Cardiomyopathy

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Cardiomyopathy

• Craig Ernst MHS, PA-C
• Lock Haven University

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Cardiomyopathy

• General term indicating disease of cardiac muscle
  resulting in abnormal function
• Divided into three types
• Dilated cardiomyopathy-ventricular dilation
• Hypertrophic cardiomyopathy-myocardial
  hypertrophy
• Restrictive cardiomyopathy-impaired ventricular
  filling
• Can have characteristics of more than one

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Dilated Cardiomyopathy (DCM)

• Characterized by dilation and impaired systolic
  function of left /or right ventricle
• Most common DCM is ischemic cardiomyopathy
• Idiopathic (ICM) next most common
• Familial autosomal dominant in 20 of cases.
• Role of coxsackie/adenovirus and immune mediated
  etiology unknown.

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DCM

• Many cases of systemic heart muscle disease
  present with features of DCM including
• Ischemic/rheumatic CVD
• Generalized disease- hemochromatosis, sarcoid
• Connective tissue disease-SLE, systemic sclerosis
• Neuromuscular disease-Friederichs ataxia etc
• Glycogen storage disease
• Primary heart muscle disease- amyloidosis
• Alcohol excess
• Cytotoxic drugs-doxorubicin, cyclophosphamide
• Pregnancy

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Starling Curve
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Starling Curve
Contractility
Volume
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DCM

• Clinical features
• R/L heart failure
• Arrhythmia
• Emboli
• Cardiomegaly
• Tachycardia
• JVD
• 3rd/4th heart sounds
• basiler crackles
• displaced PMI

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DCM

• Evaluation
• CXR cardiomegaly
• EKG diffuse non specific ST-T wave changes,
  LBBB common, tachycardia, conduction
  abnormalities, arrhythmias
• Echo poor chamber contraction and dilated
  chambers
• If CAD suspected, cardiac catheterization
• Endomyocardial biopsy for research only.

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DCM

• Treatment
• Rx failure arrhythmias
• Ace Inhibitors a must in failure management
• Non-specific Beta blockade
• Carvedilol, ??, ??
• Anticoagulation for A.fib/mural thrombus.
• CRT-D (Bi-V AICD)
• Transplant
• Sudden death
• Due to V. Tach. or V. Fib

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Hypertrophic Cardiomyopathy (HCM)

• Complex heart disease due to the asymmetric left
  ventricular hypertrophy, left ventricular
  stiffness, mitral valve changes and cellular
  changes (myocardial disarray)
• 60 inherited 40 sporadic
• HTN, Aging,
  Unknown
• Most autosomal dominant w/ variable penetrance

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Other Terms

• Hypertrophic Obstructive Cardiomyopathy (HOCM)
• Idiopathic hypertrophic subaortic stenosis (IHSS)
• Asymmetrical septal hypertrophy (ASH)
• Systolic anterior motion (SAM) of mitral
  apparatus

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Pathophysiology

• HOCM is a subvalvular obstruction!
• Distinct from valvular Aortic Stenosis (pressure
  gradient across valve)
• Gradient/obstruction increases with lower LV
  volume
• HOCM pts here do better when full and slow
• Standing after squatting/Valvsalva
• lower venous return increase outflow
  obstruction and intensity of murmur

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MV leaflet
HCM without obstruction
HCM with obstruction HOCM)
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HOCM Clinical Features

• Chest pain
• Dyspnea
• Syncope/Pre-syncope (typically with exertion)
• Palpitations
• Sudden Death (arrhythmia)
• Typically occurs in asymptomatic young adults or
  adolescents (10-35 y/o)
• Family history of sudden death, sustained
  ventricular tachycardia, B/P response to
  exercise are recognized risk factors
• Diastolic dysfunction with impaired filling
• Outflow tract obstruction occurs in 1/3 of cases

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Physical Exam

• Systolic murmur with little to no radiation to
  neck vessels (increased by maneuvers that
  decrease preload such as Valvsalva or squatting)
• Decreases ventricular filling
• May hear systolic murmur of MR
• Weak late carotid pulse (late obstruction)
• Diagnosed by echocardiogram.

