Download cardiomyopathies

Myocardial and Pericardial Disease
J.B Handler, M.D.
Physician Assistant Program
University of New England
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Abbreviations
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LV/RV- left ventricle/right ventricle
EF- ejection fraction
IVCD-intraventricular conduction
delay
MR- mitral regurgitation
LVOT- left ventricular outflow tract
SAM- systolic anterior motion
IVS- interventricular septum
DHP- dihydropyridine
Rx- treatment
Bx- biopsy
FH- family history
HJR- hepato-jugular reflux
MVO2- myocardial oxygen
consumption
OP- out patient
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Nl- normal
LSB- left sternal border
PND- paroxysmal nocturnal dyspnea
JVD- jugular venous distention
RA- rheumatoid arthritis
SLE- systemic lupus erythematosis
Dx- diagnosis
ARB’s- angiotensin receptor
blockers
ICD- inplantable cardioverterdefibrillator
HF- heart failure
HF=CHF- congestive heart failure
CHD- coronary heart disease
ASH- asymmetric septal
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hypertrophy
Dilated Cardiomyopathy
Primary: idiopathic - unknown cause
 Secondary:
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Toxic - alcohol, adriamycin, etc.
Post-partum
Post infectious - myocarditis
Endocrine: hypothyroidism, pheochromocytoma,
acromegaly and hyperthyroidism
– “Ischemic Cardiomyopathy”- avoid this terminology
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Clinical Features
Patients present with signs and symptoms of HF
which usually develops slowly.
 Left or biventricular failure
 Left sided: DOE, orthopnea, PND, weakness,
fatigue, peripheral edema, etc.
 Right sided: unexplained weight gain, peripheral
edema, abdominal fullness (hepatomegaly,
ascites).
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Dilated Cardiomyopathy
Physical Exam
Cardiomegaly (PMI displaced laterally), low
pulse amplitude (pulsus alternans when severe),
often with BP, pulmonary congestion, crackles,
S3 gallop, MR murmur.
 Elevated JVP, hepatomegaly, HJR, pitting edema,
TR murmur.
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Diagnostic Studies
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CxR: Cardiomegaly, pulmonary congestion,
pleural effusions.
Echocardiography/Doppler: LV/RV dilation,
global LV dysfunction with reduced EF; Mitral
regurgitation common.
EKG- NSST-T changes, IVCD (wide QRS),
PVC’s.
Cardiac Cath: only when necessary to exclude
alternative diagnosis i.e CHD; documents low
EF, global dysfunction, high filling pressures.
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Treatment
Management for HF:
 Afterload reduction: ACEI or alternatives (ARB’s)
 Preload reduction: Diuretics, nitrates
 Beta Blockers
 Spironlolactone
 Digoxin
 ICD’s if indicated, +/- antiarrhythmics
 Anticoagulation unless contraindicated
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Clinical Course and Prognosis
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Dependent on length of Sx and functional class. If
onset recent, some recovery of ventricular
function can occur.
Class IV patients: 50% one year mortality
Unpredictable course, often progressive
Only meds that may improve survival are
ACEI (or ARB’s), ß-Blockers and
Spironolactone.
Cardiac Transplantation: >70% 5yr. survival
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Hypertrophic Cardiomyopathy
Genetically transmitted in >50% of cases.
 Autosomal dominant with high penetrance.
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– Must perform echocardiography on all siblings and
offspring of a patient with HCM.
Remaining cases occur spontaneously; de novo
gene mutations common.
 Genetic counseling is essential.
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Pathophysiology of HCM
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Marked increase in left ventricular mass, especially the
septum - marked hypertrophy; remaining LV segments
hypertrophied to a lesser degree; septal/posterior wall
thickness > 1.5/1 “ASH”. Hypertrophy is unrelated to
pressure overload; often present at birth, progessively
worsens during childhood.
LV cavity small, systolic function normal or
hyperdynamic early on.
