cardiomyopathy in children

1. Cardiomyopathy,
Myocarditis and
Pericardial Disease
Dr. Pace
September 22, 2005
Chapter 55

2. Cardiomyopathy
3rd most common form of heart disease in
U.S.
2nd most common cause of adolescent
sudden death(IHSS or HOCM)
Directly affects cardiac structure and
impairs myocardial function

3. Cardiomyopathy
Four types
Dilated Cardiomyopathy(DCM)
Hypertrophied Cardiomopathy(HCM)
Restrictive Cardiomyopathy
Dysrhythmic right ventricular
cardiomyopathy

4. Dilated Cardiomyopathy
Dilation and compensatory hypertrophy of
myocardium
Depressed systolic function and pump
failure with low cardiac output
80% of DCM cases are idiopathic
African Americans and males have 2.5x
increased risk
Most common age of diagnosis 20-50yrs

5. Dilated Cardiomyopathy
Clinical Presentation
Signs and symptoms of CHF-dyspnea on
exertion, orthopnea and pnd.
Chest pain can occur due to low coronary
vascular reserve
Mural thrombi formation can occur
Holosystolic regurgitant murmur or gallop
may be present
Dependent edema, bibasilar rales

6. Dilated Cardiomyopathy
Diagnosis
CXR- enlarged heart, biventricular
enlargement, and pulmonary vascular
congestion(cephalization)
ECG- LVH, Left atrial enlargement, Q
waves, poor R wave progression, afib
Echo-Confirms Dx.-ventricular
enlargement, increased systolic and
diastolic volumes, decreased EF

7. Dilated Cardiomyopathy
Differential
Acute MI
Restrictive Pericarditis
Acute valvular disruption
Sepsis
Any other condition that results in low
cardiac output state

8. Dilated Cardiomyopathy
ED care and disposition
Newly diagnosed or symptomatic DCM-
admit
IV lasix and digoxin-improve symptoms
Ace inhibitors and B-Blockers-improve
survival
Amiodarone- for complex venticular ectopy
Anticoagulation can be considered

9. Hypertrophic Cardiomyopathy
Asymmetric LVH and/or RVH-primarily
involves septum-usually without dilation
Abnormal compliance-impaired diastolic
relaxation and filling-output usually normal
50% are hereditary
Prevelence 1 in 500, Mortality 1%
Mortality 4-6% in childhood/adolescence

10. Hypertrophic Cardiomyopathy
Clinical Features
Symptom severity progresses with age
Dyspnea on exertion-most common initial
or presenting symptom
Angina-like chest pain, palpitations and
syncope may also be present

11. Hypertrophic Cardiomyopathy
Exam
Fourth heart sound(S4)
Hyperdynamic apical pulse
Precordial lift
Systolic ejection murmur at apex or lower
left sternal border
Murmur increased with valsalva maneuver

12. Hypertrophic Cardiomyopathy
Diagnosis
EKG-LVH in 30%,
Left atrial enlargement In 25-50%
Large septal Q waves-25%
CXR-usually normal
Echo-study of choice-demonstrates
disproportionate septal hypertrophy

13. Hypertrophic Cardiomyopathy
ED Care and Disposition
Pts who c/o exercise intolerance or CP
with typical HCM murmur-needs echo-
refer to cardiology
B-blocker-treatment of choice for HCM
with CP
Discourage vigorous exercise(not a
problem for most ed patients)
Admit HCM with syncopal episode

14. Restrictive Cardiomyopathy
One of least common cardiomyopathies
Ventricular volume and wall thickness is
normal
Decreased volume of both ventricles
Mostly idiopathic- sometimes familial
Systemic disorders-amyloidosis,
sarcoidosis, hemochromatosis,
scleroderma, and carcinoid.

15. Restrictive Cardiomyopathy
Clinical Features
Symptoms of CHF-dyspnea, orthopnea,
pedal edema- rare chest pain
Exam-may have S3 or S4 gallop, rales,
jvd, Kussmaul’s sign(jvd with inspiration),
hepatomegaly, pedal edema or ascites

16. Restrictive Cardiomyopathy
Diagnosis
CXR-signs of CHF without cardiomegaly
EKG-nonspecific changes most likely
Conduction disturbances and low-voltage
QRS complexes are common with
amyloidosis or sarcoidosis

17. Restrictive Cardiomyopathy
Differential Diagnosis
Constrictive pericarditis
Diastolic left ventricular dysfunction(due to
ischemic or hypertensive heart disease)
Need to differentiate restrictive
cardiomyopathy from constrictive
pericarditis(surgical treatment)

18. Restrictive Cardiomyopathy
ED Care and Disposition
Symptom directed-diuretics and ace
inhibitors
Corticosteroids for sarcoidosis
Chelation therapy for hematochromatosis
Admission based on severity of symptoms
and availability of prompt follow up

19. Dysrhythmogenic Right Ventricular
Cardiomyopathy(DRVC)
Most rare form of cardiomyopathy
Progressive replacement of RV
myocardium with fibrofatty tissue
Typical presentation of sudden death in
young or middle aged pt
Exam usually normal
EKG- RBBB may be present
Echo-necessary for diagnosis

20. Myocarditis
Inflammation of myocardium
Can be result of systemic disorder or
infectious agent
Viral-Coxsackie B, echovirus, influenza,
parainfluenza, Epstein-Bar, and HIV
Bacterial-C. Diptheria, N. meningitidis, M.
pneumonia, and beta-hemolytic strep
Frequently accompanied with pericarditis

21. Myocarditis
Clinical Feature
Fever, tachycardia out of proportion to
fever, myalgias, headache,rigors
Chest pain due to coexisting pericarditis
Pericardial friction rub
Severe cases may have CHF symptoms

