Transcript CASE PRESENTATION

CASE PRESENTATION
Patricia Baile – PL1
December 16, 2009
16 year old male
presents to the ER
with chest pain
HPI
• Chest pain x 6 days
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Sharp, stabbing sensation on left side of chest
Constant
PS 7/10
Worse when lying on left side and on inspiration
Radiating to back
Mild improvement with aspirin and
acetaminophen
HPI
• Chest pain
▫ Associated with nausea, dizziness and blurring of
vision 2 days prior
▫ No diaphoresis
• On day of admission, chest pain persistent, no
improvement with acetaminophen hence BIB
EMS to ER
ROS
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Denies recent strenuous physical activity
No fever
No URI symptoms
No sick contacts
No joint pains
No dyspnea
Past Medical History
• Asthma
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9 previous hospitalizations, last 1 year prior
No ICU, no intubation
Advair BID
Singulair 10 mg PO QD
Albuterol PRN
Family History
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+ Asthma
+ DM
+ Hypertension
+ CAD
No sudden death
No CVA
No connective tissue disorders
• Birth History
▫ FT, NSVD, no complication
• Immunization History
▫ Up to date
• Adolescent History
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Currently in 9th grade (behind 2 years)
Lives with mother, stepfather and older brother
No sport or after-school activity
Denies drugs, alcohol, tobacco
Sexually active, has had 8 partners, uses condoms,
no STD history (never tested)
Physical Examination
• BP 102/58 HR 72 RR 18 O2 100%RA T 98.3
• Pain score: 6-7/10
• Wt: 76.9 kg (90%) Ht: 185 cm (90%)
• GS: non-toxic appearing
• HEENT: NCAT, congested turbinates, TMI, mild
erythema OP, no CLAD
• C/L: SCE, good air entry b/l, CTAB, no crackles, no
rales, no tenderness on palpation of chest
• CV: RRR, no m/r/g
• Abdomen: soft, ND, NT, no organomegaly
• Ext: well-perfused, good distal pulses
EKG
Labs
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CBC: 6.9 > 13.7/39.1< 251 N 68 L 20
BMP: 138/3.8/100/26/7/0.6/104/8.3
LFT: 3.9/6.9/25/109/0.7/74
Lipase: 12
CPK: 1751
CKMB: 168.8
Troponin T: 8.77
Labs
• Lipid profile
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Cholesterol: 106
TG: 49
HDL: 31
LDL: 65
• CRP: 7.5
• Urine Drug Screen: Negative
Labs
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RSV/Flu: Negative
Rapid strep test: negative
Throat culture
ASO: 165
Streptozyme: Positive
Respiratory viral panel: Negative
Ancillary Tests
• Chest Radiograph
▫ Normal chest
Ancillary Tests
• Initial Echo
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Normal LV ejection fraction
Normal RV function
Mild inferolateral akinesis
Inferior wall akinesis
Small pericardial effusion
Initial Impression
• 16 year old male with Juvenile pattern
pericarditis; Asthma exacerbation
Hospital Course
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Admitted to PICU
Cardiac enymes gradually decreased
Repeat Echo done
Cardiac catheterization done
Ancillary Tests
• Repeat Echo
▫ Myocarditis with LV inferior/posterior wall
motion abnormality: persistent abnormal LV wall
motion
▫ Suboptimal LV shortening fraction
▫ Normal diastolic LV function
▫ Trace inferior and posterior pericardial effusion
Ancillary Tests
• Cardiac catheterization
▫ Clear vessels
▫ Decreased ejection fraction
▫ Decreased motion of LV
FINAL DIAGNOSIS
• 16 year old male with chest pain secondary to
Myocarditis; Asthma exacerbation
MYOCARDITIS
Introduction
• Clinical syndrome characterized by
inflammation of myocytes resulting from
infectious, toxic, and autoimmune etiologies.
• Ongoing viral infection, myocardial destruction,
and adverse remodeling can lead to persistent
ventricular dysfunction and dilated
cardiomyopathy.
Introduction
• Infectious etiologies, particularly viral, are most
common in children.