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Evaluation

• EKG-LVH with ST-T wave changes
• CXR-normal
• ECHO indicated if PE suggests IHSS
• Pedigree analysis (ECHO to screen 1st relatives)
• Genetic analysis
• XST/Holter

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Treatment of HOCM Relief of symptoms, prevention
of endocarditis, arrhythmias and sudden death

• B-Blockers or verapamil
• better filling, slow, bigger heart-less
  obstruction
• Amiodarone or procainamide for A Fib
• need atrial kick to adequately fill LV
• Avoid afterload reducing agents and vasodilators
  (no ACEI/A2RB, NITRO)-refractory hypotension
• These agents increase outflow obstruction
• actually do better with increased SVR
• Slow controlled emptying from increased SVR
• SBE prophylaxis

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Treatment of HOCM

• Implantable defibrillators
• may be indicated if at risk for SCD
• Dual-chamber pacemakers reverse of
  resynchronization therapy for LVEF
• Surgical myotomy myomectomy
• Non surgical ablation of the septum (alcohol
  ablation through cath)

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Sudden Cardiac Deaths

• 2-3 per year
• Sudden unexpected
• Sudden death may be the initial (only)
  presentation
• NPR link
• Risk for SCD
• Extreme LVH
• Family history of SCD
• History of Vtach or syncope
• Failure of BP to rise with exercise
• Treatments that lower gradient do not prevent SCD

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Restrictive Cardiomyopathy (RCM)

• May cause systolic diastolic dysfunction
• All increase LV stiffness
• Characteristic ventricle filling pressures
• Over time filling dramatically ceases

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Restrictive Cardiomyopathy

• Some cardiomyopathies do not present with
  dilation or hypertrophy but rather restricted
  ventricular filling (as with pericarditis)
• Amyloidosis
• Sarcoidosis
• Hemochromoatosis
• Endomyocardial fibrosis
• Atrial dilation, atrial fibrillation and clot
  formation common in restrictive

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Restrictive Cardiomyopathy

• Clinical Features
• Dyspnea
• Fatigue
• Embolic phenomena
• Elevated venous pressures
• JVD
• Hepatomegaly
• Edema
• Ascites

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Restrictive Cardiomyopathy

• CXRcardiac enlargement
• EKG-low voltage and ST-T wave abnormalities
  (Exaggerated Septal Qs Think MI)
• Echo-symmetrical myocardial thickening
• Endomyocardial biopsy may be useful.
• Is it restrictive pericarditis?

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Pre-op effusion
Post op effusion
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Restrictive Cardiomyopathy

• Treatment
• No specific treatment
• Treat underlying cause results?
• Those with amyloidosis may recur after transplant

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Amyloidosis

• Systemic disorder, but if DHF occurs usually from
  multiple myeloma
• Fibrillar protein deposited throughout the
  myocardium leading to rubbery consistency and
  concentric hypertrophy
• RV LV hypertrophy
• Absence of high voltage QRS on EKG despite LVH on
  ECHO
• Appearance on Echo

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Amyloidosis

• Fat pad aspirate or tissue biopsies for systemic
  amyloidosis
• Endomyocardial biopsy if questionable etiology
• Poor prognosis

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Hemochromatosis

• Hereditary disorder characterized by excess
  dietary iron absorption and deposition in tissues
  with resulting end-organ damage.
• Affects liver first and most frequently
• Pancreatic involvement results in DM
• Cardiac deposits leads to dilated cardiomyopathy
• Skin deposits leads to bronze discoloration that
  results from increased melanin production.
  Hyperpigmentation
• Remember liver, pancreas, heart
• Dx AST, ALT, serum iron, TIBC, ferritin

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