• Restrictive Cardiomyopathy (RCM)
    • Hypertrophic Cardiomyopathy (HCM)
    • Dilated Cardiomyopathy (DCM)
    • ArrythmogenicRight Ventricular Cardiomyopathy (ARVC)

    Restrictive Cardiomyopathy (RCM)

    • Infiltration or fibrosis of the myocardium causing impaired ventricular filling with preserved systolic function.
      • Reduced or normal diastolic ventricular cavity volume + stiff ventricular myocardium.
      • Causing high filling pressures with small amounts of volume.
      • Early in course biventricular systolic function is normal, but can deteriorate later in disease.
    • Systolic function may diminish in end-stage disease
    • Typical have RAPID early diastolic filling on echo (and JVP - Prominent x/y descents)
    • NOTE: Constrictive pericarditis vs. restrictive cardiomyopathy --> RV and LV share same space in constrictive pericarditis.  Relative increase in filling of RV, which restricts LV filling and vice versa.  Look for this ventricular interaction on echo.
      • Also restrictive cardiomyopathy have higher BNP levels than constrictive pericarditis. (>800)
    • Causes:
      • Infiltrative (rule these out!)
        • Amyoidosis
        • Sarcoidosis
        • Hemochromatosis
      • Non-Infiltrative
        • Scleroderma
        • Radiation
        • Fibrosis following cardiac surgery
        • Myocardial Storage Conditions
        • anthracyclines (doxorubicin, donorubicin)
    • Symptoms:
      • Dyspnea, fatigue, peripheral edema
    • Exam:
      • Elevated JVP (increases with inspiration - Kussmaul's Sign)
      • Normal LV impulse
      • If advanced: Heptosplenomegaly + Ascites
    • DDx:
      • Hypertrophic cardiomyopathy, Dilated cardiomyopathy
      • Constrictive pericarditis: Clinical presentation and physical exam may yield findings identical to RCM.
        • MRI shows pericardial thickening(>5mm), and right heart catheterization demonstrates equalization of diastolic pressures in contrictive pericarditis.
    • Diagnosis:
      1. ECG
        • Conduction System Disease
        • Low QRS voltage
        • Nonspecific ST-T wave changes
      2. Echocardiography
        • Restrictive filling pattern (preserved systolic function)
        • (If infiltrative, myocadium can look granular)
        • Biatrial enlargement
      3. R-Heart Catheterization
        • Dip & plateau vetnricular filling ("Square root sign")
        • Pulmonary hypertension
        • Respiratory concordance of ventricles
      4. Myocardial Biopsy
        • Detects infiltrative disease (amyoidosis & sarcoidosis)
    • Treatment
      • Treat underlying disease
      • Diuretics
        • Can improve symptoms, but hard b/c overdiuresis causes hypotension (preload dependence, no cardiac index)
      • B-Blockers & CCB
        • Can improve diastolic function early in disease. (slow heart rate & increase filling time)
        • Caution: can drop cardiac output
        • CCB: careful in amyloid (causes negative inotropy & conduction problems)
      • Cardiac transplant
        • If no systemic disease


    Hypertrophic Cardiomyopathy (HCM)

    • Autosomal-dominant disorder of myocardial structural proteins that causes premature, severe LVH
      • A subset of HCM can cause asymmetrical septal hypertrophy causing dynamic outflow tract obstruction (HOCM!)
    • Symptoms:
      • Syncope, chest pain, dyspnea
    • Exam:
      • If LVOT Obstruction:
        • Systolic crescendo-decrescendo murmur that
          • intensivies with reduction of LV volume (Standing, valsalva)
          • Decreases with increase LV volume (handgrip, raising legs when supine, stand to sit)
      • S4 and sustained apical impulse are characteristic
      • Carotid upstrokes bifid (mid-systolic obstruction)
    • Treatment:
      • Agents that DECREASE LV VOLUME (preload) such as nitrates & diuretics increase LVOT obstruction --> contraindicated in HCM!

