Myocardial and pericardial disorders Flashcards
Normal Pericardium Definition
- double-walled sac
- surrounds the heart and great vessels origin
Normal Pericardium Components
Two layers
- Fibrous Parietal layer (2 mm thick, accelular)
- Serous Visceral layer = epicardium- contact with mioyocardium
- Pericardial cavity
- Pericardial fluid → 15 –50 ml (serous fluid- plasma ultrafiltrate)
→ secreted / absorbed continuously
→ 800 ml/day
! well Innervated → inflammation may produce severe pain
→ trigger vagus mediated reflexes
PHYSIOLOGY OF THE PERICARDIUM
PERICARDIUM ROLES
- Fixes the heart to the mediastinum (ligamentous attachments to the diaphragm, sternum, and other structures)
- Limits friction between the heart and surrounding structures
- prevents displacement of the heart and kinking of the great vessels
- Provides lubrication for the heart
- Protection against extension of mediastinal infections
- Delaying extension of infections / malignancy
- HEMODYNAMIC Role → distribution of the hydrostatic forces
- Coupling of ventricular diastolic pressures
- Facilitating atrial filling in ventricular systole
- Increasing blood volume in ventricular systole
- Preventing acute dilations of heart
Classical pericardial syndromes
- Pericarditis (Inflammatory Diseases)
- Pericardial effusion
- Cardiac tamponade
- Constrictive pericarditis
- Other
• Congenital absence of pericardium
• Tumors
Acute pericarditis Definition
Syndrome due to Pericardial Inflammation
- without pericardial effusion
- with pericardial effusion
Not causing compression
Causing compression
ESC Classification
- Acute pericarditis : New-onset pericarditis
- Incessant : Lasting for >4–6 Weeks but <3
Months without remission - Chronic Pericarditis : lasting for >3 Months
- Recurrent Reccurence of pericarditis after a documented first episode of acute pericarditis and a
symptom-free interval of 4–6 Weeks or longer
Pericarditis- Etiologic Classification
1.Infectious Pericarditis
2.Non - Infectious Pericarditis
3. Pericarditis most likely related to hypersensitivity or
autoimmunity
Infectious Pericarditis
- Viral (coxsackievirus A and B, echovirus, mumps, adenovirus, HIV)
- Bacterial → Pyogenic (pneumococcus, streptococcus, staphylococcus, etc)
- Tuberculous
- Fungal
- Other infections (syphilitic, protozoal, parasitic)
Non- Infectious Pericarditis
- Acute myocardial infarction
- Uremia
- Neoplasia
a) Primary tumors (benign or malignant, mesothelioma)
b) Tumors metastatic to pericardium (lung and breast cancer, lymphoma, leukemia) - Myxedema
- Chylopericardium
- Trauma
a) Penetrating chest wall
b) Nonpenetrating - Aortic dissection (with leakage into pericardial sac)
- Postirradiation
- Familial Mediterranean fever
10.Acute idiopathic
11.Whipple’s disease
12.Sarcoidosis
III. Pericarditis most
Pericarditis most likely related to hypersensitivity or
autoimmunity
- Rheumatic fever
- Collagen vascular disease (systemic lupus erythematosus, rheumatoid arthritis, ankylosing spondylitis, scleroderma, granulomatosis with polyangiitis (Wegener’s)
- Drug-induced (e.g., hydralazine, phenytoin, Isoniazide, anticoagulants)
- Post-cardiac injury
a) Postmyocardial infarction (Dressler’s syndrome)
b) Postpericardiotomy
c) Posttraumatic
Pericarditis
Clinical diagnosis
At least 2 out of 4 following criteria:
(1) Pericarditic Chest Pain
(2) Pericardial Rubs
(3) Dynamic Ecg changes (New widespread ST-
elevation or PR depression)
(4) Evidence of Pericardial effusion (new or worsening)
Additional supporting findings: - ↑ marker of inflammation CRP, erythrocyte sedimentation rate, and WBC - Evidence of pericardial inflammation (imaging technique: CT, CMR)
ACUTE PERICARDITIS
Fibrinous or Effusive
Without compression of the heart
CLINICAL aspect
- Symptoms suggestive for etiology (ex.: fever, myalgia)
- Typical features
- Nerves compression / iritation
Symptoms
Dyspnea Cough
Dysphagia Hiccup
Dysphonia Nausea
ACUTE PERICARDITIS
Fibrinous or Effusive
Without compression of the heart
(1) CHEST PAIN characteristics
- Localization Retrosternal
- Left precordial area
