Hypertrophic Cardiomyopathy Mcphs

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Key Features & Essentials of Diagnosis
(Hypertrophic Cardiomyopathy)

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• Dyspnea
• Systolic ejection murmur (w/ characteristic changes during bedside maneuvers)
• Markedly asymmetric LVH on ECHO
• Normal or hyperkinetic LV systolic fxn

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General Considerations: Autosomal Genetic Disorder (Hypertrophic Cardiomyopathy)

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HC is an autosomal genetic disorder. Cuaed by abnormalities in genes that encode myocardial sarcomeric proteins.

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General Considerations: Varying Expression (Hypertrophic Cardiomyopathy)

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Asymptomatic -> disabling

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General Considerations: Asymmetric LVH in 2/3rd (Hypertrophic Cardiomyopathy)

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Septum or, rarely, apex involved; the rest concentric LVH.

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General Considerations: Histology (Hypertrophic Cardiomyopathy)

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Myofiber disarray with undersized intramural arteries for the mass of myocardium.

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General Considerations: Hyperdynamic Function (Hypertrophic Cardiomyopathy)

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The left ventricle cavity is usually small and shows hyperdynamic function.

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General Considerations: Symmetric Hypertrophy (Hypertrophic Cardiomyopathy)

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Intracavitary gradients that worsen with increased contractile stimuli and improve with increased afterload.

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General Considerations: Asymmetric septal hypertrophy (Hypertrophic Cardiomyopathy)

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Characteristic systolic murmur that varies in intensity with loading conditions.

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General Considerations: Mitral Regurgitation (Hypertrophic Cardiomyopathy)

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Systolic anterior movement of the mitral valve often leads to mitral regurgitation.

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Symptoms and Signs (Hypertrophic Cardiomyopathy)

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• Could show no symptoms (asymptomatic)
• Dyspnea, fatigue
• Chest pain
• Syncope
• Palpitation

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Fact: The No. 1 cause for athlete death under 35... (Hypertrophic Cardiomyopathy)

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...is Genetic Autosomal Dominant

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Physical Exam Findings (Hypertrophic Cardiomyopathy)

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• May be unremarkable
• Left ventricle lift
• S4
• Systolic ejections murmur (increases in intensity with maneuvers that decrease left ventricular filling, such as standing; and softens with increased filling, such as squatting.

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Differential Diagnosis (Hypertrophic Cardiomyopathy)

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• Left ventricular hypertrophy secondary to other conditions (e.g. hypertension)
• Aortic stenosis with marked left ventricular hypertrophy
• Restrictive cardiomyopathy with hypertrophy (such as amyloidosis)
• Ischemic heart disease with compensatory septal hypertrophy
• Athlete's heart

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Lab Tests (Hypertrophic Cardiomyopathy)

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• Increased BNP
• Appropriate genetic abnormality

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Electrocardiography (Hypertrophic Cardiomyopathy)

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• Nonspecific abnormalities
• LVH
• Apical variant will have giant negative T waves in precordial leads

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Imaging Studies: ECHO (Hypertrophic Cardiomyopathy)

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ECHO: hypertrophy, MV problems, intracavitary gradients

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Imaging Studies: Nuc (Hypertrophic Cardiomyopathy)

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Nuc: may show infarct and ischemia in the absence of any actual CAD

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Diagnostic Procedures (Hypertrophic Cardiomyopathy)

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Cardiac catheterization: better at measuring intracavitary gradients and can also exclude CAD while you're at it.

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Medications (Hypertrophic Cardiomyopathy)

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Prevent Heart Failure: Beta blockers, calcium channel blockers and antiarrhythmic agents as appropriate

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Therapeutic Procedures (Hypertrophic Cardiomyopathy)

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• Septal involvement: Intracoronary ethanol septal ablation
• Pacemaker to set atrioventricular delay to minimize left ventricular outflow tract gradient
• AICD b/c patients at risk for ventricular arrhythmias

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Surgery (Hypertrophic Cardiomyopathy)

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• Septal myectomy to relieve left ventricular outflow tract obstruction
• Mitral valve replacement in selected cases

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Diet and Activity (Hypertrophic Cardiomyopathy)

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Avoid dehydration

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Complications (Hypertrophic Cardiomyopathy)

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• Sudden cardiac death
• Atrial fibrillation
• Heart failure
• Infective endocarditis

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Prevention (Hypertrophic Cardiomyopathy)

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• Genetic counseling
• Endocarditis prophylaxis

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Practice Guidelines (Hypertrophic Cardiomyopathy)