Diastolic dysfunction common
Obstructive and non-obstructive forms
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HCM
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When present LVOT obstruction is dynamic and
varies with activity/rest, and LV volume.
– Obstruction: MV moves abnormally towards the IVS,
obstructing the LVOT.
Pathology: myocardial fiber hypertrophy and
disarray, primarily in IVS.
 Mitral valve often thickened and moves
abnormally as noted above, well seen on
echocardiogram.
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Clinical Manifestations
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Often asymptomatic in childhood; may be detected via
ultrasound in the offspring of patients with known
disease.
Symptoms: dyspnea, chest pain and syncope are most
common. In some, sudden death may be presenting
symptom. One of few causes of sudden death in young
athletes.
Sudden death often occurs during strenuous activity.
Arrhythmias are common: ventricular and
supraventricular; Afib may lead to sudden
decompensation and is a bad prognostic sign.
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Physical Exam
Pulse brisk, often. with bisferiens carotid pulse.
 Double or triple apical impulse due to atrial filling
wave and early and late systolic impulses.
 Loud S4 and S3 gallops.
 Loud harsh aortic outflow murmur (creshendodecreshendo) best heard along left sternal border
with characteristic features (see below); MR
common.
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Effects of Maneuvers on Murmur
Most cardiac murmurs are increased by squatting
and decreased by standing or with valsalva.
 The murmur of HCM is increased with
standing & valsalva and decreased with
squatting. This is the opposite of how the
murmur of aortic stenosis acts. Other things that
 the murmur include hypovolemia, tachycardia
or increases in cardiac contractility (inotropes,
exercise).
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Diagnostic Studies
ECG- LVH with secondary ST-T changes
common. Septal Q waves may mimic MI.
 Echocardiogram/Doppler diagnostic
 CxR often unimpressive.
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HCM: Management
Essential to minimize strenuous physical exertion.
 Beta Blockers: slow HR, ’s diastolic filling
time, ’s MVO2 with additional anti-arrhythmic
effects:
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– Angina, dyspnea and presyncope may all improve.
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Beta blockers also may prevent the increase in
outflow obstruction that occurs with exercise.
Large doses of ß-blockers well tolerated.
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HCM: Management
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Calcium channel antagonists (verapamil)- used
with (or as an alternative) to ß-blockers.
– Only time when verapamil added to ß-blocker
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Improve diastolic filling and compliance via
negative inotropic and chronotropic effects
decrease LVEDP.
– Symptomatic improvement and improved exercise
tolerance in over 2/3 of treated patients.
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Verapamil in high dosage is well tolerated.
Don’t use DHP Ca blockers can worsen Sx.
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HCM: Interventional Therapy
Surgery or procedures to reduce septal muscle:
myomectomy, alcohol ablation: severe
obstruction or symptoms.
 Dual chamber pacemaker may improve septal
motion and decrease progression of obstruction if
severe.
 ICD: high risk patients (documented v-tach,
aborted sudden death) or FH of sudden death.
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Restrictive and Infiltrative
Cardiomyopathies
Hallmark: Abnormal diastolic function.
 Ventricular walls excessively rigid and impede
diastolic filling; systolic function may be normal
or reduced.
 Pathophysiology resembles constrictive
pericarditis.
 Least commonly seen of the cardiomyopathies.
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Restrictive Cardiomyopathy:
Findings
Jugular venous distention
 S3 and/or S4
 Inspiratory increase in venous pressure
(Kussmaul’s sign)
 Findings of Rt. Heart Failure may predominate
i.e. edema, hepatomegaly.
 Symptoms include dyspnea, exercise intolerance
and fatigue.
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Restrictive
Cardiomyopathy
Echo-doppler findings include: LV wall
thickening; decreased diastolic relaxation.
 Systolic function-preserved or diminished
 Disease process may have characteristic echo
findings i.e. amyloidosis.