- Referred to : Neck base, left Trapeziums ridge, Left arm
- Aggravated by : Inspiration, coughing, deglutition, changes in body position, dorsal decubitus
- Relieved by : Leaning Forward, Sitting Up
Duration Hours, Days
Intensity ↑ → Infectious AP - Absent → fluid develops within long time
(neopasms, tuberculosis, uremia)
Differentiation of pericarditis from
myocardial ischemia or infarction
image slide 7
Pericarditis
Differential diagnosis
- AMI
- Aortic dissection
- Pulmonary embolism
- Pneumonia
- Mediastinitis
ACUTE PERICARDITIS
Fibrinous or Effusive
Without compression of the heart
PHYSICAL EXAMINATION
Position: upright and leaning forward position
Auscultation (diagnostic feature):
(2) PERICARDIAL FRICTION RUB
- Does not respect cardiac cycle
- It’s over the cardiac sounds
- Superficial
- Inconstant
- May disappear (with great quantity of fluid)
- Characteristics : high-pitched, scratching, grating
Percution Cardiac dullness:
Normal (pericarditis without effusion or↓↓effusion) OR
Increased (pericarditis with large effusion)
Palpation: Apex beat
Interior of the dullness (if fluid +++) OR
Normal in dry pericarditis
Auscultation (other):
Cardiac sounds
Faint ( if fluid +++ ) OR
Normal ( if no fluid or ↓↓↓ fluid)
ACUTE PERICARDITIS
Fibrinous or Effusive
Without compression of the heart
(3) Ecg - dynamic changes
- PR depression
- ST pattern - I. ST elevation +Twave, STE without ST
- depression, in all
(dynamic) leads, except V1 and aVR - II. Isoelectric ST segment and flattened T wave
- III. Inverted T waves later
- IV. normalisation Ecg
- ST-T Changes
- QRS ± reduction in voltage of the QRS complexes
- electrical alternans (in Cardiac Tamponade)
- Rhythm disorders: sinus tachycardia (AFi, AFl)
- Conduction disorders: usually with myocarditis
ACUTE PERICARDITIS
Fibrinous or Effusive
Without compression of the heart
(4) Pericardial effusion evidence
Physical exam Echocardiography Chest Radiography ─ An increased cardiothoracic ratio occurs only with pericardial effusions > 200- 300 ml
Radioscopy
─ Diminished pulsations of the cardiac border
ECHOCARDIOGRAPHY
↑ Specificity, Sensibility →“gold standard”
has replaced other methods
• M mode detects quantity of fluid as small as 20 ml
• 2D detects circumferential / localized pericardial effusion
Detects
• Variable amount of Pericardial effusion (if present)
An ECHO-FREE space between the epicardium and
pericardium behind LV, in front of RV
( !!!! If this space is small and only in systole =
PHYSIOLOGICAL)
- If large fluid present →swinging heart
- Thickened and hyper-reflective pericardial layers
- Wall motion abnormalities in myo-pericarditis
- Normal in some patients
II. PERICARDIAL EFFUSION
Definition
- ↑ production of pericardial fluid by inflammatory processes
(exudate) - ↓ reabsorption due to a ↑systemic venous pressure (transudate)
II. PERICARDIAL EFFUSION
Classification
Onset
− Acute or subacute
− Chronic >3 months
Distribution
− Circumferential
− Loculated
Haemodynamic impact
− None
− Cardiac tamponade
− Effusive-constrictive
Size (semiquantitative echo)
− Mild (<10 mm)
− Moderate (10–20 mm)
− Large (>20 mm)
II. PERICARDIAL EFFUSION
Clinical aspect
- Acute effusive pericarditis without compression of the heart → See previous text for clinical aspect
- Pericardial effusions within specific etiologies have the clinical aspect of the basic etiology
- Cardiac tamponade → See next text
ACUTE PERICARDITIS
Fibrinous or Effusive
Without compression of the heart
(3) Ecg - dynamic changes
- PR depression
- ST pattern - I. ST elevation +Twave, STE without ST
- depression, in all
(dynamic) leads, except V1 and aVR - II. Isoelectric ST segment and flattened T wave
- III. Inverted T waves later
- IV. normalisation Ecg
- ST-T Changes
- QRS ± reduction in voltage of the QRS complexes
- electrical alternans (in Cardiac Tamponade)
- Rhythm disorders: sinus tachycardia (AFi, AFl)
- Conduction disorders: usually with myocarditis
III. CARDIAC TAMPONADE
Definition:
Large Effusive Pericarditis that compress the heart and affect ventricular diastolic filling with severe hemodynamic consequences.