• The most common causes of viral myocarditis
are enterovirus (coxsackie group B) and
adenovirus
Incidence
• Incidence of myocarditis in children is unknown
▫ Inflammatory infiltrates and myocardial cell
damage were found at autopsy in 3 to 40% of
infants and children who died suddenly unrelated
to trauma
▫ 17 percent of infants who died of SIDS had
histopathologic evidence of myocarditis
Incidence
• In one retrospective study from a single tertiary
Canadian center, the estimated prevalence of
myocarditis presenting to their emergency
department was 0.5 cases per 10,000 visits
• A review of all the autopsies performed at a
single English pediatric tertiary center over a
ten-year period (1996 to 2005) identified 28 of
1516 cases with myocarditis (1.8 percent)
Pathophysiology
• In susceptible patients:
▫ Viral RNA uptake  cytotoxic necrosis  rapid
cell death
• More common presentation
▫ 4-14 days post-infection  immune response
(macrophage activation and cytokine expression)
 natural killer cells target myocardium
expressing the viral RNA and continue myocyte
necrosis
Pathophysiology
▫ TNF is involved in rapidly clearing virus and
signals additional proinflammatory cells, activates
endothelial cells, and has direct negative inotropic
effects
▫ Cytotoxic T lymphocytes infiltrate myocytes and
trigger lysis of these cells
Pathophysiology
• In the chronic phases, the effects of either
inadequate or inappropriately abundant
immune response can lead to the long-term
sequelae of dilated cardiomyopathy and heart
failure
Pathophysiology
• Ongoing study has demonstrated the presence of
antimyosin autoantibodies and other
immunomodulators long after initial viral
infection
Clinical Manifestation
• Nonspecific illness
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Fatigue
Mild dyspnea
Myalgias
Fever (20%)
• Chest pain (35%)
▫ most commonly described as a pleuritic, sharp,
stabbing precordial pain
▫ may be substernal and squeezing
Clinical Manifestation
• In a 6-year study of pediatric ED patients, the
most common presenting symptom was dyspnea
and more than half of patients were initially
diagnosed with asthma or pneumonia.
• May be asymptomatic
Clinical manifestation
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Symptoms of heart failure
Dyspnea on exertion
Orthopnea
Shortness of breath
Palpitation
Physical Findings
• Tachypnea and retractions
• S3 and occasionally S4 gallops may be present
and are important signs of impaired ventricular
function
• If the right or left ventricular dilation is severe,
auscultation may reveal murmurs of functional
mitral or tricuspid insufficiency
Physical Findings
• Signs of low cardiac output
• Pericardial friction rub and effusion may become
evident in some patients with myopericarditis
• A widely inflamed heart shows the classic signs
of ventricular dysfunction including the
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Jugular venous distention
Bibasilar crackles
Ascites
Peripheral edema
Causes
• Infectious
• Toxic
• Immunologic
Infectious Causes
• Viral myocarditis is the most common
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Parvovirus B19, 36.6%
Enterovirus, 32.6%
Human herpesvirus 6 (HHV-6), 10.5%
Adenovirus, 8.1%
Co-infection with HHV-6 and parvovirus B19,
12.6%
• HIV
Infectious Causes
• Bacterial causes
▫ Most common worldwide is Diphtheria
▫ Streptococcal and staphylococcal species and
Bartonella, Brucella, Leptospira, and Salmonella
species can spread to the myocardium as a
consequence of severe cases of endocarditis
• Chagas disease
• Parasitic myocarditis from trypanosomiasis
Toxic Causes
• Numerous medications (eg, lithium,
doxorubicin, cocaine, numerous catecholamines,
acetaminophen) may exert a direct cytotoxic
effect on the heart
• Zidovudine (AZT) has been associated with
myocarditis
Toxic causes
• Environmental toxins include lead, arsenic, and
carbon monoxide
• Wasp and scorpion stings and spider bites,
specifically black widows, may cause myocarditis
• Radiation therapy
Immunologic Etiology
• Connective tissue disorders
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Systemic lupus erythematosus (SLE)
Rheumatoid arthritis
Scleroderma
Dermatomyositis
• Idiopathic inflammatory and infiltrative
disorders such as Kawasaki disease, sarcoidosis,
and giant cell arteritis may be a cause
Differential Diagnoses
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Acute Coronary Syndrome
Pneumonia
Congestive Heart Failure
Aortic Dissection
Pulmonary Embolism
Esophageal Perforation, Rupture and Tears
Viral syndrome
Diagnostic Studies
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EKG
CXR
Cardiac enzymes
Echo
MRI with contrast
Cardiac catheterization
Other tests
EKG
• Classical triad
▫ Sinus tachycardia (>100 in a child; >120 in an
infant; >150 in a neonate)
▫ Low voltage complexes
▫ ST segment and T wave changes
• Other abnormalities like, varying AV blocks,
bundle branch blocks, both supraventricular and
ventricular arrhythmias and an even an anterior
wall myocardial infarction pattern
Chest Radiograph
• Typically include cardiomegaly, although heart
size may be normal
• Pulmonary vascular congestion is often present
Cardiac Enzymes
• Elevation reflects myocardial necrosis
• Seen in some patients with myocarditis
• Experimental and clinical findings in adults
suggest that elevations of cardiac troponin I or T
(cTnI or cTnT) levels may be more common than
CK-MB elevations in patients with biopsyproven myocarditis
Echocardiography
• Enlarged Left Ventricular (LV) dimensions, left
atrial enlargement and impaired ejection
fraction (EF) and shortening fraction
• Normal EF in children is 64+4%
• 2D echo reveals a large, hypo contractile LV
which is globular, with thin walls, mild
pericardial effusion and occasional regional wall
motion abnormalities
MRI
• Said to pick up earliest abnormality in
Myocarditis
• Document the location and extent of
inflammation
• Gadolinium enhancement was greater in
patients with myocarditis than in normal
controls
Cardiac Catheterization
• Reveals depressed cardiac index, elevated left
ventricular end diastolic pressure, and elevated
mean atrial pressure
• Angiography shows decreased left ventricular
function with or without mitral regurgitation
• Main purpose is to obtain samples by
endomyocardial biopsy (EMB) for pathologic
and microbiologic analysis
Endomyocardial Biopsy (EMB)
• Gold standard for the diagnosis of myocarditis
• Dallas criteria
▫ Active myocarditis is defined as "an inflammatory
infiltrate of the myocardium with necrosis and/or
degeneration of adjacent myocytes not typical of
the ischemic damage associated with coronary
heart disease"
EMB
• Dallas criteria
▫ Borderline myocarditis is the term used if
lymphocytic infiltration is present without
myocyte destruction
▫ Ongoing Myocarditis – both inflammation and
necrosis present.