    Notes on Specific Types


    • Heart failure, hepatomegaly, proteinuria, bruising
    • Low voltage ECG (despite increased wall thickness on echo)
    • Diagnose with: sPEP, uPEP, abdominal fat pad or gingival biopsy
    • Cardiac involvement = BAD prognosis



    • Patchy involvement of the myocardium (inflammation & fibrosis due to granuloma formation)
    • Myocardium is not replaced or displaced hence ECG = normal!  (not low voltages like Amyoid)
    • Systemic Symptoms:
      • Pulmonary Involvement
      • Skin involvement




    Giant Cell Myocarditis

    • Presents with heart failure & cardiogenic shock within several days (can be up to months) of onset.
      • Often refractory ventricular arrhythmias
    • High mortality rate
    • Does not have systemic symptoms (unlike amyloid)



    Constrictive vs. Restrictive vs. Tamponade


    Condition History ECG Physical Exam CXR ECHO



    TB, Cardiac Surgery,


    CTD, Trauma,

    Prior Pericarditis


    Pulsus Par. - may have

    JVP (prominent x/y descents,


    Heart Sounds (pericardial




    Pericardial Thickening

    Pericardial Effusion


    Ventricular septal flattening

    with inspiration

    Restrictive CM

    Amyoid, Sarcoid,

    Hemochromatosis, etc..

    - R or L Atrial


    - AV Delay, BBB

    Pulsus Par. - RARE

    JVP - Prominent x/y


            - Kussmaul's

    Heart Sounds - S4

    Murmurs - MR, TR


    - Atrial Enlargement

    - Mod/Severe Diastolic




    Prior effusion,

    cardiac surgery,

    malignancy (i.e. breast Ca),

    recent MI

    Low Voltage,

    Electrical Alternans

    Pulsus Par. - Frequent

    JVP - Abscent/diminished


    Heart Sounds - muffled

    Murmurs - NONE


    Globular Heart

    - Pericardial Effusion

    - RV collapse during









    Cardiac Tamponade

    Equalization of diastolic pressures YES

    L-side is higher

    (LV more restricted)

    Dip & Plateau (Square Root Sign)


    (restricts at end)


    (Restricts at end)


    (restricts entire cycle)

    Respiratory Variation in LV/RV


    Discordant peak

    RV and LV pressures

    (outline restricts/pulsus)

    Concordant peak 

    RV and LV pressures

    (septum restricts too!)


    likely discordant

    (Outline restricts/pulsus)


    Takotsubo Cardiomyopathy

    • Aka stress-induced cardiomyopathy
    • Transient cardiac dysfunction... apical ballooning triggered by emotional stress.  (in some cases no trigger identifiable).
    • Presenting picture looks like ACS:
      • Chest pain, troponin, ischemic ECG
      • Echo or ventriculogram make diagnosis.  (absence of CAD).
    • Treatment:
      • B-blocker therapy + supportive, but does not protect against recurrence.
      • Ejection fraction quickly normalizes, but small percentage take months to recover.


    Tachycardia-Induced Cardiomyopathy

    • Months to years of tachycardia.
      • Atrial or ventricular tachycardia.
      • Frequent PVCs can cause this (>1000 per day).
    • Rate control improves or resolves cardiomyopathy.
    • Recurrence occurs fast if tachycardia recurs.


    Acute Myocarditis

    • Inflammation of myocardium
      • Many causes: toxins, infections (most common: viral infections).
      • Pathophysiology is unkown.
    • Symptoms (range):
      • From minor symptoms to cardiogenic shock.
    • Treatment:
      • Immunosuppressives do not benefit.
      • Supportive care, and therapy for systolic HF for any cause.
      • Fulminant presentations (acute, cardiogenic shock), have higher recovery rates.


    Giant-Cell Myocarditis

    • Form or acute myocarditis (often fatal), affecting those in 40's.
    • Characterized by: rapid onset of fulminant heart failure (days-months).
    • Refractory ventricular arrhythmias are common.
    • Mechanism unknown, thought to be autoimmune.  
    • Treatment:
      • Immunosuppressives (steroids etc..) do not change outcomes.
      • Often need mechanical support or transplant.