!! A Lifethreatening cardiovascular emergency
ECHOCARDIOGRAPHY
↑ Specificity, Sensibility →“gold standard”
has replaced other methods
• M mode detects quantity of fluid as small as 20 ml
• 2D detects circumferential / localized pericardial effusion
Detects
• Variable amount of Pericardial effusion (if present)
An ECHO-FREE space between the epicardium and
pericardium behind LV, in front of RV
( !!!! If this space is small and only in systole =
PHYSIOLOGICAL)
- If large fluid present →swinging heart
- Thickened and hyper-reflective pericardial layers
- Wall motion abnormalities in myo-pericarditis
- Normal in some patients
II. PERICARDIAL EFFUSION
Definition
- ↑ production of pericardial fluid by inflammatory processes
(exudate) - ↓ reabsorption due to a ↑systemic venous pressure (transudate)
CARDIAC TAMPONADE
Beck’s Triad
suggest Diagnostic Suspicion
• ↓ arterial pressure→ hypotension
• rising systemic venous pressure ( jugular veins distension)
• heart “small and calm” (↓ intensity of the cardiac sound)
II. PERICARDIAL EFFUSION
Clinical aspect
- Acute effusive pericarditis without compression of the heart → See previous text for clinical aspect
- Pericardial effusions within specific etiologies have the clinical aspect of the basic etiology
- Cardiac tamponade → See next text
Pericarditis ≠ pericardial effusion
Pericarditis is a clinical diagnosis that cannot be made
independently by echocardiagraphy
Pericardial effusion diagnosis can be made independently by echocardiagraphy
CARDIAC TAMPONADE
“Empty” Lung (=without rales)
+ Signs /Symptoms /Ecg / Rx described previously
CARDIAC TAMPONADE
Clinical Findings determined by
- Low cardiac output
2. Hypotension
CARDIAC TAMPONADE
Diagnostic Confirmation
Echocardiography
- Echo-free space posterior to the LV > 20 mm
- Hyperkinetic heart
- RV- Diastolic collapse
- RA - systolic collapse >1⁄3 systole
- Variation with respiration of ventricular diameters
( ↓ RV in expiration )
Reciprocal respiratory changes in RV and LV volumes (septal shifting)
Reciprocal respiratory changes (>25%) in RV and LV filling
6. Reduced early-diastolic tissue Doppler velocity
7. Paradoxical septal motion
8. Severe dilation of the inferior vena cava
ALTERNATING PULSE
regular alternation of weak and strong beats
without changes in cycle length because of variation of RV debit and filling of LV
IV. CONSTRICTIVE PERICARDITIS
Etiology
- Tuberculosis (frequent)
* Other
IV. CONSTRICTIVE PERICARDITIS
CLINICAL syndrome → almost like Cardiac Tamponade
SISTEMIC CONGESTION determined by