▫ Resolving Myocarditis – inflammation may be
present; cell necrosis not
▫ Resolved Myocarditis – No inflammation / cell
necrosis.
EMB
• The American Heart Association, the American
College of Cardiology, and the European Society of
Cardiology released a scientific statement on the
role of EMB
▫ Limited data are available on EMB in children
▫ EMB is reasonable in the setting of unexplained
cardiomyopathy in children.
▫ Indications include fulminant or acute unexplained
heart failure, cardiac transplant rejection monitoring,
certain unexplained arrhythmias, and idiopathic
dilated cardiomyopathy
Treatment
• Mainstays of therapy for acute or fulminant
myocarditis are monitoring, supportive care, and
standard regimens for heart failure
• Patients should be monitored in a pediatric
intensive care unit
Treatment of Heart Failure
• In the acute phase, diuretics, afterload reducing
agents, and inotropic drugs are used to treat
heart failure
• Dopamine, dobutamine, and milrinone are used
to prevent circulatory collapse in the case of
acute fulminant myocarditis
Treatment of Heart Failure
• Mechanical ventilation
• ECMO
• Ventricular Assist Device
• For patients who progress from acute
myocarditis to chronic heart failure, diuretics,
angiotensin inhibitors, digoxin, and aldosterone
inhibitors (ie, spironolactone) are well-accepted
therapies
Anti-arrhythmic Drugs
• Most antiarrhythmic drugs have negative
inotropic activity and may therefore worsen
cardiac function and lead to acute hemodynamic
instability
• Should be used only when the expected benefit
exceeds the risk, and only in consultation with a
pediatric cardiologist
Immunosuppressive Therapy
• Myocardial damage in infectious myocarditis is
thought to be due to both direct viral damage
and the immune response to infection.
▫ Myocarditis Treatment Trial:
 111 patients with a histopathologic diagnosis of
myocarditis and a left ventricular ejection fraction
(LVEF) of less than 45 percent were randomly
assigned to receive conventional therapy alone or
immunosuppression with either cyclosporine or
azathioprine for 28 weeks [7]. There was no
difference in outcome in the two treatment groups.
Immunosuppressive Therapy
• Corticosteroids
▫ Case series and a small trial are inconclusive with
respect to the beneficial effects of prednisone
and/or other immunosuppressive agents
(azathioprine, cyclosporine) on left ventricular
function and ventricular arrhythmias in children
with myocarditis, but definitive data are lacking
Immunosuppressive Therapy
• IVIG
▫ use is supported by experimental data in which
polyclonal immunoglobulin protects against
myocardial or arterial damage in mouse models of
viral and autoimmune myocarditis
▫ no randomized controlled trials of IVIG for the
treatment of myocarditis in children have been
reported, some clinical evidence suggests it may
be beneficial
Pain control
• Narcotic analgesic
• Avoid NSAIDs which are relatively
contraindicated in this condition
PROGNOSIS
• Most cases are believed to be clinically silent and
resolve spontaneously without sequelae
• Patients who present with CHF experience
morbidity and mortality based on the degree of
left ventricular dysfunction.
• Patients who do not fully recover cardiac
function may develop dilated cardiomyopathy.
OUTPATIENT FOLLOW UP
• Recovered patients should have restricted
activity for 6 months because rapid return to
activity has provoked recurrent inflammation in
animal models
• Follow-up visits with a cardiologist