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
13.
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
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
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
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)
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
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
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