• diastolic filling
• pressures in RV, RA, cava veins
Without a great quantity of fluid
Right Heart Failure • RHF Signs • RHF complications: - Hepatic Cirrhosis - Effusive enteropathy - Nefrotic Syndrome - Renal Veins Thrombosis
LOW CARDIAC OUTPUT Syndrome
- Fatigue
- Low BP
- Cardiac cashecsia
- Dyspnea during exertion
IV. CONSTRICTIVE PERICARDITIS
Diagnostic
Clinical Suspicion
►small heart with signs of global Heart Failure
IV. CONSTRICTIVE PERICARDITIS
Diagnostic
Characteristic elements:
- Kussmaul sign ( ↑vv with inspiration)
- Pericardial knock after S2
- Apical pulse is reduced and retracts in systole
- Paradoxical pulse (1/3 )
IV. CONSTRICTIVE PERICARDITIS
Radiology:
“small heart”, thickened pericardium
IV. CONSTRICTIVE PERICARDITIS
Jugular venous pulse:
“square root” sign ( Y)
IV. CONSTRICTIVE PERICARDITIS
Echocardiography
Thickened pericardium , ↓ Diastolic ventricular
filling (+/- diastolic collapse of RV)
IV. CONSTRICTIVE PERICARDITIS
Diagnostic
- Clinical Suspicion
- Characteristic elements
- Radiology
- Jugular venous pulse
- Echocardiography
- CT, CMR definitive confirmation
SPECIFIC DISORDERS OF MYOCARDIUM
Definition
Heterogeneous group of myocardial diseases associated with
Mechanical dysfunction or
Electrical dysfunction
‒ Characterized by Hypertrophy or Inadequate Dilation → usual (but not mandatory)
‒ multiple causes → most commonly genetic often
‒ generates: progressive heart failure and death
SPECIFIC DISORDERS OF MYOCARDIUM
Diagnosis made by exclusion
NOT SECONDARY to the following etiologies:
- Hypertensive
- Valvular
- Pericardial
- Ischemic
Cardiomyopathy syndrome
Signs and symptoms determined by the primary
myocardial involvement with wide etiology: idiopathic,
genetic, inflammatory, infectious
SPECIFIC DISORDERS OF MYOCARDIUM
Categories
Primary cardiomyopathies: predominantly confined to heart muscle
- Inflammatory diseases → MYOCARDITIS Acute/Chronic
- Non-inflammatory diseases→ CARDIOMYOPATIES
- Dilated (DCM)
- Hypertrophic (HCM)
- Restrictive (RCM); Infiltrative, endomyocardial fibrosis
Other: alcoholic, metabolic, toxic, peripartum, Tachycardia-related cardiomyopathy, Left ventricular noncompaction (LVNC),
Arrhythmogenic right ventricular cardiomyopathy (ARVC), Stress induced -TAKOTSUBO
Myocarditis
Definition
Inflammatory disease of the myocardium
diagnosed by established
Histological, Immunological and Immunohistochemical criteria
Myocarditis
Focal or Diffuse alteration of
- Myocytes: Degenerescence or/and necrosis Systolic dysf.
