Table of contents
.
Introduction
- 3 Morphologic Categories:
- Multiple Etiologies for each morphologic change
- Only diagnose condition 25% of the time
- Hazebroek et al JACC 2015 - Registry of all cardiomyopathies
- Found cause in 75%
- Common Exam Question:
- Constructive Pericarditis vs. Restrictive CMPY vs. Cardiac Tamponade
-
Entity History ECG Physical Exam CXR Constrictive
Pericarditis
TB, CV surgery,
radiation, CTD
trauma, prior
pericarditis
non-specific Pulsus Paradoxus
JVP: (prominent X and Y descents)
+ Kussmaul's
Heart Sounds: Pericardial knock
No Murmurs
Pericardial
Calcification
Restrictive
Cardiomyopathy
Amyloidosis,
Sarcoidosis,
Hemochromatosis
RAE or LAE
AV delay, BBB
JVP: prominent X and Y descents
+Kussmaul's
Heart sounds: Prominent S4
Pulsus Paradoxus - RARE
Non-Specific Cardiac
Tamponade
Prior effusion,
CV surgery,
malignancy
(breast ca)
recent MI
Low voltage,
electrical alternans
JVP: Absent or diminished Y descent
"lose your Y before you die"
Heart Sounds: Muffled
No Murmurs
Cardiomegaly
(globular heart?)
-
Entity Echo Hemodynamics Constrictive
Pericarditis
Pericardial thickening
Pericardial effusion (maybe)
Ventricular septal flattening with inspiration
Equalization of Diastolic Pressures:
YES
Dip-And-Plateau Sign ("Square Root Sign"):
YES (restricts at end)
LV/RV Respiratory Variation:
Discordant peak RV/LV pressures
Restrictive
Cardiomyopathy
Atrial enlargement
Mod-to-Severe Diastolic Dysfunction
Equalization of Diastolic Pressures:
L-sided higher (more restricted on L)
Dip-And-Plateau Sign ("Square Root Sign"):
YES (restricts at end)
LV/RV Respiratory Variation:
Concordant peak RV/LV pressures
(septum restricts too!)
Cardiac
Tamponade
Pericardial Effusion Present
RV Collapse in Diastole
Equalization of Diastolic Pressures:
YES
Dip-And-Plateau Sign ("Square Root Sign"):
NO!! (restricted throughout cycle)
LV/RV Respiratory Variation:
Variable (more discordant likely)
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 pattern:
- ASH (Asymmetric Septal Hypertrophy)
- Some cause asymmetrical septal hypertrophy and dynamic LVOT obstruction
- ASH (Asymmetric Septal Hypertrophy)
- Symptoms:
- Syncope, Chest Pain, Dyspnea
- 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:
-
- If LVOT obstruction present:
- Mid-systolic murmur caused by LVOT obstruction.
- Valsalva or Squat-to-Stand manouvres, decrease preload, shrinks chamber, more opposition of hypertrophic muscles, murmur becomes louder (40% sensitivity, quite poor)
- Opposite maneuver to increase LV volume decreases murmur:
- Lift Legs (pt supine)
- Hand Grip
- Independent of obstruction:
- S4
- Sustained apical impulse
- Carotid upstrokes are bifid (mid-systolic obstruction)
- Typical Exam Question: Distinguish HCM from AS:
- Murmur of AS radiates to neck, weak and delayed carotid pulse (bifid in HCM)
- Typical Exam Question: Distinguish HCM from Hypertensive Disease
- Not LVOT obstruction
- No asymmetric septal hypertrophy
- If LVOT obstruction present:
- NOTE: There are other causes LVOT obstruction (HOCM is a dynamic obstruction).
- Investigations:
- EKG:
- LVH
- LAE
- Increased QRS voltage, ST abnormalities (repolarization abnormalities). (many have normal EKG).
- Prominent Q-waves (i.e. infero-lateral - precordial and leads I and II) are not due to infarcts (pseudoinfarction pattern), but due to septal hypertrophy
- T-waves upright in leads with Q-waves
- Can cause giant anterior T-wave inversions
- Echocardiography (≥15mm septum).
- LVH
- Systolic Anterior Motion of mitral valve (SAM)
- Maybe asymmetric septum and LV outflow obstruction (depends on type)
- LVEF is usually ok
- LV cavity is SMALL and HYPERCONTRACTILE --> diastolic dysfunction present
- 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.
-
- EKG:
- 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.
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.
- Genetic Testing:
- Not routinely done for diagnosis
- Used to identify genotype (for prognosis)
- Screen family members
HCM Mimics
- Athlete's Heart
- The athlete is 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)
Complications
- 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).
- High risk if :
Management
- 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.
- Nitrates and diuretics are contraindicated (increase LVOT obstruction, incrase murmur intensity)
- Reduce HR and increase diastolic filling time!!
- Beta-Blockers - reduce HR, diastolic filling, LV contractility, 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
- Some say all HCM patients should receive ICD regardless of presence of symptoms.
- Other guidelines: Indications for ICD in HCM patients:
(ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy)
- Pacemaker
- Massive myocardial hypertrophy (wall thickness ≥30 mm);
- Previous cardiac arrest due to ventricular arrhythmia;
- Blunted blood pressure response or hypotension during exercise;
- Unexplained syncope;
- Nonsustained ventricular tachycardia on ambulatory electrocardiography;
- Family history of sudden death due to HCM
- Remember: EP study and/or ICD placement indicated in pts with syncope and family history of sudden cardiac death.
