Dilated Cardiomyopathy Daily Report Jafer Jeelani

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Dilated CardiomyopathyDaily Report Jafer
Jeelani
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• Dilated Cardomyopathy (DM) dilation and impaired
  contraction of one/both ventricles.
• Diagnosis requires evidence of dilatation and
  impaired contraction of either Left or both
  ventricles
• EFlt40 percent
• fractional shortening less than 25 percent

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• Why is it important
• Responsible for
• 10,000 deaths annually
• 46,000 hospitalizations annually.
• Affects 2-3 million people in US
• Incidence of CHF (largely dilated cardiomyopathy)
  is 400,000 cases per year.
• Framingham study found that 5 years after initial
  presentation of CHF, 42 of women and 62 of men
  had died.

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• Pahophysiology
• Cardiomegaly-LV/biventricular dilatation.
• Decreased systolic function
• Reduced LV contraction ability ? decreased
  cardiac output ? increased residual volumes in
  end-systole and end-diastole.

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• Symptoms
• Fatigue
• Dyspnea on exertion
• Shortness of breath
• Orthopnea, paroxysmal nocturnal dyspnea
• Decreased exercise tolerance
• Peripheral edema
• Thromboembolic complications
• Chest pain
• Syncope

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• Physical exam
• Tachypnea
• Tachycardia
• Hypertension
• Signs of hypoxia
• Jugular venous distension (JVD)
• Pulmonary edema (crackles and/or wheezes)
• S3 gallop
• Enlarged liver or hepatojugular reflex
• Peripheral edema
• Hepatomegaly
• Splenomegaly
• Displaced PMI

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• Complications
• Heart failure
• Atrial or ventricular arrythmias
• Sudden death

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• Necessary information
• Age
• Sex
• Race
• Medical history, especially the following
• Hypertension
• Angina
• Coronary artery disease
• Anemia
• Thyroid dysfunction
• Breast cancer
• Medications
• Social history (eg, tobacco, alcohol, illicit
  drug use)

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• Epidemiology
• Range children to elderly
• Most pts present b/w ages 20-60
• Men 3x women
• Black 3x white

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• Causes
• Idiopathic 50 percent
• Myocarditis 9 percent
• Ischemic heart disease 7 percent
• Infiltrative disease 5 percent
• Peripartum cardiomyopathy 4 percent
• Hypertension 4 percent
• HIV infection 4 percent
• Connective tissue disease 3 percent
• Substance abuse 3 percent
• Doxorubicin 1 percent
• Other 10 percent

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• Causes continued w/ focused selections
• Idiopathic Dilated Cardiomyopathy
• considered idiopathic if
• exclusion of 50 percent obstruction of one or
  more coronaries
• r/o active myocarditis
• r/o primary/secondary forms/causes of heart
  muscle disease
• 30 of idiopathic DCM cases have a familial
  distribution.
• Association w/ the dystrophin gene
• Which causes X-linked dilated cardiomyopathy

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• Ischemia
• overall CAD in the most common cause of
  cardiomyopathy in US but is responsible for only
  7 of cases for DCM
• Stress-induced cardiomyopathy
• Uncommon ST elevation ACS in absence of critical
  CAD
• Aka transient left ventricular apical ballooning
• takotsubo cardiomyopathy
• broken heart syndrome
• brought-out on by intense psychologic stress
• occurring mostly in postmenopausal women.
• transient left ventricular apical ballooning
• apical ballooning is seen on left
  ventriculography or echocardiography
• Complications
• hemodynamic instability/cardiogenic shock.