- Interstitial: Inflammatory infiltrate Diastolic dysfunction
!! Severity of disease depends on quantity of modifications
Myocarditis
Etiology
- Infectious
- Toxic
- Allergic
- Physical agents (radiation)
Myocarditis
Mechanism
- Invasion (Bacteria, viruses)
- Toxic (bacterial toxins)
- Auto- immune (immune response to aggression)
Myocarditis
Diagnostic
Clinical → Suspicion: epidemiological context, symptoms, signs
Paraclinic: Lab + imaging Line I (Ecg, Eco, Tn I, CMR), Line II (coro, EMB)
Myocarditis
Clinical Findings
- Asymptomatic state
2. Symptomatic state → ± Rapid evolution to DCM
Myocarditis- Clinical findings
Symptoms
- Symptoms of the underlying disease (ex: Fever, myalgia)
+ - Heat failure HF
Fatigue (← ventricular dysfunction)
Dyspnea (← Acute heart failure)
Nocturia – early sign (← low CO during day in orthostatism)
- Anterior chest pain (precordial discomfort)
- Palpitations (tachycardia, arrhythmias)
- Embolim
- Sudden cardiac death SCD
Myocarditis
Signs
Physical examination
Non-specific
- Tachycardia disproportionate to the degree of fever
- ↓ S1 intensity
- S3 – protodiastolic gallop (secondary to LV dysfunction)
- ± Cardiomegaly (↑ CMA)
- ± murmurs of Mitral or Tricuspid regurgitation
(regurgitation because of dilatation of the mitral anulus)
‒ HF signs
Myocarditis - Diagnostic
Imaging methods:
CMR – gadolinium enhancement
Myocarditis
Ecg
- Arrhythmias (any arrhythmia: non-sustained VT- common, AF)
- Conduction disturbances: heart block → bad prognosis
- Non-specific ST-T abnormalities (early)
- ST segment Depression
- Negative, flattened T waves
- Pathological Q waves (V2-V4) → doesn’t mean necrosis (infflammatory infiltrate→ interstitial fibrosis)
Myocarditis
Echocardiography
→ the most specific method – Dilation of the ventricular cavities ± – Valvular regurgitation with normal valves (MR, TR) ± – Regional and/or global wall motion abnormalities ± – Intracardiac thrombi ± – Diastolic dysfunction of LV ± – Systolic dysfunction ( ± ) – Pericardial effusion ±
Myocarditis
Chest X-ray
→ guide the physician
– Underlying disease Lesions (ex: flu→ interstitial infiltrate)
– Pulmonary congestion (heart failure)
– Cardiomegaly ±
– Sometimes: pleural and/or pericardial effusion
Myocarditis
• Laboratory
– ↑ myocardial enzymes (< 2 x TnI, TnT)
– Inflammatory syndrome
– Specific serology (ANA, anti-viral antibodies titer)
Myocarditis
• Scintigraphy:
Ga 67
Myocarditis
Myocardial Biopsy
– Accidents: perforations, arrhythmias, emboli→ SCD risk
Myocarditis
Evolution
- Healing
- Chronic myocarditis → DCM
Depends on
• Extension of lesions (quantity of lesions)
• Etiology can affect evolution some time
• Patients characteristics (immune-suppressed/
healthy patients)
Does not depends fully on etiology
Myocarditis
Complications
- Heart failure
- Arrhythmias
- SCD
- Dilated Cardiomyopathy
CARDIOMYOPHATIES
= heart muscle diseases
No universal definition of cardiomyopathy
CARDIOMYOPHATIES
Formal Definition:
Myocardial disorders in which the myocardium presents with structural and functional abnormalities in the absence of
- Coronary artery disease,
- Hypertension,
- Valvular disease, or
- Congenital heart disease sufficient to cause the observed myocardial abnormality.
CARDIOMYOPHATIES
ESC position statement- Eliott et al. Eur Heart J. 2008 ;29(2):270-6.
“A myocardial disorder in which the heart muscle is structurally and functionally abnormal, in the absence of coronary artery disease, hypertension, valvular disease and congenital heart disease sufficient to cause the observed myocardial abnormality.”
CARDIOMYOPHATIES
American Heart Association definition, Maron et al. Circulation. 2006;113:1807–1816.
“a heterogeneous group of diseases of the myocardium associated with mechanical
and/or electrical dysfunction that usually (but not invariably) exhibit inappropriate ventricular hypertrophy or dilation and are due to a variety of causes and frequently are genetic”.