 Tricuspid and mitral regurgitation are common.
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Natural History
Relentless symptomatic progression; >90% dead
at 10 years.
 No specific treatment other than symptomatic.
 Calcium Channel Antagonists may improve
diastolic function in selected individuals.
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Etiologies
Amyloidosis
 Hemochromatosis
 Fabry Disease
 Gaucher Disease
 Endomyocardial Fibrosis-Loeffler Endocarditishypereosinofilia syndrome
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Myocarditis
A primary inflammatory process of the
myocardium, most often caused by an
infectious agent.
 Unrecognized myocarditis may be the initial
event culminating in an “idiopathic dilated
cardiomyopathy”.
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Infectious Myocarditis
Viral - most common
 Bacterial- numerous
 Fungal-aspergillosis, candidiasis, etc.
 Parasitic – Trypanosoma cruzi (Chagas’)
 Rickettsial
 Spirochetal
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Viral Myocarditis and Pericarditis
Coxsackievirus (B>A)
 CMV
 Echovirus
 Adenovirus
 HIV
 Influenza
 Infectious mononucleosis
 Rubella, Rubeola
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Clinical Manifestations
May be asymptomatic
 Prodromal viral syndrome followed
symptoms of myopericarditis.
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– Chest pain, fatigue, dyspnea, palpitations are
common initial symptoms. Often progresses to
HF.
Initial presentation may be HF.
 Exam : Tachycardia, elevated temp, muffled
heart sounds; signs of HF in severe cases.
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ECG: sinus tachycardia with NSST-T changes.
Other findings: ST elevation consistent with
pericarditis may occur.
CXR: heart size normal or enlarged; pulmonary
congestion may be present.
Echo-doppler: some degree of LV dysfunction
(regional or global); LV size normal or increased,
wall thickness usually normal. Thrombus may be
present with severe dysfunction.
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Diagnosis
Difficult to confirm acute diagnosis
 Acute and convalescent viral titers; presence of
virus in other tissues.
 Troponin levels or CK-MB isoenzyme are often
normal but may be mildly elevated.
 Endomyocardial biopsy may isolate virus (rare),
or show characteristic pathology of myositisinflammatory infiltrate.
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Treatment
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Supportive Rx
Rx HF if present. Important to limit activity.
Specific anti microbial Rx if an infecting agent is
identified (if treatable).
Biopsy guided immunosuppressive and
corticosteroid Rx is available under investigative
protocols - no proven benefit.
Avoid NSAIDs: may increase myocardial damage.
Prognosis variable: Ranges from death to varying
degrees of recovery
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Acute Pericarditis
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A syndrome due to inflammation of the
pericardium characterized by chest pain, a
pericardial friction rub, and serial ECG
abnormalities.
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Causes of Pericarditis
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Viral most common; same spectrum of
viruses as seen with myocarditis- Coxsackie
most common.
Idiopathic (non specific)
Tuberculosis
Acute bacterial infections
Fungal
Uremia-untreated or with dialysis.
Radiation
Autoimmune-RA, SLE, scleroderma, PAN
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Drug induced: hydralazine, procainamide,
isoniazid, penicillin.
Trauma: Chest trauma; post thoracotomy;
pacemaker insertion; post cath., PTCA.
Early post MI
Delayed post myocardial-pericardial injury
syndromes: late post MI (Dressler’s syndrome) or
post heart surgery (postpericardotomy syndrome).
Neoplastic disease- lung cancer, breast cancer,
lymphoma, Hodgkins disease, leukemia.
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Clinical Manifestations
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Chest pain-frequent; quality and location variable;
retrosternal and often left sided. Pain is intenseaggravated by lying supine, with inspiration,
coughing, swallowing, laughing; improved
sitting up, leaning forward, shallow inspiration.
Chest pain may be felt with each heart beat.
On occasion pain may be identical in quality to the
pain of myocardial infarction.