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• The major indication for treatment is the presence of symptoms
• The only therapy to prolong life is an AICD. Patients with several high-risk factors should be considered for this therapy

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Prognosis of High-Risk Features (Hypertrophic Cardiomyopathy)

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• Risk of sudden death is 1 - 4% annually
• Risk predictors:
- the younger the disease presents
- ventricular arrhythmias or syncope
- family history of sudden death
- marked left ventricular hypertrophy
- hypotension with exercise

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Essentials of Diagnosis (Dilated Cardiomyopathy)

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• Dilation of both ventricles with diffusely reduced contractile functions
• Idiopathic though 25-30% may be genetic

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General Consideration (Dilated Cardiomyopathy)

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• Hallmark: reduced contractile functions & dilations of both ventricles
• Most common type of cardiomyopathy (accounts for 25% of CHF)
• Black or Male most likely (much more than white or female)
• affects any age range
• can also manifest after viral myocarditis

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Symptoms and Signs (Dilated Cardiomyopathy)

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• decreased exercise tolerance
• dyspnea
• orthopnea
• paroxysmal nocturnal dyspnea
• fatigue
• palpitations
• presyncope or syncope

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Physical Exam Findings (Dilated Cardiomyopathy)

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• tachycardia
• tachypnea
• jugular venous distention may be present
• pulmonary rales
• laterally displaced and diffuse PMI; left and right ventricular lifts
Soft S1, paradoxically split S2 with a left bundle branch block
• S3 and S4 gallops
• hepatomegaly due to elevated venous pressures
• ascites, peripheral edema

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Differential Diagnosis (Dilated Cardiomyopathy)

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• Ischemic cardiomyopathy
• valvular heart disease
• hypertensive heart disease
• alcoholic cardiomyopathy
• cardiomyopathy related to thyroid dysfunction
• HIV cardiomyopathy

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Lab Tests (Dilated Cardiomyopathy)

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• electrolytes, BUN and creatinine
• CBC
• liver function tests: elevations may be due to hepatic congestion
• cardiac enzymes: to exclude acute or recent myocardial injury
• thyroid function tests: rule out hyperthyroidism
• urine pregnancy test: in women of childbearing age
• urine toxicology screen

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Electrocardiography (Dilated Cardiomyopathy)

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• PVCs/Ectopy
• nonspecific ST- and T-wave changes
• LVH
• LBBB
• +/- AV blocks

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Imaging Studies: X-ray (Dilated Cardiomyopathy)

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• enlarged cardiac silhouette
• pulmonary vascular congestion
• pleural effusions

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Imaging Studies: Echocardiography (Dilated Cardiomyopathy)

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• all four cardiac chambers are typically enlarged, reduced LV fxn
• MR and TR from annular dilation

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Diagnostic Procedures (Dilated Cardiomyopathy)

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• stress test/Nuc: eliminate coronary artery disease as cause
• endomyocardial bx: only if you suspect cardiac amyloidosis in pre-transplant pt

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Medications (Dilated Cardiomyopathy)

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• Treat for CHF
- ACE-1. beta blockers, spironolactone, diuretics, +/- digoxin
- low sodium diet and fluid restriction
• Treat any A-Fib to prevent RVR (e.g. propranolol)

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Therapeutic Procedures (Dilated Cardiomyopathy)

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• Automatic Internal Cardiac Defibrillator (AICD) in patients with an EF <35%
• Cardiac Resynchronization Therapy (CRT) aka Biventricular Pacing, Bi-V Pacing

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Surgery (Dilated Cardiomyopathy)

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• Cardiac transplantation in atients w/ severe progressive disease despite optimal therapy
• left and right ventricular assist devices (LVAD) may be used as a bridge to cardiac transplantation or as destination therapy in some who are not candidates for heart transplantation

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Diet and Activity (Dilated Cardiomyopathy)

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• low-sodium, fluid-restricted diet
• mild to moderate regular exercise encouraged; cardiac rehabiliation shown to improve patient outcomes

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Complications (Dilated Cardiomyopathy)

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• progressive heart failure
• arrhythmias
• sudden death
• complications of device implantation: perforation with myocardial rupture and cardiac tamponade, infection and bleeding

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Prognosis (Dilated Cardiomyopathy)

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• depends on severity of of symptoms
- generally, approx 50-80% 5-year survival rate
- patients w/ advanced disease less than/equal to 50% 1-year survival rate
• Approx 10% patients have spontaneous improvement, normalization of cardiac function

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Prevention (Dilated Cardiomyopathy)

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Avoidance of all cardiotoxic agents and treatment of any underlying condition that has been associated w/ dilated cardiomyopathy

