Case Rounds Laura Miles March 3 2012. 6 month old female Several day hx of cough and increased work...
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Transcript of Case Rounds Laura Miles March 3 2012. 6 month old female Several day hx of cough and increased work...
Further Testing…Further Testing… EGC
Evidence of LVH and possible RVH Inferior T wave inversion
Echo Globally poor LV function Dilated and globular LV SF 11 % and EF 22% Normal coronaries
ClassificationClassification Dilated Cardiomyopathy
Hypertrophic Cardiomyopathy
Restrictive Cardiomyopathy
Dilated Dilated CardiomyopathyCardiomyopathy
Dilation and impaired systolic function of left or both ventricles
Most common type of cardiomyopathy in children Up to 50% 0.5 - 8 per 100 000 children
PresentationPresentation Initial signs and symptoms may be very
vague Irritability Anorexia Abdominal pain Cough (from pulmonary congestion)
PresentationPresentation Signs and symptoms of CHF
Poor systolic function Limited cardiac output
Ventricles enlarge and myocardium hypertrophies in an effort to maintain cardiac output
Enlargement of ventricles increases systolic wall tension in ventricle and increases myocardial oxygen consumption
PresentationPresentation Decreased cardiac output decreases renal
blood flow Enhances renin-angiotensin system Fluid retention (necessary to maintain output)
Stimulation of the sympathetic nervous system increased heart rate
Valvular regurg – dilation of the valve annulus Further myocardial oxygen demand
Physical ExamPhysical Exam Signs of CHF
Cardiomegaly Hepatomegaly Tachycardia Tachypnea
Decreased arterial pulse pressure
Narrow pulse pressure
Gallop
Murmur (MR)
Further SequelaeFurther Sequelae Abnormal myocytes conduction
abnormalities Nonspecific ST and T wave changes Atrial arrhythmias Ventricular arrhythmias
Intraventricular thrombus formation low flow state
A HUGE Differential!!A HUGE Differential!! Majority are idiopathic
20-50% familial Autosomal dominant is most common 5-10% are X linked Small number of autosomal recessive
Mitochondrial
Viral myocarditis
A HUGE Differential!!A HUGE Differential!! Neuromuscular
Duchenne’s Muscular Dystrophy Becker’s Muscular Dystrophy
Later onset and slower progression Myotonic Dystrophy
Other less common Other less common causes:causes:
Infectious (excluding viral myocarditis)
Metabolic Storage diseases
Nutritional deficiencies
Endocrine Hypo/hyperthyroidism Pheochromocytoma
Other less common Other less common causes:causes:
Coronary artery abnormalities Congenital
Abnormal placement of the right coronary artery between the great vessels
Acquired Kawasaki’s
Cardiotoxins Anthracyclines
Doxorubicin Donorubicin Can occur anywhere from months after
treatment completion to years later
Other less common Other less common causes:causes:
Connective tissue diseases
Tachyarrhythmias
Familial hypercholesterolemia
HIV associated
Iron overload
Severe Hypertension
TreatmentTreatment Look for an identifiable cause and treat it!
Majority of patients however have no specific etiology identified
TreatmentTreatment Afterload reduction to improve forward
failure
Diuretics as needed for symptoms of backward failure
Inotropic agents to improve symptomatic forward failure
TreatmentTreatment Treatment is supportive
Either the process resolves The heart improves Or the heart is transplanted
Natural HxNatural Hx Extremely variable
Transient dysfunction with complete recovery and minimal symptoms
Rapid progression to cardiogenic shock and death
Anything in between
Most deaths occur within the first 6 months to 2 years after presentation
Hypertrophic Hypertrophic CardiomyopathyCardiomyopathy
Left ventricular disease
Thickening of the ventricular wall
Nondilated
Hypertrophic Hypertrophic CardiomyopathyCardiomyopathy
Can be symmetric or asymmetric Asymmetric hypertrophy can obscure outflow
tract (HOCM – hypertrophic obstructive cardiomyopathy)
Diagnosis is appropriate only after other causes of left ventricular hypertrophy are excluded
CausesCauses Can be divided into:
Primary (genetic mutation) Secondary
Obstructive congenital heart disease Inborn error of metabolism
CausesCauses Familial
Most often autosomal dominant Wide variability in penetrance Often denovo Generally worse prognosis with early signs of
disease
CausesCauses Transient
Infant of a diabetic mother Can develop with corticosteroid use in prems
Metabolic Glycogen storage disease
Pompe
PresentationPresentation Up to 50% of cases present
asymptomatically Evaluation of a murmur Screening due to a family history
Chest pain
Exercise intolerance
Arrhythmia
Near death event
TreatmentTreatment Supportive
Important to maintain preload and avoid hypovolemia
No role for afterload reduction or inotropic support (systolic function usually ok)
Use of beta blockers, calcium channel blockers Prevention of sudden death debatable May reduce symptoms
TreatmentTreatment Indications for ICD treatment
Family history Severe hypertrophy History of ventricular arrhythmia Near sudden death
Restrictive Restrictive CardiomyopathyCardiomyopathy
Restriction of flow into the ventricles
Accounts for less than 5% of cardiomyopathies in children
PathophysiologyPathophysiology Abnormal relaxation of ventricular
myocardium
Decreased ventricular compliance
Altered diastolic filling Increased ventricular end-diastolic pressure Elevated atrial pressure Enlargement of atrium
CausesCauses Idiopathic
Systemic disease Scleroderma Amyloidosis Sarcoidosis
Inborn errors of metabolism Mucopolysaccharidosis Hypereosinophilia
Radiation
Malignancy
Noncompaction of the left ventricular myocardium
TreatmentTreatment Medical treatment has limited success
Pay close attention to fluid balance High atrial pressures required for adequate
preload If atrial pressures too high, may lead to
congestion Difficult to achieve adequate balance
Usually require early consideration for transplant