Dyspnea may be related to shallow breathing from
inspiratory chest pain.
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Pericarditis: Physical Exam
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Pericardial Friction Rub-pathognomonicscratching, grating, high pitched sound due to
friction between the pericardium and epicardium.
3 components related to cardiac motion: presystole (atrial contraction), ventricular systole
(loudest component), and early diastole. Usually
hear 2 components (S/D).
Rub is evanescent; best heard with diaphragm at
LLSB; best heard with patient sitting, leaning
forward in full expiration.
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Diagnostic Findings
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ECG: Changes begin within hours of the onset of
pain; several stages.
ECG abnormalities reflect inflammation involving
the pericardium and epicardium. Initially there is
diffuse ST segment elevation in all leads except
aVR and V1. Later ECG’s show normalization of
ST elevation followed by T wave flattening and T
wave inversion.
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ECG Pericarditis
Diagnostic Findings Cont....
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Early repolarization, a normal variant often seen in
young healthy individuals (male> female) may
mimic the ECG findings seen in the acute phase of
pericarditis, but less ST  & seen in fewer leads.
Echo-doppler: nl LV size and function rules out
myocarditis; a small pericardial effusion may be
seen; this rarely progresses with viral pericarditis,
but a repeat study to document resolution is
indicated.
CXR: usually normal; occasionally cardiomegaly
due to a pericardial effusion is seen.
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Management
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Determine etiology where possible.
Bed rest until pain and fever resolved.
Most patients Rx’d as OP. Hospitalization may be
indicated if MI suspected or if large effusion present.
Pain rapidly responds to NSAIDs: Ibuprofen, high dose
ASA, etc; steroids rarely necessary.
Oral anticoagulants should be avoided in patients with
pericarditis.
Symptoms usually resolve in 2-4 weeks.
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Pericardial Effusion Without Cardiac
Compression
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Can occur with all forms of pericarditis
Symptoms (if present) include chest pressure,
dyspnea, hiccups, nausea, abd. fullness, cough.
CxR-mild cardiomegaly if >250 cc. fluid.
ECG: NSST-T changes; decreased QRS voltage.
Echo: Best technique to Dx. and follow; useful to
determine presence of tamponade.
Management-depends on the presence or absence
of hemodynamic compromise, and the underlying
disease process.
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Pericardial Effusion With
Compression: Tamponade
Increasing pericardial fluid raises intrapericardial
pressure resulting in compression of the heart.
 There is progressive limitation of ventricular
diastolic filling leading to reduction of stroke
volume and cardiac output.
 Fatal if not recognized and aggressively treated.
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Cardiac Tamponade
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Hemodynamics - marked elevation and
equilibration of LV and RV diastolic pressures;
LA and RA pressures elevated.
Marked decrease in CO.
RA and RV collapse is seen on echo.
Beck’s triad: decline in arterial pressure,
elevation of systemic venous pressure, quiet heart.
Cardiac output is extremely volume sensitive.
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Pulsus Paradoxus
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Normally during inspiration-increase venous
return, slight increase in RV volume, IVS
displaced from rt to lt- slight decrease in LV
volume. RV output increases, LV output slightly
falls- results in minimal (2-3%) drop in systolic
BP (2-4mm).
Pulsus paradoxus is a marked exaggeration of
this process; intrapericardial pressure is markedly
increased-RV and LV volumes are already
diminished; inspiration results in a marked  in
LV volume resulting in a systolic BP drop >
10mm.
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Cardiac Tamponade
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CxR and ECG are ancillary tests.
Echocardiogram is often diagnostic.
Pericardiocentesis: may be life saving; IV fluids given
to increase preload; should be done with Rt Ht Cath to
optimize hemodynamics; subxiphoid approach with
flouroscopic guidance is successful in 95%; fluid is
cultured and sent for cytology and chemistry analysis.
Surgery: Pericardectomy and pericardotomy are
necessary in 25% for recurrent tamponade.
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