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Practice Guidelines (Dilated Cardiomyopathy)

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• patients w/ NYHA class III-IV symptoms and intraventricular conduction delay should be considered for CRT (and refer for transplant)
• patients w/ left ventricular EF less than/equal to 35% should be considered for an AICD

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Key Features & Essentials of Diagnosis (Restrictive Cardiomyopathy)

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• symptoms & signs of heart failure w/ mainly right-sided findings
• normal left & right ventricular size and systolic function w/ dilated atria
• abnormalities of diastolic ventricular function suggestive of reduced ventricular compliance
* Increased ventricular filling pressure (left > right) and reduced cardiac output

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General Considerations: Rarity (Restrictive Cardiomyopathy)

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only 5% of all cases of primary cardiomyopathies

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General Considerations: Causes (Restrictive Cardiomyopathy)

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• linfiltrative processes (e.g. amyloidosis (MOST COMMON CAUSE!!!), hemochromatosis, sarcoidosis, glycogen storage disease)
• Eosinophilic endomyocarditis
• endomyocardial fibrosis (in Africa, called Loffler Syndrome)
• Other: metastatic malignancy, carcinoid heart disease, mediastinal radiation

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General Considerations: Disease Course (Restrictive Cardiomyopathy)

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the course varies depending on the pathology

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General Considerations: Pathophysiology (Restrictive Cardiomyopathy)

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Abnormal myocardial stiffness -> increased diastolic filling pressure -> decreased cardiac output -> heart failure!!

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Symptoms & Signs (Restrictive Cardiomyopathy)

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• chest pain
• palpitations
• one third of patients may present with thromboembolic events

• dyspnea and exercise intolerance
• fatigue and weakness
• increasing abdominal girth (ascites)

**THE LAST THREE SYMPTOMS LOOK LIKE HEART DISEASE***

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Physical Exam Findings (Restrictive Cardiomyopathy)

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• reduced pulse pressure
• tachycardia
• JVD
• loud S3
• murmurs of mitral and tricuspid
• ascites
• palpable liver
• peripheral edema
• decreased breath sounds
• pulmonary rales may be heard

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Physical Exam Findings: Which three findings look more "congestive" than other cardiomyopathies? (Restrictive Cardiomyopathy)

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Loud S3, peripheral edema, pulmonary rales may be heard.

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Differential Diagnosis (Restrictive Cardiomyopathy)

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• Dilated cardiomyopathy w/ restrictive physiology
• hypertrophic cardiomyopathy
• constrictive

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Lab tests (Restrictive Cardiomyopathy)

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• rule out eosinophilia
• iron studies to rule out hemochromatosis
• serum protein electrophoresis & serum and urine immunofixation to rule in/out amyloid

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Electrocardiography (Restrictive Cardiomyopathy)

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• sinus sythmn, sinus tachycardia or atrial arrhythmias may be present
• varying degrees of atrioventricular block may be present
• Low QRS voltage *
• Nonspecific ST & T-wave abnormalities *

* = bolded in notes, therefore important!

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Imaging Studies: Chest X-ray (Restrictive Cardiomyopathy)

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• dilated atria with normal ventricular size
• signs of CHF

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Imaging Studies: ECHO (Restrictive Cardiomyopathy)

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• restrictive diastolic
• normally contracting right and left ventricles w/ normal or increased (infiltrative diseases) wall thickness w/ normal or reduced ventricular cavity size
• markedly dilated atria
• maybe some mural thrombi; maybe some valve insufficiency
• Doppler evidence of restrictive diastolic filling pattern *

* bolded = important!!

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Medications (Restrictive Cardiomyopathy)

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• decrease ventricular preload w/ loop diuretics
• get rid of a-fib or at least maintain rate control of a-fib
• anticoagulation for those w/ atrial fibrillation & endocardial fibrosis
• treatment of the underlying systemic disease

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Therapeutic Procedures (Restrictive Cardiomyopathy)

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pacing for extreme bradycardia or conduction block

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Surgery (Restrictive Cardiomyopathy)

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• transplant in severe, refractory heart failure
• surgical resection of endocardial fibrosis in selected cases

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Diet & Activity (Restrictive Cardiomyopathy)

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• two-gram dietary sodium restriction
• activity as tolerated

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Complications (Restrictive Cardiomyopathy)

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• Progressive heart failure *
• atrial and ventricular tachyarrhythmias
• heart block
• sudden death
• thromboembolic events
• syncope

* = important

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Prognosis (Restrictive Cardiomyopathy)

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• largely depends on underlying pathology
• survival limited for most patients - 70% die within 5 years of sx onset!!!

