Myocardial%20and%20Pericardial%20Disease

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Myocardial and Pericardial Disease

• J.B Handler, M.D.
• Physician Assistant Program
• University of New England

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Abbreviations

• 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

• Nl- normal
• LSB- left sternal border
• PND- paroxysmal nocturnal dyspnea
• JVD- jugular venous distention
• RA- rheumatoid arthritis
• SLE- systemic lupus erythematosis
• Dx- diagnosis
• ARBs- angiotensin receptor blockers
• ICD- inplantable cardioverter-defibrillator
• HF- heart failure
• HFCHF- congestive heart failure
• CHD- coronary heart disease
• ASH- asymmetric septal hypertrophy

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Dilated Cardiomyopathy

• Primary idiopathic - unknown cause
• Secondary
• 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
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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

• 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), PVCs.
• 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 (ARBs)
• Preload reduction Diuretics, nitrates
• Beta Blockers
• Spironlolactone
• Digoxin
• ICDs if indicated, /- antiarrhythmics
• Anticoagulation unless contraindicated

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Clinical Course and Prognosis

• 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
  ARBs), ß-Blockers and Spironolactone.
• Cardiac Transplantation gt70 5yr. survival

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Hypertrophic Cardiomyopathy

• Genetically transmitted in gt50 of cases.
• Autosomal dominant with high penetrance.
• 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

• Marked increase in left ventricular mass,
  especially the septum - marked hypertrophy
  remaining LV segments hypertrophied to a lesser
  degree septal/posterior wall thickness gt 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

• 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
  (creshendo-decreshendo) 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
• Angina, dyspnea and presyncope may all improve.
  
• 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

• Calcium channel antagonists (verapamil)- used
  with (or as an alternative) to ß-blockers.
• Only time when verapamil added to ß-blocker
• 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.
• Verapamil in high dosage is well tolerated.
• Dont 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
  (Kussmauls sign)
• Findings of Rt. Heart Failure may predominate
  i.e. edema, hepatomegaly.
• Symptoms include dyspnea, exercise intolerance
  and fatigue.

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RestrictiveCardiomyopathy

• 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 gt90 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
  Endocarditis-hypereosinofilia 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 (BgtA)
• 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.
• 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
  myositis-inflammatory infiltrate.

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Treatment

• 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

• 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

• 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 (Dresslers syndrome) or
  post heart surgery (postpericardotomy syndrome).
• Neoplastic disease- lung cancer, breast cancer,
  lymphoma, Hodgkins disease, leukemia.

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Clinical Manifestations

• Chest pain-frequent quality and location
  variable retrosternal and often left sided. Pain
  is intense-aggravated 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

• Pericardial Friction Rub-pathognomonic-scratching,
  grating, high pitched sound due to friction
  between the pericardium and epicardium.
• 3 components related to cardiac motion
  pre-systole (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

• 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 ECGs show
  normalization of ST elevation followed by T wave
  flattening and T wave inversion.

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ECG Pericarditis
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Diagnostic Findings Cont....

• Early repolarization, a normal variant often seen
  in young healthy individuals (malegt 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

• Determine etiology where possible.
• Bed rest until pain and fever resolved.
• Most patients Rxd 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

• Can occur with all forms of pericarditis
• Symptoms (if present) include chest pressure,
  dyspnea, hiccups, nausea, abd. fullness, cough.
• CxR-mild cardiomegaly if gt250 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

• 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.
• Becks triad decline in arterial pressure,
  elevation of systemic venous pressure, quiet
  heart.
• Cardiac output is extremely volume sensitive.

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Pulsus Paradoxus

• 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 gt 10mm.

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Cardiac Tamponade

• 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.