CARDIOMYOPHATIES
Classification
according to ventricular morphology and pathophysiology
Dilated
• Left and/or right ventricular enlargement
• Impaired systolic function
• Heart failure
Hypertrophic
• Disproportionate LV hypertrophy (typically involving septum)
• Diastolic dysfunction
• Arrhythmias
Restrictive (infiltrative)
• Diastolic dysfunction without hypertrophy of ventricle
Other
Dilated Cardiomiopathy (DCM) Definition
“Cardiomyopathy is a disease of the heart muscle
sufficient to cause global systolic impairment”
associated with the following:
The presence of
1. Left ventricular (LV) or biventricular dilatation
2. Depressed contractility and Systolic dysfunction
(reduced EF)
3. Increased enddiastolic & endsystolic volumes
4. Increased enddiastolic pressures
5. Low cardiac otput
6. Heart failure- when adaptive mechanisms failed
In the absence of 1. abnormal loading conditions or Hypertension Valvular heart disease 2. coronary artery disease
Causes of DCM
genetic
non-genetic
The final common pathway of different aggressions on the myocardium
+ slide 25
Dilated Cardiomyopathy (DCM) • Idiopathic Dilated Cardiomyopathy
Account of 50% of DCM (etiology not detected, considered primary disorder)
• CMPs with special etiologies = have the same clinical
picture, but with variable prognosis appearance
• Globally dilated cord with Diffuse Depressed
Contractility
• Heart failure occurs when dilation adaptation is exceeded
Dilated Cardiomyopathy (DCM)
• Idiopathic Dilated Cardiomyopathy
Characteristics
SOS SLIDES : 28 ,29
Onset
• Insidious frequently
• Few clinical findings or None
Symptoms
• Fatigue → reflect Low cardiac output
• Atypical chest pain (feeling the heart)
• Palpitation determined by Tachycardia, Arrhythmias,
• Systemic, pulmonary emboli
• Sudden death (Ventricular arrhythmias)
• Syncope, Near syncope
• Effort dyspnea → → ortopnea→ reflect Heart failure
Dilated Cardiomiopathy
Clinical Findings
Physical examination
Inspection
Distented jugular veins
Peripheral cyanosis
Cheine-Stokes respiration
Palpation
Displaced apical impulse down to the left
Double apical impulse OR
Alternant pulse (severe HF) / weak pulse
Decreased pulse pressure
Low cardiac output signs –cold extremities,
Percution
• Cardiomegaly
Auscultation
• S3, S4, summation gallop
• S1 split → LBBB*
• Mitral Regurgitation – systolic murmur
• Tricuspid Regurgitation (→if biventricular CMD)
- Systolic Tricuspidian murmur
- Distented jugular vein, hepato-jugular reflux, Harzer+
• Pulmonary congestion- wet crepitant rales
Dilated Cardiomiopathy
Clinical Findings
Physical examination
Other: signs determined by complications
- Hypotension whith Low EF
- Acute hear failure ( pulmonary edema&/or cardiogenic shock
- Pulmonary congestion
- Systemic congestion (hepato-splenomegaly, edema, jaundice, jugular vein distension)
- Systemic emboli signs
DCM: Diagnostic methods
Ecg
same as myocarditis: ! LBBB, Afib/ sinus
Supraventr. Arrhithmias (20% FiA) / ventricular Arr (TVns 50%),
sinus Tahyccardia ( notspecifica)
pathologic Q wave fromV 2 - 4→ extensive fibrosis
LBBB
ST-T changes
DCM : Diagnostic methods
Ecocardiography:
- Dilated cavities
- thin walls
- diffuse hypokinesia
- EF< 40 %,
- Mitral & Tricuspid regurgitation
- intracardiac thrombi
- +/- pericardial effusion
DCM : Diagnostic methods
Chest X ray + CMR + Natriuretic peptides
Chest X ray
• Cardiomegaly, Pulmonary stasis; Pleural, pericardial effusion
CMR - late gadolinium enhancement for fibrosis detection
Natriuretic peptides
↑ NTpro BNP/ BNP
DCM : Diagnostic methods
Finding etiologies:
+ slide 32
Coronaroangiography: normal coronary arteries
EMB → reveal etiology
RESTRICTIVE CARDIOMYOPATHY
Definition
myocardial disorders characterized by
altered diastolic function (restrictive pattern)