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• Infectious cardiomyopathy
• variety of infections can lead to myocarditis and
  DCM
• Most common cause of infectious myocarditis is
  viral
• Ex Coxsackievirus, influenza virus, adenovirus,
  echovirus, cytomegalovirus, and human
  immunodeficiency virus (HIV).
• Pathogenesis if immune response is not
  sufficient myocyte injury occurs via
• Direct cytotoxicity
• Persistent immune response against viral genomic
  fragments.
• HIV infection
• Heart disease secondary to HIV is increasing in
  frequency.
• Mechanisms drug toxicity, secondary infections,
  myocyte damage by HIV. Autoimmune response
  induced by HIV or other viruses ex
  coxsackievirus,CMV, EBV

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• Chagas' disease
• infection from Trypanosoma cruzi
• leading cause of DCM in Central and South
  America.
• Characterized by acute myocarditis, cardiac
  enlargement, tachycardia, and nonspecific ECG
  abnormalities including RBBB and PVCs.
• LV apical aneurysms pathognomonic.
• Toxic cardiomyopathy
• Secondary to alcohol, cocaine, medications (esp.
  chemotherapeutic drugs), radiation and many
  others.

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• Alcohol
• Leading cause of DCM.
• prevalance of ACM is variable and ranges from 23
  to 40 (CHEST)
• Direct toxicity to myocytes (oxygen free
  radicals, defects in protein synthesis)
• Risk related to mean daily intake and duration.
• Individual susceptibility is still a big factor
• Occurs in 2 stages (CHEST)
• Asymptomatic
• Consumption of gt 90 g of alcohol a day
• (seven to eight standard drinks per day)
• for gt 5 years are at risk for development of
  asymptomatic ACM
• symptomatic is anything in excess of above

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Impact of Alcohol on survival

• Survival curves of cardiac deaths in male
  patients with ACM and IDCM. The solid line
  indicates patients with ACM and alcohol
  abstinence, the small dashed line indicates IDCM,
  and the large dashed line indicates patients with
  ACM without abstinence.

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Hypothetical scheme of pathogenesis of ACM
(CHEST)
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• Cocaine
• relationship less well understood than between
  cocaine and coronary ischemia.
• Mechanisms direct toxic effect, the
  cocaine-induced hyperadrenergic state, and
  infectious cardiomyopathy (parenteral abuse).
• Abstinence leads to reversal of the myocardial
  dysfunction.
• End-stage renal disease
• Pts w/ ESRD on HD can develop Dilated CM via an
  uncertain mechanism.
• Obstructive sleep apnea
• Impairment of LV function.
• Therapy w/ nasal CPAP can lead to significant
  improvement

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• Workup
• History
• Exam
• Labs
• EKG
• CXR
• Cardiac CATH
• CT scan
• Gated pool scan
• Biopsy

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• Workup cont
• Lab Studies
• CBC r/o anemia.
• Chem 7 assess BUN/Creat/K
• LFT may rise with hepatic congestion
• Fasting glucose screen for DM
• TFT r/o thyrotoxicosis (arrythmias) or
  hypothyroidism
• Fe/TIBC if hemochromatosis is a risk
• BNP -- gt indicator of failure
• ANA possible lupus
• Viral studies
• Urine toxicology screen

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• Other studies
• Chest x-ray
• Cardiomegaly
• Prominent upper lobe vessels (cephalization)
• Kerley B lines
• Pleural effusions
• Bats wing pulmonary edema
• Electrocardiogram
• Look for arrhythmias e.g. A fib, V Tach
• Conduction abnormalities esp. seen in pts with
  dilated cardiomyopathy BBB

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• Other studies cont
• Echocardiogram
• EF helps distinguish systolic and diastolic HF
• Regional wall motion abnormalities suggesting
  ischemia
• Valvular disease
• Pulmonary artery pressures.
• Dilated chambers/ thin walls
• Endomyocardial biopsy may be helpful in
  diagnosing myocarditis, connective tissue
  disorders, and amyloidosis.

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• Treatment
• Lifestyle changes
• Medical management
• Surgical management

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• Lifestyle
• Daily weighing
• Low salt diet
• Limit fluid intake
• Medical
• ASA
• B-Blocker
• ACE
• Spirnolactone
• Lasix
• Nitrates
• Hydralazine
• Surgical
• Cardiac resynchronization (CRT)
• Biventricular pacemaker
• Improves contraction
• Implantable cardioverter defibrillators (ICD)
• Those at risk for life threatening arrythmias or
  sudden death
• CABG for CAD, correction of valvular dz.

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