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Updated: Nov 23 2022

Dilated Cardiomyopathy

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  • Snapshot
    • A 60-year-old man presents to his cardiologist for worsening shortness of breath and easy fatigability. He began developing symptoms a few months prior to presentation. His shortness of breath is most significant with exertion and he notes having swollen feet. His medical history includes hypertension, hyperlipidemia, alcohol use disorder. He currently takes hydrochlorothiazide and atorvastatin. On physical exam, an S3 heart sound and a systolic regurgitant murmur is appreciated on cardiac auscultation. An echocardiogram shows markedly dilated ventricles with a reduced ejection fraction.
  • Summary
    • Dilated cardiomyopathy (DCM) is the most common type of cardiomyopathy. It is caused by cardiac chamber dilation and systolic dysfunction. The condition typically presents in adults with symptoms of fatigue, dyspnea, edema, and decreased exercise tolerance.
    • Diagnosis is made based on assessment of ventricular dilation and systolic dysfunction while ruling out other causes. Echocardiography can confirm diagnosis by visualizing ventricular changes.
    • Treatment usually consists of addressing the underlying cause of DCM. In severe cases, operative treatment including cardioverter defibrillator, ventricular assist devices, and heart transplantation are considered. 
  • Epidemiology
    • Incidence
      • most common cardiomyopathy
        • 90% of all cardiomyopathies
      • common 6/100,000
    • Demographics
      • 1.5:1 male:female overall DCM
      • 3:1 male:female idiopathic DCM
    • Location
      • cardiac chambers
    • Risk Factors
      • heavy alcohol consumption
      • toxins
        • cocaine
        • amphetamine
        • doxurubicin
      • autoimmune disease
        • systemic lupus erythematosus
        • sarcoidosis
      • stress
        • Takutsubo cardiomyopathy or broken heart syndrome 
      • infectious
        • viral
          • parvovirus B19
          • HSV 6
          • coxsackievirus
          • influenza virus
          • adenovirus
          • echovirus
          • cytomegalovirus
          • HIV
        • Chagas disease
        • Lyme disease
      • inherited syndromes
        • hemochromatosis
      • pregnancy
      • obstructive sleep apnea
  • Etiology
    • Pathogenesis
      • eccentric hypertrophy 
        • underlying causative disease process reduces the contractility of myocardium
          • myocardium stretches and results in left ventricle (LV) dilation
            • ↑ end-diastolic volume (EDV) (preload) results in ↓ EF
              • ↓ ejection fraction (EF)
        • dilation leads to decreased LV contractility
          • leads to left heart failure
            • over time this results in right heart failure
    • Pathoanatomy
      • sarcomeres are added in series
        • results in thinning and stretching of myocardium
          • primarily affects LV and atria
  • Anatomy
    • Cardiac walls are stretched and thinned 
      • results in enlarged ventricles
  • Presentation
    • Symptoms
      • progressive congestive heart failure
        • dyspnea on exertion
        • orthopnea
        • paroxysmal nocturnal dyspnea
        • fatigue
        • pulmonary edema
    • Physical exam
      • inspection
        • jugular venous distention
        • peripheral edema
        • ascites
      • auscultation
        • S3 gallop
        • systolic regurgitant murmur
          • secondary to mitral valve regurgitation or tricuspid valve regurgitation
        • pulmonary rales
  • Imaging
    • Radiography
      • chest x-ray
        • indications
          • for all patients with clinical suspicion
        • findings
          • cardiomegaly
            • heart looks like a balloon
          • pulmonary congestion
    • Echocardiogram
      • indications
        • for all patients
        • best initial test to assess ejection fraction
      • findings
        • ventricular dilation
          • with of without atrial dilation
        • reduced ejection fraction
        • can see wall motion abnormalities in cases with underlying pathologies
    • Cardiac MRI
      • indications
        • suspicion of infiltrative DCM
        • evaluation of cardiac function and morphology
      • findings
        • myocardial edema
        • disease-specific pattern of gadolinium enhancement
  • Studies
    • Electrocardiography (ECG)
      • indications
        • for all patients
        • screen first-degree relatives of patients with familial DCM
      • findings
        • may have bundle branch block
        • may have widened QRS
    • Labs
      • CBC, CRP, ESR
        • indications
          • evaluate for signs of infection or inflammation
        • findings
          • elevated in cases of infection or pericarditis
      • cardiac enzymes
        • indications
          • to assess for myocardial damage
        • findings
          • elevated in cases of myocardial infarction
    • Genetics
      • indications
        • suspected familial or idiopathic DCM
        • family members of DCM patients with an identified genetic etiology
      • findings
        • discovery of underlying disease
  • Diagnosis
    • Based on LV dilation and reduce EF as demonstrated by echocardiography.
  • Differential
    • Restrictive cardiomyopathy
      • distinguishing factors
        • Kussmaul sign on physical exam
        • electrocardiogram with low voltages
    • Hypertrophic cardiomyopathy
      • distinguishing factors
        • echocardiogram with normal ejection fraction
        • S4 gallop on physical exam
  • Treatment
    • Goals
      • treat underlying cause of DCM
      • treat CHF if present
    • Medical
      • drugs that lower mortality
        • angiotensin converting enzyme inhibitors (ACE inhibitors)
          • indication
            • for all patients
        • β-blockers
          • indication
            • for all patients
          • drugs with mortality benefits
            • metoprolol
            • carvedilol
            • bisoprolol
        • spironolactone or eplerenone
          • indications
            • for all patients
            • decreases work of the heart
      • cessation of alcohol
        • indications
          • for all patients
    • Operative
      • automatic implantable cardioverter/defibrillator (AICD)
        • indications
          • ejection fraction < 35%
      • biventricular pacemaker
        • indications
          • QRS > 120 ms
          • with a widened QRS, the 2 ventricles are not in synchrony
          • a biventricular pacemaker will resynchronize the 2 ventricles
      • heart transplant
        • indications
          • the only definitive treatment
  • Techniques
    • automatic implantable cardioverter/defibrillator (AICD)
      • approach
        • evaluate patient suitability, select appropriate candidates, and address underlying heart conditions
      • technique
        • insert leads into the heart chambers via veins
        • position the device in a subcutaneous pocket
        • confirm proper lead placement and device functionality
      • complications
        • infection
        • lead-related issues
        • bleeding
        • pneumothoraxarrhythmias, allergic reactions, device malfunction, and potential migration of the device or leads
        • arrhythmias
        • potential migration of the device or leads
    • biventricular pacemaker
      • approach
        • assess patient suitability, select appropriate candidates, and address the specific heart condition and conduction abnormalities
      • technique
        • position leads in the right atrium, right ventricle, and coronary sinus
        • synchronize to optimize ventricular contraction and improve cardiac output
      • complications
        • infection
        • lead-related issues
        • bleeding
        • pneumothorax
        • arrhythmias
        • potential for migration of leads, as well as the need for ongoing device monitoring and adjustments
    • heart transplant
      • approach
        • meticulous patient evaluation, donor organ matching, and comprehensive preoperative preparation to ensure optimal outcomes
      • technique
        • remove the recipient's diseased heart and implant a healthy donor heart
        • connect major blood vessels and ensuring proper blood flowmeticulous postoperative care
        • meticulous postoperative care
      • complications
        • graft rejection
        • infection
        • organ failure
        • complications related to immunosuppressive medications
        • vascular issues
        • need for ongoing post-transplant monitoring and management
  • Complications
    • Heart failure
    • Thromboembolism
  • Prognosis
    • Around 50% of patients die within 2 years from heart failure or arrhythmias
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