    Hypertrophic Cardiomyopathy

    • Primary myocardial diesease: Diffuse or focal LV hypertrophy in the absence of afterload-increasing conditions (AS, HTN, etc.)
    • Prevalence: 0.02%.  50% have familial origin.
    • Most common patern: ASH (Asymmetric Septal Hypertrophy)
    • Often benign
    • Complications:
      • Syncope
      • Arrhythmia
      • Ischemia
      • HF
      • Stroke.
    • Annual mortality: 3-6%.
    • Suspicions:
      • Often sent for screening when family members found this.
      • Often have angina, dyspnea, palpitations, fatigue, dizziness, true syncope.
        • Diastolic dysfunction.
        • Myocardial ischemia
        • LV outflow tract obstruction
        • AFib
    • On Exam:
      • Mid-systolic murmur caused by LVOT obstruction.
      • Valsalva or Squat-to-Standmanouvres, decrease preload, shrinks chamber, more opposition of hypertrophic muscles, murmur becomes louder (40% sensitivity, quite poor)
      • If lift legs, increasepreload, murmur gets quieter.
    • NOTE: Other causes LVOT obstruction (HOCM is a dynamic obstruction).
    • EKG:
      • Increased QRS voltage, ST abnormalities (repolarization abnormalities). (many have normal EKG).
      • Prominent Q-waves (i.e. infero-lateral) are not due to infarcts, due to septal hypertrophy (T-waves upright in leads with Q-waves).
    • Diagnosis:
      • On Echocardiography (≥15mm septum).
        • Often LVEF ok, but diastolic dysfunction and L-atrial enlargement.
        • Often hyperdynamic LV with small cavity.
      • Often need Stress Echocardiogram!!!
        • 40-50% of patients have provokable obstruction on exercise
        • Or any other afterload reduction agent (like amyl nitrite, dobutamine).
        • Diagnosis of HCM on Echo:

          • Resting gradient >30mmHg or provokable gradient of >50mmHg is diagnostic.
    • SAM (Systolic anterior  motion of the mitral valves)
      • Most common mechanism of dynamic outflow tract obstruction.
      • Anterior leaflet of the mitral valve is pushed into the hypertrophied basal septum causing obstruction.
      • The anterior leaflet is then separated from posterior leaflet during systole (should be coapting) --> causing MR that is often directed posterior into the atrium.
      • In HCM, SAM is prolonged



    Continuous-wave Doppler after exercise stress testing in a pt with hypertrophic cardiomyopathy (left panel) demonstrates a high-velocity late-peaking systolic waveform (arrow) across the LVOT from an exercise-provoked dynamic obstruction. Peak velocity is 4.48 M/sec, indicative of a peak gradient of 80 mm Hg. For comparison, the right panel shows pulsed Doppler echocardiography across the left ventricular outflow tract in a patient without hypertrophic cardiomyopathy. There is an earlier-peaking systolic waveform (arrow) with a normal velocity of 0.9 M/sec, indicative of no outflow gradient. Note the difference in velocity scales.



    HCM Mimics

    • Athlete's Heart
      • Athlete is elitely trained in exercise with aerobic and anaerobic demands develops LVH.
      • Lesser degree of hypertrophy (<15mm of any wall thickness), cavity volume enlarged due to high volume state  (rather than reduced in HCM)...  Also no LA enlargement.
    • Infiltrative causes (Sarcoid, Amyloid) --> often symmetric and concentric wall thickening (HCM is asymmetric)
      • Cardiac MRI or PET helps separate them from HCM (delayed hyper-enhancement).
    • Fabrey's Disease (alpha-galactosidase enzyme deficiency)



    • High risk of atrial fibrillation (need atrial kick), may precipirate heart failure and stroke.
    • Some patients can progress to dilated CMTHY with impaired systolic function.
    • Sudden death, heart failure, stroke.
    • Sudden death is infrequent --> more common in young (15-35yo).
      • High risk if :
        • Prev cardiac arrest
        • VTach
        • 1st deg family member with sudden cardiac death with known HCM
        • Recurrent unexplained loss of consciousness (hard to separate from obstructive)
        • Extreme increased wall thickness (>30mm [normal <10mm])
        • Abnormal blunted BP response during exercise, if BP fails to rise during exercise.
        • NSVT (even if asymptomatic on ambulatory ECG).