- Indicated for LV systolic dysfunction with LVEF <35%.
- Heart transplant
- For refractory symptoms that cannot be managed medically
OR - Progressive systolic dysfunction
OR - Refractory ventricular arrhythmias
- For refractory symptoms that cannot be managed medically
- 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 symptoms, 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.
Restrictive Cardiomyopathy
- Hallmarks:
- Fibrosis/infiltration of myocardium
- Impaired Ventricular Filling with PRESERVED systolic function
- NOTE: In end-stage disease, systolic dysfunction may develop
- Hemodynamics:
- Reduced or normal diastolic ventricular cavity volume, but myocardium is stiff
- Causing high filling pressures with small amounts of volume.
- Early in course biventricular systolic function is normal but can deteriorate later in disease.
- Characterized by RAPID early diastolic filling.
- Causes: (but many idiopathic):
- Non-infiltrative (Scleroderma, Radiation, Fibrosis after CV surgery)
- Infiltrative (Amyloidosis, Sarcoidosis, Hemochromatosis)
- Myocardial Storage Conditions
- Endomyocardial Disorders
- Drugs (anthracyclines - cause dilated or restricted dz)
- R/O: amyloid, sarcoid, hemochromatosis, anthracycline use.
- Symptoms:
- Dyspnea, Fatigue, Peripheral Edema
- Physical Exam
- ↑ JVP (increases with inspiration = Kussmaul's Sign)
- Normal LV impulse
- HSM and ascites if advanced
- DDx: Hypertrophic CMP, Dilated CMP, Constructive Pericarditis (identical to RCM on exam)
- Investigations:
- ECG: conductive system disease, low QRS voltage, non-specific ST-T wave changes.
- Echo:
- Restrictive filling pattern
- Preserved EF
- Bi-atrial enlargement
- (If infiltrative = granular apperance of myocardium)
- R-Heart Cath:
- Dip-and-plateau ventricular filling pressure ("square root sign")
- Pulmonary HTN
- Respiratory concordance of ventricles
- Myocardial Biopsy
- Detects infiltrative causes (amyloid & sarcoid)
- Common Exam Question:
- Distinguish: Constrictive pericarditis vs. restrictive cardiomyopathy
- Echo: RV and LV share same space in constrictive pericarditis. Constrictive pericarditis = Relative increase in filling of RV, which restricts LV filling and vice versa. Look for this ventricular interaction on echo.
- MRI: Pericardial thickening > 5mm
- Also restrictive cardiomyopathy has higher BNP levels than constrictive pericarditis. (>800)
- R-heart cath: (constrictive pericarditis = equalization of diastolic pressures)
- Distinguish: Constrictive pericarditis vs. restrictive cardiomyopathy
- Management:
- Prognosis is poor (surival is 64% at 5 years, 37% 10-years post-dx.).
- Treat underlying cause
- Pharmacotherapy:
- None improve mortality (except heart transplant).
- Diuretics (but very preload dependent, can lead to syncope)
- B-Blockers & CCB:
- improve diastolic function early in disease by increasing filling time.
- CAUTION: can cause drop in cardiac output
- Avoid CCB in amyloid (can cause significant negative chronotropy due to conduction disease).
- Transplant
- Esp good candidates if no severe systemic disease.
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.
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.
ARVC(D)
- Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia
- Genetic disorder leading to fibrofatty replacement of RV myocardium.
- Early changes localized to one of 3 RV sites ("triangle of dysplasia")
- Inflow Tract
- Outflow Tract
- Apex
- Late in disease can affect most of RV myocardium and even posterolateral LV wall.
- Familial autosomal dominant disorder (recessive forms present)
- Symptoms/Presentation:
- YOUNG! and Adolescents
- Palpitations, Syncope, or aborted sudden death
- Investigations:
- ECG:
- Baseline: TWI in V1-V3, PVCs of LBBB morphology
- VT of LBBB morphology
- CXR
- Other:
- 24-hour ambulatory ECG
- Signal Averaged ECG
- Stress Test
- 2D Echo
- If inconclusive evaluation thus far:
- MRI
- Contrast Angiography
- Endomyocardial biopsy
- ECG:
- Diagnosis:
-
Categories:
- Structural (Global or regional dysfunction and structural alterations)
(Echo, MRI - late gadolinium enhancement) - Tissue
- Repolarization Abnormalities
(on surface ECG - i.e. inverted T-waves in precordial leads) - Depolarization/Conduction Abnormalities
(Signal Averaged ECGs, surface ECGs - i.e. epsilon wave) - Arrhythmias
(RV origin) - Family History
(1st Degree relative who meets task force criteria)
- Definitive Diagnosis
- 2 major criteria
- 1 major plus 2 minor criteria
- 4 minor criteria from different categories.
- Borderline Diagnosis
- 1 major and 1 minor
- 3 minor criteria from different categories
- Possible Diagnosis
- 1 major
- 2 minor criteria from different categories
- For Details see TASK FORCE 2010 RECOMMENDATIONS
- Structural (Global or regional dysfunction and structural alterations)
- Treatment
- Low EF
- Medical Therapy
- Arrhythmias
- Antiarrhythmics, ICD, etc...
- Ablations (often temporary relief of arrhythmias b/c disease is progressive)
- Transplant (rare)
- If very severe symptoms.
- Although heart transplant is rarely needed.
- Low EF
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