This document discusses various cardiomyopathies including dilated, hypertrophic, and restrictive cardiomyopathies. It provides details on dilated cardiomyopathy including causes, clinical presentation, workup, and management. Hypertrophic cardiomyopathy etiology, presentation, and treatment are summarized. The document also reviews pericarditis including presentation, ECG and imaging findings, differential diagnosis, and treatment. Common pediatric heart murmurs and features that suggest an innocent murmur versus potential cardiac pathology are presented.

1. Cardiology Review
Part 2
Oct 2018

2. Cardiomyopathies
 Dilated
– Myocarditis
 Hypertrophic
 Restrictive

3. Dilated Cardiomyopathy
Dilated, poorly functioning left ventricle

4. DDx Dilated
Cardiomyopathy
 Cardiomyopathy
– Idiopathic – 60% of cases
– Familial-usually AD
 Congenital heart disease
 Coronary abnormalities
– Kawasaki, ALCAPA (anomalous left coronary
artery from the pulmonary artery)
 Arrhythmias-incessant SVT (babies)
 Metabolic disorders/rare syndromes
 Neuromuscular disorders
– Duchenne MD

5. DDx Dilated
Cardiomyopathy
 Infectious (myocarditis)
– Adenovirus, HIV, cocksakie, mycoplasma
– Rheumatic Heart Disease (MR,MS,AI)
 Inflammatory
– SLE, collagen vascular disease
 Endocrine
– IDDM, thyroid disease
 Drug induced
– Anthracyclines – most common
– Related to total accumulated dose
 400-600mg/m2 increased risk

6. DDx Dilated
Cardiomyopathy
 Toxins
– Lead, cobalt
 Vitamin deficiency
– Selenium, carnitine, thiamine

7. Dilated CHF – Clinical
Presentation
 Depends on age of presentation
 Vague sx – fatigue, malaise, low grade
fever, anorexia, decreased exercise
tolerance
 CHF sx – dyspnea, diaphoresis, poor
feeding, orthopnea, paroxysmal nocturnal
dyspnea
 Syncope
 Palpitations
 Sudden death / cardiac arrest

8. Dilated Cardiomyopathy CXR
Cardiomegaly, increased PVM, pulmonary edema

9. Figure 33: 13-day-old infant with intrauterine infection, cardiomegaly

10. DCM: Presentation
 CHF
– Tachypnea/respiratory distress
– Hepatomegaly
– Diaphoresis
– FTT
– Edema
– Gallop rhythm
 Fatigue/exercise intolerance
 Arrhythmia
– Palpitations
– Syncope
– ALTE/death

11. Dilated Cardiomyopathy Work-
up
 Thorough history and physical
 ECG, CXR
 ECHO, Holter
 Extensive Bloodwork (with DDx in
mind)
 Specialist consults
– Metabolics, genetics, ID

12. ALCAPA
(Anomalous left coronary from
pulmonary artery)
• Does not present prenatally/at birth:
• Equivalent PVR/SVR
• relatively similar O2 concentrations pulm vs. systemic (fetal)
• Typically presents > 4 weeks:
• LV myocardium perfused by relatively desaturated blood under
low pressure from PA as PVR falls  myocardial ischemia
• Increased ischemia during periods of increased myocardial ischemia
(feeding and crying)
• Leads to LV dysfunction, Mitral regurgitation
Classic presentation:
• CHF symptoms (FTT, tachypnea)
• Irritable with feeds
• Exam: Gallop rhythm, murmur (MR), hepatomegaly, increased WOB
• Classic CXR and ECG

14. 

ECG FINDINGS:
- Q waves- I and AVL
- deep in V5 and V6
-ST elevation/depression inferior (II,III, avF) + lateral (V5,V6)
ALCAPA

15. ECG- Myocarditis
 Overall variable ECG findings
 Sinus tachy
 Low voltage QRS (<5mm limb leads,
<10mm precordial leads)
 Q waves, poor R progression
 ST segment changes, flat T waves or
inverted
 Arrhythmias (ventricular!)

16. Management of low cardiac output
 Initial approach with any patient should be
A,B,C’s
 With impending circulatory collapse
– Oxygen, ventilatory assistance, vascular access
– Inotropes
– Sedation and vagal effects of intubation are
extremely hazardous in the setting of decreased
function
– Non-invasive positive pressure ventilation
– Volume overload is virtually universal
 Diuretic therapy

17. Congestive Heart Failure
General Management
–ACE Inhibitors
 potential for benefit with  afterload to increase
systemic blood flow
 may not be necessary where surgery available,
however some studies showed improved
symptoms and growth
 Renal insufficiency in shunt lesions
–B-Blockers
 Improved neurohormonal profile
  symptoms and improved growth
 May not be necessary where surgery is available
 Digoxin – mixed results in studies
If stable off
inotropes, can
introduce oral
therapy slowly

18. DCM: Treatment
 Anti-failure therapy
– Diuretics, after-load reduction, ACEi, spironolactone, -
blockers,
 Anticoagulation if EF < 25%
 Treat specific cause when possible
– SVT  anti-arrhythmics
– Myocarditis  supportive care, anti-inflammatory treatment
– AI  valve replacement
 Complication specific
– Anti-arrhythmics, defibrillators
 Heart transplant

19. DCM - Outcomes
 Significant variability depending on
etiology
 1 year survival – 60-90%
 5 year survival – 20-60%
 Best prognosis if preceding (<3mos) viral
infection – probably recovering viral
myocarditis