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Practice Guidelines (Restrictive Cardiomyopathy)

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• Restrictive cardiomyopathy should be considered in patients w/ signs & symptoms of progressive congestive heart failure and preserved ventricular systolic function
• hemodynamic findings - useful to distinguish restriction from constriction include:
- right ventricular systolic pressure greater than 50 mm Hg
- greater than/equal to 5 mm Hg difference between right and left ventricular end-diastolic pressures
- absence of respirophasic variation in left-sided filling pressures
- marked atrial dilatation
- absence of pericardial thickening and calcification

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Key Features & Essentials of Diagnosis (Myocarditis)

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• Presentation: new onset CHF with preceding viral syndrome (looks like an MI but it isn't!)
• Labs: increases ESR +/- cardiac biomarkers w/ acute myocarditis
• ECG: sinus tachycardia, nonspecific ST-T changes*, atrial or ventricular arrhythmias, or conduction abnormalities
• Echo: chambe enlargement, globally reduced left ventricular (LV) contractility
• Warning: mural thrombi may be present!!

* = important!!

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General Considerations (Myocarditis)

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• Inflammatory disease of cardiac muscle
• can be acute, subacute, chronic
• most commonly initiated by viral infection* - tho may occur as a result of other infectious organisms, drugs, toxins, collagen vascular diseases, or autoimmune or hypersensitivy reactions
• Coxsackie B* = most common virus associated w/ myocarditis (others include adenovirus, echovirus, influenza virus, Hep C, cytomegalovirus, and parvovirus B19
• endomyocardial Bx (not recommended): inflammatory infiltrate w/ adjacent myocyte injury

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Symptoms & Signs (Myocarditis)

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• usually asymptomatic
• preceding viral syndrome
• chest pain **
• dyspnea, fatigue, decreased exercise tolerance **
• palpitations, dizziness or syncope **
• cardiogenic shock **

** = think new onset CHF!!!

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Physical Exam Findings (Myocarditis)

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• fever
• tachycardia
• hypotension **
• signs of fluid overload **
• murmurs of mitral or tricuspid regurgitation **
• S3 and occasionally S4 gallops **
• pleural rubs and pericardial friction **
• Circulatory collapse and shock (rare) **

**= think new onset CHF!!!

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Differential Diagnosis (Myocarditis)

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• Acute myocarcial ischemia or infarction due to coronary artery disease
• pneumonia
• congestive heart failure due to other causes

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Lab Tests (Myocarditis)

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Increased ESR and increased Troponin T or I and CK-MB seen in some patients

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Electrocardiography (Myocarditis)

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• nonspecific ST-T wave changes, ectopy
• atrial tachycardia or fibrillation and AV blocks or bundle brance blocks

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Imaging Studies (Myocarditis)

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• Chest x-ray: normal or cardiomegaly, may have pulmonary edema
• Echocardiography: LV enlargement; globally reduced LV contractility, mural thrombi may be present
• Gallium-67 imaging: highly sensitive for identifying active inflammation (but rarely ordered!!!!!)

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Diagnostic Procedures (Myocarditis)

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• Cardiac catheterization: only if the presentation really looks like acute myocardial infarction
• Endomyocardial biopsy: considered the gold stadard for establishing the diagnosis; however rarely used due to poor sensitivity

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Medications (Myocarditis)

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• narcotic analgesics for pain relief
• specific antimicrobial treatment if an infections agent is indentified
• Treat CHF
- ACE-I
- beta blockers
- Ddigoxin
- spironolactone
- diuretics (lasix)
• Avoid NSAIDS
• steroids are NOT helpful except in giant cell myocarditis and autoimmune diseases (e.g. SLE)

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Therapeutic Procedures (Myocarditis)

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• pericatdiocentisis for pericardial effusion and signs of cardiac tamponade
• pacemaker insertion for heart block

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Diet and Activity (Myocarditis)

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• low-sodium diet and fluid restriction in patients with CHF
• restrict activity until ECG returns to normal

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Prognosis (Myocarditis)

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• transplantation: patients have increased risk of recurrent myocarditis and transplant rejection
• Of those who develop CHF: 50% get better; 25% same; 25% worse

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Practice Guidelines (Myocarditis)

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• myocarditis may present subtly and should be considered in patients with chest pain and signs of heart failure
• most patients respond to medical therapy alone
• endomyocardial biopsy should be considered in patients with heart failure refractory to medical therapy*, a rapidly deteriorating course* or suspected secondary causes of dilated cardiomyopathy*

* = IMPORTANT!!