    • Lifestyle changes
      • Level of exercise (Moderate level of exercise - not competitive or strenuous.  Avoid activities that require sudden intense bursts of energy such as full court basketball, soccer, football).  These are considered high risk for provoking sudden death.
    • Medical therapy warranted if symptomatic.
      • Beta-Blockers - reduce HR, diastolic filling, LV contarctility, dynamic LVOT obstruction.
      • CCB (verapamil) - can use if don't tolerate BB
        • Do not combine with BB due to negative inotropy (severe bradycardia).
        • Vasodilatory effect can cause worse LVOT obstruction, severe symptoms.
      • Disopyramide
        • Very old antiarrhythmic.  
        • Very negative inotropic agent, used in addition to BB therapy.
        • Can prolong QT interval / QRS duration.  
    • Avoid worsening outflow gradient
      • Exercissive diuresis, vasodilators, digoxin.
    • Device therapy
      • Pacemaker
        • For those with bradycardia during medical titration
        • Not used to reduce LVOT obstruction.
      • ICD
        • All HCM patients should receive ICD regardless of presence of symptoms.
        • Primary prevention of sudden cardiac death for HCM and multiple RFs for sudden death.
        • Indicated for LV systolic dysfunction with LVEF <35%.
    • Heart transplant
      • For refractory symptoms that cannot be managed medically 
      • Progressive systolic dysfunction
      • Refractory ventricular arrhythmias
    • Atrial Fibrillation
      • Same rules for anticoagulation, but rhythm control may be preferred to maintain ventricular filling.
      • AFib ablation, amiodarone are options.
    • Septal Reduction therapy
      • Reduce thickness of basal septum to reduce systolic anterior motion of mitral valves => greater forward flow / (stroke volume).  Very significant surgery!
      • Only for medically-refractory cases, and severe cases.
      • However, operativive mortality is quite low (<1%), improves symptoms, quite effective for LVOT.
      • NEW: Alcohol Septal Ablation
        • Catheter passed into septal perforator artery that supplies the hypertrophied part of ventricle with blood.
        • Alcohol injected into that area, causing therapeutic infarction.
        • Initially area remains thick, but over time will thin, reducing LVOT.
        • No trials comparing these two therapies.  
        • Concern about scar causing future arrhythmias.
    • Genetic counselling. (autosomal dominant) --> screen family members.
      • Many genes in effect.  (Sarcomeric proteins).
      • Negative echo in family member does not rule out HCM...
        • 1st degree relatives should undergy q5y echo screening in adulthood to ensure no late-onset HCM.
        • In adolescence echo q1year.

    Cardiac Tumors

    • Atrial Myxomas (L-atrial 80% of the time).
      • Atrial myxomas must be surgically removed (especially if L-sided) to avoid embolic phenomena even in asymptomatic patients.
    • Rhabdomyosarcomas (show up bright on echo)
    • Fibroelastoma (attached to heart valves or mitral cordal aparatus)
      • Can also present with stroke or another embolization phenomena.
    • Secondary tumors 20 times more common than primary.  Originate from lung or breast carcinomas + renal, hepatocellular etc.
    • Cardiac tumors often cause non-specific symptoms... often heart failure and pericardial constriction.
      • Occasionally syncope, stroke (embolization), heart block (invasion), Ventricular arrhythmias.
    • Echo is gold standard in identifying tumors.  TEE specifically is more effective.
    • Generally requires surgical removal, and sent for biopsy.
      • (can't biopsy in cath lab, high risk of embolization).
      • If not removed, very high risk of embolization despite anticoagulation.
    • Malignant tumors are not amenable to curative surgery.
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