20. Hypertrophic
Cardiomyopathy
 Characterized by a thickened but non-dilated
LV

21. Hypertrophic Cardiomyopathy
Etiology
Genetic- Autosomal dominant mostly
Metabolic disease
– Storage disease
 Glycogen storage disease –Pompe’s disease
 Hunter-Hurler
– Mitochondrial myopathies
Other Syndromes
– Noonan syndrome (~25%)
Infants of diabetic mothers
– Generally regresses over time
– Outflow tract obstruction uncommon
Neuromuscular disorders
– Friedrich Ataxia

22. Presentation
 Asymptomatic
 Easy fatiguability/SOBOE
 Syncope
 Murmur
– Outflow tract obstruction – HOCM
 Arrhythmia/palpitations
– Vtach

23. Presentation
 Syncope
– Outflow tract obstruction
– Coronary insufficiency
– VT/Vfib
 Chest pain
– myocardial bridging of coronary arteries
– Subendocardial ischemia
 ALTE/sudden cardiac death

24. Treatment
Medical
 Medications
– B-blockers
– disopyramide
– Verapamil
 Surgery
– Resection of heart muscle
 Disease specific
– Enzyme replacement - Pompe disease
– Carnitine Coenzyme Q - Mitochondrial disease
– Idebenone - Freidrich’s ataxia
 Complication specific
– Anti-arrhythmic
– Implantable cardioverter-defibrillator (ICD)

25. Infant of Diabetic Mother
 Cardiomegaly is common (30%)
– Transient Hypertrophic cardiomyopathy may occur
Asymmetric septal hypertrophy +/- LVOTO
– Rarely Heart failure < 5–10%.
 ***Inotropic agents worsen the obstruction and are
contraindicated!
 If need to treat  give fluids
 The increased left ventricular mass usually regresses
within several months

26. PERICARDITIS

27. Case
12yo with chest pain
 Previously well
 Mild URTI symptoms over last 2 weeks
 Retrosternal, sharp stabbing, 8/10, crying,
better when tripoding, unable to lie flat,
worse with breathing over last 24hours
 HR 140, BP 90/80, jugular venous
distention, muffled heart sounds,
hepatomegaly to 3cm below RCM

28. Pericarditis
Diffuse ST elevation, PR depression

29. Pericarditis/effusion ECG
 Progressive changes
ST elevation T waves: flat, then biphasic or
inverted
 PR depression
 With large effusion: low voltage QRS,
“electrical alternans” (alternating amplitudes
of QRS complex)

30. CXR-chest pain

31. Pulsus paradoxus:
 On inspiration SBP drops more than
10mmHg
 Ddx: asthma, tamponade, emphysema,
hypovolemia, PE, effusion, pericarditis

32. Know DDx
 Infectious: viral, bacterial, TB
 Rheumatologic: ANA, dsDNA
 Inflammatory: ESR, CRP
 CBC, lytes, BUN, Cr
 Smear

33. Pericarditis: Treatment
 Depends on etiology
 Typically NSAIDS
– Ibuprofen 2-3 weeks
– Occasionally colchicine, especially if recurrent
– Occasionally steroids or other anti-inflammatory
 Recurrence up to 15-30%

34.  Postpericardiotomy syndrome
– Up to 30% of patients post heart surgery but
less frequent in <2yo
– Fever the first week after surgery, malaise,
chest pain, irritabilty,+rub
– ASA 50-75mg/kg for 4-6weeks (indomethacin
or NSAIDs)
– Steroids: prednisone 1-2mg/kg/day
– 20% recurrence rate

35. MURMURS

36. Innocent Murmurs
 More than 80% of children have an innocent heart
murmur of one type or another sometime during
childhood
 All innocent heart murmurs are accentuated in
high-output states (usually a febrile illness)
 A left ventricular false tendon may be associated
(normal variant)
 Associated with normal ECG and X-ray findings

37. Potential Red Flags
 Cardiac pathology should be suspected in
infants when:
– Poor feeding
– Failure to thrive
– Unexplained respiratory symptoms
– Cyanosis
– Associated syndrome (e.g., Down syndrome)

38. Potential Red Flags
 In older children :
– Chest pain (especially with exercise)
– Syncope
– Exercise intolerance
– Family history of sudden death in young
people
– Family history of Marfan syndrome

39. Classic Auscultation Areas

40. TABLE 1
Listening Areas for Common Pediatric
Heart Murmurs
 Upper right sternal border :
– Aortic stenosis, venous hum
 Upper left sternal border:
– Pulmonary stenosis, pulmonary flow murmurs, atrial septal
defect, patent ductus arteriosus
 Lower left sternal border :
– Still's murmur, ventricular septal defect, tricuspid valve
regurgitation, hypertrophic cardiomyopathy, subaortic
stenosis
 Apex :
– Mitral valve regurgitation

41. TABLE 2
Physical Findings in Functional
(Innocent) Heart Murmur
 Precordial activity: Normal
 First heart sound (S1): Normal
 Second heart sound (S2) : Splits and moves with
respiration
 Systolic murmur (supine):
– Crescendo/decrescendo
– Possibly vibratory at lower left sternal
border
 Systolic murmur (standing): Decreases in intensity
 Diastolic murmur: Venous hum

42. Common Innocent Heart
Murmurs

43. Innocent Heart Murmurs:
Diagram

44. TABLE 3
Features That Increase the Likelihood of
Cardiac Pathology
 Increased precordial activity
 Decreased femoral pulses
 Abnormal second heart sound
 Clicks
 Loud or harsh murmur
 Increased intensity of murmur when
patient stands