Cardiomyopathy and Pericardial Diseases Flashcards
What is the difference between primary and secondary cardiomyopathies?
Secondary is due to a specific disease
What is the definition of cardiomyopathy?
Myocardial disorder in which heart muscle is structurally or functionally abnormal, in absence of CAD, HT, valvular disease, and congenital heart disease sufficient to cause observed myocardial abnormality
What is the definition of dilated cardiomyopathy?
Left ventricular dilatation and reduced systolic function in absence of abnormal loading conditions or CAD sufficient to cause it
What are the causes of dilated cardiomyopathy?
Idiopathic - most common
Toxins - alcohol, anthracycline agents
Inflammatory - post partum
Infectious - post viral (most have prodromal URI 2-4 weeks before onset of symptoms), HIV, chagas, t Cruzi
Familial - 20% have first degree relatives
What can a patient with dilated cardiomyopathy present with?
Left heart failure symptoms
Right heart failure symptoms
Low output state symptoms
Any combo of the above
What does physical exam of dilated cardiomyopathy show?
JVD lateral PMI s3, s4 universal RHF absent in 50% Mitral/tricuspid regurg Resting tach, narrow pulse pressure Generalized cardiomegaly
What are bad and good indicators of prognosis in dilated cardiomyopathy and other systolic dysfunctions?
LVEF - bad
Good - symptom class and functional abilities
Best - maximal oxygen consumption
What are the 3 big consequences of untreated dilated cardiomyopathy?
Lethal arrhythmias and SCD
Progressive heart failure
Thromboembolic events
What does the EKG of dilated cardiomyopathy show?
Sinus tach A fib LVH with strain Conduction defects Possible atrial enlargement Atrial and ventricular ectopy Nonspecific ST-T wave abnormalities Pseudo infarction - q waves in ant or inf leads in absence of MI
What is the definition of hypertrophic cardiomyopathy?
Inappropriate LV hypertrophy resulting in impaired diastolic function in absence of apparent etiology
Usually asymmetric involving septum, apex, or lateral wall
Might be obstructive - LV outflow tract
What 3 findings characterize obstructive HCM?
Thickened and asymmetric septum (>1.3x wall)
Abnormal movement of anterior mitral valve leaflet during systole
Cavity obliteration during systole
What kinds of mutations are responsible for familial HCM?
Mutations in sarcomeric proteins
Beta Myosin heavy chain gene mutation
Troponin I mutations
Tropomyosin mutations
What is the pathophysiology of HCM?
Abnormal diastolic relaxation –> increased LV end diastolic pressure –> increased LA pressure –> pulm congestion and dyspnea
What is HCM responsible for?
Leading cause of death in young athletes
What kinds of symptoms will a patient with HCM present with?
Primarily left sided heart failure symptoms
What is found on the physical exam of a patient with HCM?
Bisferiens pulse Forceful and lateral PMI Prominent s4 Apical systolic thrill Systolic murmur - do maneuvers to differentiate from aortic stenosis
What kinds of maneuvers will affect the murmur in HCM?
Increases when standing or Valsalva maneuver - reduces LV size and increases contractility
Decreases when squatting or hand grip - increases LV size and decreases contractility
What are three things that can cause worsening symptoms in HCM?
Increased hypertrophy
Development of a fib
Worsening mitral regurg
What does the EKG of HCM show?
LVH
Prominent q waves due to septal hypertrophy in inf and lateral leads
Giant inverted t waves in precordial leads
What is restrictive cardiomyopathy?
Infiltration, scarring, and fibrosis of myocardium and results in diastolic dysfunction initially
Systolic dysfunction appears late in disease
What is restrictive cardiomyopathy?
Diastolic dysfunction with normal systolic ventricular function and normal wall thickness
Excessive rigidity of right and left ventricle
What are the three most common causes of restrictive cardiomyopathy?
Amyloidosis - pink amorphous material in endocardium biopsy of RV
Hemochromatosis
Sarcoidosis - granulomas in myocardium
What are the signs and exam findings of restrictive cardiomyopathy?
Diminished co and primarily right sided symptoms - fatigue, exercise intolerance
A fib or heart block - respond poorly to medical therapy, common thromboembolic events
Signs of right heart failure
Kussmauls sign
S1 and S2 normal
What does a ground glass appearance of myocardium on echo suggest?
Cardiac amyloid
