathy

Associated Cardiac Manifestations

Leading cause of morbidity and mortality in KD Myocarditis, pericarditis, valvulitis, and coronary vasculitis ECG: sinus tachycardia, prolonged PR or QTc intervals, arrhythmias, decreased QRS voltages, abnormal Q waves, ST-T wave changes

Associated Cardiac Manifestations

Echo findings
Coronary aneurysms Pericardial effusion Mitral insufficiency Ventricular dysfunction

Associated Non-cardiac Manifestations

GI abdominal pain, diarrhea, vomiting, hepatic enlargement with jaundice, gallbladder hydrops (~15%) GU urethritis CNS irritability, transient sensorineural hearing loss M-S arthralgia, arthritis (~40%)

Associated Lab Findings

Leukocytosis with left shift Anemia Elevated ESR, C- reactive protein Thrombocytosis (500,000 to >1 million) during subacute phase Elevated transaminases (~40%) & GGT (67%) Hypoalbuminemia Sterile pyuria (~33%) Pleocytosis of CSF (~50%)

Higher Risk for Coronary Complications

Male gender < 1 yo or > 8 yo Prolonged &/or recurrent fever Other CV involvement (myocarditis, effusion, arrhythmia) Hypoalbuminemia, anemia Thrombocytopenia No or delayed therapy

Treatment
Aimed at reduction of inflammation and prevention of thrombosis by inhibiting platelet aggregation IV -globulin at 2 grams/kg/dose + Aspirin therapy at 80-100 mg/kg/day within 10 days of onset

Treatment
(Refractory Cases)
Retreatment with IVIG 2 grams/kg/dose indicated for persistent or recurrent fever 36 hours after 1st dose completed Steroids are controversial but may help in cases refractory to 2 doses of IVIG

Treatment
Reduce aspirin to 3-5 mg/kg/day (antiplatelet dose) once afebrile for 4872 hours or at day 14 of illness Discontinue aspirin 6-8 weeks after onset of illness if no coronary abnormalities identified Long-term aspirin therapy is indicated if coronary abnormalities present at 6-8 weeks

Case Presentation
4 year old presents with fever, chest pain, and shortness of breath PE: T emp 39C, HR 120, RR 60, JVD (increases with inspiration), BP 100/65 (80/66 with inspiration), muffled heart tones, rales, hepatomegaly, edema

Acute Pericarditis

From: www.learningradiology.com/images/../tn_pericardial%20effusion.jpg

From: www.med.nus.edu.sg/paed/medical_education/.../effusion.jgp

Acute Pericarditis
Symptoms
Precordial chest pain
In up to 80% in children Worse with coughing, breathing, or motion

Most comfortable in upright position Fever T achycardia out of proportion to degree of fever Respiratory distress- uncommon unless tamponade or pneumonitis also present

Acute Pericarditis
Signs of Effusion
Friction rub
Grating, scratching sound Best heard during inspiration with patient leaning forward

Ewarts sign
Subscapular dullness to percussion Represents compression of left lung by enlarged heart

Acute Pericarditis
Signs of Tamponade
Low cardiac output Elevated CVP Pulsus paradoxus: > 10 mmHg fall in SBP with inspiration Muffled or diminished heart sounds JVD increasing with inspiration (Kussmauls sign) Hepatomegaly, peripheral edema

Acute Pericarditis
Clinical Findings
ECG
PR depression ST elevation Low voltages in presence of large effusion

Chest x-ray
Cardiomegaly if effusion present Water bottle heart enlarged triangular heart with smoothed-out cardiac borders with massive effusion

Acute Pericarditis
Etiologies
Infectious Rheumatic/collagen vascular Drug therapy Cardiac surgery- post-pericardiotomy Renal failure Idiopathic up to 30% of cases (presumed to be viral)

Infectious Pericarditis
Bacterial
Life-threatening Mortality rates 25-75% May be primary or secondary to dissemination from another site (lung, brain, bone, joint) Staph aureus 50-80% of cases Also see H flu, Strep pneumo, and others Treatment
DRAINAGE Antibiotics for 3-4 weeks

Infectious Pericarditis
Viral
Occurs more often in children Preceding respiratory or GI illness in 40-75% of cases Coxsackie, Echovirus, Adenovirus, Influenza, EBV, Mumps, VZV, HIV Ill-appearing but not as toxic as in bacterial disease Friction rub in up to 80% of patients Treatment
Drainage if tamponade +/- pericardial drain Bedrest, salicylates or NSAIDs, rarely steroids

Infectious Pericarditis
Tuberculous
More common in underdeveloped countries Onset may be insidious (weight loss, night sweats, dyspnea, and chest pain) Mantoux test positive (place anergy panel in immunocompromised) Combination therapy required due to drug resistance

Non-Infectious Pericarditis
Rheumatic/Collagen Vascular
Acute rheumatic fever usually with pancarditis JRA symptomatic in 10% SLE -symptomatic in 25% Treatment: anti-inflammatory agents

Drug-induced
Hydralazine, isoniazid, procainamide Cause lupus-like syndrome Treatment: Stop drug; anti-inflammatory agents

Non-Infectious Pericarditis
Post-pericardiotomy syndrome
Occurs in up to 30% following cardiac surgery Symptoms: fever, chest pain, irritability, decreased appetite Treat with aspirin 50-75 mg/kg/day for 4-6 weeks Steroids (2 mg/kg/day) effective but increase risk of immunosuppresion

Uremic
Sign of end-stage renal disease Dialysis resolves most effusions Anti-inflammatory agents aid with chest pain and fever but do not resolve effusions

Quick Quiz!

Which valve is most commonly affected in acute rheumatic fever?

Quick Quiz!

Which valve is most commonly affected in acute rheumatic fever? Mitral valve

Quick Quiz!
What is the most common organism in infective endocarditis?

Quick Quiz!
What is the most common organism in infective endocarditis? lpha-hemolytic Strep

Quick Quiz!
What is the most common cause of acquired heart disease in U.S. children?

Quick Quiz!
What is the most common cause of acquired heart disease in U.S. children? Kawasaki Disease

Bonus Question!
Where did Dr. T. Duckett Jones graduate from medical school?

Bonus Question!
Where did Dr. T. Duckett Jones graduate from medical school? THE University of Virginia!