22. Myocarditis
Diagnosis and Differential
EKG-nonspecific changes, av block,
prolonged QRS suration, or ST
elevation(with pericarditis)
CXR-Normal
Cardiac Enzymes- may be elevated
Differentail-ischemia or infarct, valvular
disease, and sepsis

23. Myocarditis
ED Care and Disposition
Supportive care
Blood cultures
Antibiotics for bacterial cause
Watch for signs of progressive heart failure

24. Acute Pericarditis
Loose visceral pericardium and dense
parietal pericardium surround heart
Pericardial space may contain up to 50ml
normally
Etiologies of acute pericarditis-viral,
bacterial, fungal, malignancy, drugs,
radiation, connective tissue disorder,
uremia, myxedema, post-MI, or idiopathic

25. Acute Pericarditis
Clinical Features
Most common-sudden or gradual onset of
sharp or stabbing pain with radiation to
back, neck, L shoulder or arm
Radiation to L trapezial ridge is
distinguishing
Pain more severe with lying supine and
relieved with sitting
Low grade fever, dyspnea and dysphagia
Transient, intermittent friction rub

26. Acute Pericarditis
Diagnosis
EKG-changes in four stages
1-ST elevation in I, V5 and V6, PR depression in
II, aVF and V4-V6
2-ST segment normalizes, T wave decreases
3-Inverted T waves in leads with previous ST
elevation
4-Return to normal ECG
In I, V5, or V6 ST:Twave ratio >0.25 most likely
acute pericarditis

27. Acute Pericarditis
Diagnosis and Differential
Chest Xray-normal and can help r/o other
disease
Other tests of value-CBC, bun and cr,
streptococcal serology, viral serologies,
antinuclear/anti-DNA abs, thyroid function,
ESR, Cardiac Enzymes

28. Acute Pericarditis
ED Care and Disposition
Idiopathic or presumed viral etiology-
outpatient with NSAIDs for 1-3 weeks
Treat any indentified specific causes
Admit for myocarditis or hemodynamic
instability

29. Nontraumatic Cardiac Tamponade
Pressure in pericardial sac exceeds
normal filling pressure in RV-restricts filling
and cardiac output
Etiology-metastatic malignancy, uremia,
hemorrhage(over-anticoagulation),
bacterial or tubercular disorders, chronic
pericarditis, SLE, post radiation,
myxedema

30. Nontraumatic Cardiac Tamponade
Clinical Features
Dyspnea and decreased exercise
tolerance-wt loss, pedal edema, ascites
Tachycardia, Narrow pulse pressure
Pulsus paradoxus
JVD, Muffled heart sounds, Hypotension

31. Nontraumatic Cardiac Tamponade
Diagnosis and Differential
EKG-low voltage QRS with ST elevation
and PR depression possible
Electrical Alternans-classic finding—P and
R wave beat to beat variability
CXR-+/- enlarged cardiac silhoutte
ECHO-diagnostic modality of choice

32. Nontraumatic Cardiac Tamponade
ED Care and Disposition
IV Fluid Bolus-improves RV filling and
improves hemodynamics
Pericardiocentesis-therapeutic and
diagnostic
Admission to ICU or monitored setting

33. Constrictive Pericarditis
Occurs when fibrous thickening and loss
of elasticity interfere with diastolic filling
Cardiac trauma, pericardiotomy,
intrapericardial hemmorhage, fungal or
bacterial pericarditis, uremic pericarditis
are most common causes

34. Constrictive Pericarditis
Clinical Features
Sx’s gradually develop-mimics restrictive
CM- CHF, DOE, and decreased exercise
tolerance
Chest pain, orthopnea and pnd are
uncommon
Exam-Pedal edema, hepatomegaly,
ascites, jvd, and Kussmaul’s sign.
Pericardial “knock”-early diastolic sound
may be heard at apex

35. Constrictive Pericarditis
Diagnosis
EKG-not very helpful-may show low
voltage QRS and inverted T waves
CXR-pericardial calcifications seen in 50%
on lateral view(not diagnostic)
ECHO, CT, MRI are diagnostic

36. Constrictive Pericarditis
Differential Diagnosis
Consider acute pericarditis, myocarditis,
exacerbation of chronic ventricular
dysfunction, or systemic process resulting
in decreased cardiac performance(sepsis)

37. Constrictive Pericarditis
ED Care and Disposition
Supportive care
Symptomatic patients require admission
and pericardiectomy

38. QUESTIONS
Which is the 2nd most common cause of adolescent
sudden death?
A. Suicide
B. MVA
C. Overdose
D. Hypertrophic cardiomyopathy
D

39. Which of the following is not useful in cadiac
tamponade?
a. IV fluids
b. Pericardiocentesis
c. Bedside ultrasound
d. Diuretics
e. Ekg
d.

40. Which of the following is not found on the
EKG of acute pericarditis
A. ST elevation and PR depression
B. Normal EKG
C. Inverted Twave
D. Electrical Alternans d

41. Which is not a sign or symptom of acute
pericarditis?
A. Sharp stabbing chest pain with
radiation to back, neck or L shoulder
B. Osler’s Nodes
C. Friction Rub
D. Pain worse with lying supine
B,

42. b
Which is not one of the 4 types of
cardiomyopathy?
A. Infectious
B. Dilated
C. Hypertrophied
D. Restrictive
E. Dysrhythmic Right Ventricular
A

43. Head CT
Look at bony structure-fx?
Look at periphery for epidural
Or Subdural bleeding
Look at parenchyma
Is there bleeding or infarct?
Is there a mass
Look at ventricles-
Are they normal size?
Are they clear