normal or “reduced” volumes LV and RV
Enlarged atria
normal or almost normal systolic function of LV and RV
RESTRICTIVE CARDIOMYOPATHY
Causes
- Infiltrative: amyloidosis, sarcoidosis
- Non-infiltrative myocardial lesions: scleroderma, diabetes
- Tezaurismosis: hemochromatosis, glicogenosis
- Endomyocardial lesions: endomyocardial fibrosis
RESTRICTIVE CARDIOMYOPATHY
Clinical features
underlying disease (1-4) sign & symptoms +
- Fatigue, Dyspnea, effort intolerance
- Effort angina
- Syncope
- Palpitation: AFib
- Right HF→ edema, ascites, distented JV
- S3 or S4
- Regurgitation of a-v valves (mitral & tricuspid)
- Kussmaul sign (inspiration)
- Cardiomegaly, palpable apical impulse
RESTRICTIVE CARDIOMYOPATHY
Diagnostic
• Ecocardiography • Impaired diastolic function • Dilated atria • Cardiac cavities: − ↓ LV, dilated RV • Ventricular walls : ± thickened • Systolic function: − initially=normal, ↓ as the disease progress • Valve Regurgitation: Mi, Tri • Pulmonary hypertension • Cardiac Cateterism, CT, CMR • Biologic: BNP x 5 (compared to constrictive pericarditis) • EMB → etiology
RESTRICTIVE CARDIOMYOPATHY
Differential diagnosis
with constrictive pericarditis
Genetic Primary CMP
HYPERTROPHIC CARDIOMYOPATHY
Definition: Genetic disease with
• Left ventricular Hypertrophy without obvious cause
• excessively hypertrophy+ Disarrangement of cardiac muscle cells
• Increased Stiffness of the hypertrophied muscle
→This results in Increased Diastolic Filling Pressures, abnormal diastolic function
HYPERTROPHIC CARDIOMYOPATHY
Classification
- Symmetrical → concentric involvement of IVS & LVPW (post wall)
- Asymmetrical → preferential hypertrophy of the IVS (septum)
- Obstructive
- Non-obstructive
HYPERTROPHIC CARDIOMYOPATHY
Etiology
Genetic disorder with various phenotypic pattern
- autosomal-dominant transmission ( positive family history)
- gene Mutations
HYPERTROPHIC CARDIOMYOPATHY
Histology + Other abnormalities
Histology
• Left ventricular hypertrophy → hypertrophied muscle
• Small ventricular cavities
• Atria – dilated / hypertrophied
Other abnormalities:
• Elongation of mitral valve
• Abnormal insertion of papillary muscles
HYPERTROPHIC CARDIOMYOPATHY
Microscopic + Physiopathology
Myocardial hypertrophy
• Disorganized arrangement of cardiac muscle cells
• Disorganization of the myofibrillar architecture
• Myocardial fibrosis
• Thickening of the small intramural coronary arteries
Physiopathology
Systole - small left ventricular outflow- obstruction
Diastole - Increased stiffness → elevated diastolic filling pressures
HYPERTROPHIC CARDIOMYOPATHY
Symptoms
- Asymptomatic OR
- Symptomatic
• Sudden death
• Syncope, Presyncope ( effort → low cardiac output)
• Palpitations (arrhythmias)
• Effort angina pectoris, Acute myocardial infaction
(relative ischemia, stable supply, increased demand)
• Dizziness
• Effort dyspnea, orthopnea
• Acute pulmonary edema
Heterogeneous clinical expression
Variable natural history
HYPERTROPHIC CARDIOMYOPATHY
Physical examination
CMH without obstruction
1. Double or triple apical impulse (palpation) lateraly
displaced, strong (Ventricular contraction, presisitolic atrial contraction)
2. S4 OR S4+S3 auscultation
3. Pulse: bisferian
4. S1 N/ ↑
5. S2 Physiologically split or Paradoxically split (LBBB)
HCM with Obstruction = as previous +
• Systolic murmur determined by obstruction (↑ gradient)
– Usually begins well after S1
– Harsh, diamond-shaped
– Best heard at the lower left sternal border as well as at the apex
– The intensity depends on the gradient
– It ends before S2
– Without irradiation on carotid arteries
HYPERTROPHIC CARDIOMYOPATHY
Physical examination
Maneuvers for differential diagnosis
− ↑ murmur: (↓ PREload -> ↑ gradient)
Valsalva, in time or immediately after exertion, in orthostatism
sudden hangs from Squatting to- Orthostatic
(↓ POST)
Inhalation of amyl nitrite
− ↓ murmur: squatting ↑ PRE , shake hand ↑ POST load
sudden Ortho after squat ↑ PRE ↑ POST
+ Associated Systolic murmur det. by Mitral Regurgitation
HYPERTROPHIC CARDIOMYOPATHY
Diagnosis
Ecg
• Normal ECG 15 %
• LVH → tall QRS complexes
• Pathological Q wave inferior and lateral, Large septal Q waves
• LAH 30 %
• Giant negative T waves (-)
• Arrhythmias: non-sustained VT, AF, SVT, WPW
can help, but in nondiagnostic
HYPERTROPHIC CARDIOMYOPATHY
Echocardiography
• LV Hypertrophy
SIV, PP > 15 mm
Asymmetric: SIV / PP >1,3
- Diastolic dysfunction
- N / ↑ Systolic function
- Obstructive forms: SAM of AMV, mitral regurgitation
SAM-systolic mitral movement
AMV- anterior mitral valve
OTHER CARDIOMYOPATHIES
Left ventricular noncompaction cmp
(LVNC)
Definition
Structural LV abnormalities
Represents the failure of the trabecular or spongiform layer of the myocardium to compact
Characterized by decreased coronary flow reserve and a thickened, prominently trabeculated myocardium with deep recesses that communicate with the ventricular chamber
It can mimic congenital diseases
May be associated with congenital disease
Left ventricular noncompaction cmp
SIGNS AND SYMTOMS
─Asymptomatic or heart failure syndrome when evolve to DCM ─Arrhythmias, conduction abnormalities, embolism ─Due to embolism Chest pain Stroke Abdominal pain- mesenteric infarct Peripheral embolism ─SCD due to ventricular arrhythmias
Arrhythmogenic right ventricular cardiomyopathy (ARVC)
Definition
“is a genetically determined cardiomyopathy characterized by fibro-fatty
replacement of the myocardium
biventricular involvement occurs in up to 50% of cases
a small proportion of cases affect predominantly the left ventricle”
Arrhythmogenic right ventricular cardiomyopathy (ARVC)
Three stages:
- Early subclinical phase
Imaging studies are negative
Sudden cardiac death can still occur - A phase in which
RV abnormalities (usually) are obvious
Without any clinical manifestation of RV dysfunction
Development of a symptomatic ventricular arrhythmia - Progressive fibrofatty replacement and infiltration of the myocardium
Severe RV dilation & associated right-sided heart failure
Aneurysm formation - LV dilation and failure may arise
Arrhythmogenic right ventricular cardiomyopathy (ARVC)
ELECTRICAL MANIFESTATIONS
Early stage
• fatal arrhythmia due to slow conduction and electrical uncoupling
Further progressive fibrofatty infiltration results in
Inhomogeneous activation and a further delay in conduction
Typical monomorphic ventricular tachycardia (VT) characterized with LBBB aspect and typical T wave inversions extending to V3 or beyond.
“epsilon wave” in the right precordial leads is specific but insensitive
Arrhythmogenic right ventricular cardiomyopathy (ARVC)
SIGN & SIMPTOMS
~ Miocarditis
Palpitation, syncope (during effort)
Cardiac arrest or SCD
Heart failure symptoms if LV involvement coexist
Stress induced –
TAKO TSUBO cardiomyopathy
DEFINITION:
is an acute, reversible stress-induced cardiomyopathy that affect the LV apex (resulting in the synonym of “apical ballooning syndrome” , but not only
wall motion abnormalities without pattern of a single coronary artery distribution
coronary angiography: without acute coronary obstruction
occurs typically in older women after sudden intense emotional or physical stress
Arrhythmogenic right ventricular cardiomyopathy (ARVC)
PRESENTATIONS
+ slide 41
Recent emotional stress with Anterior chest pain Dyspnea of various degree Palpitation Anxiety/ depression Pulmonary edema Hypotension ECG changes mimic acute infarction
Physical exam
LV heart failure
Ejection systolic murmur ~ hypertrophic cardiomyopathy
