Recipient(s) will receive an email with a link to 'Cardiomyopathy, End Stage' and will have access to the topic for 7 days.
Subject: Cardiomyopathy, End Stage
(Optional message may have a maximum of 1000 characters.)
Cardiomyopathy encompasses a large group of diseases of the myocardium that commonly result in mechanical pump dysfunction. The current classification scheme attempts to differentiate between myocardial diseases confined to the myocardium (primary) and those due to systemic disorders (secondary). Specific causes of myocardial dysfunction due to other cardiovascular disorders are considered a third, separate category (1).
Classification of cardiomyopathy
Hypertrophic cardiomyopathy (HCM)
Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D)
Left ventricular (LV) noncompaction (LVNC)
Glycogen storage (Danon type, PRKAG2)
Ion channel disorders: long QT syndrome, Brugada, short QT syndrome, catecholaminergic ventricular tachycardia (CVPT), Asian SUNDS
Dilated cardiomyopathy (DCM) (genetic or nongenetic)
Myocarditis, stress cardiomyopathy, peripartum, tachycardia-induced, infants of type 1 diabetic mothers
Secondary (see list below)
Congenital heart disease
Patients with end-stage cardiomyopathy have stage D heart failure or severe symptoms at rest refractory to standard medical therapy.
System(s) affected: cardiovascular; renal
60,000 patients <65 years die each year from end-stage heart disease.
35,000–70,000 people might benefit from cardiac transplant or chronic support.
Ischemic heart disease: most common etiology; up to 66% of patients
Valvular heart disease
Primary genetic causes
Beriberi, pellagra, scurvy, selenium, carnitine, kwashiorkor
Systemic lupus erythematosus
Viral (e.g., HIV, coxsackievirus, adenovirus)
Bacterial and mycobacterial (e.g., diphtheria, rheumatic fever)
Parasitic (e.g., toxoplasmosis, Trypanosoma cruzi)
Glycogen storage disease (type II, Pompe)
Duchenne and Emery-Dreifuss muscular dystrophies
Drugs and chemotherapy: anthracyclines, cyclophosphamide, Herceptin
Heavy metal, chemical agents
Hypereosinophilic syndrome (Loeffler endocarditis)
Coronary artery diease
Excessive alcohol intake
Obstructive sleep apnea
Dyspnea at rest or with exertion
Paroxysmal nocturnal dyspnea
Right upper quadrant pain or bloating
Low pulse pressure
Jugular venous distention
Displaced point of maximal impulse (PMI)
Blowing systolic murmur
Severe pulmonary disease
Primary pulmonary hypertension
Recurrent pulmonary embolism
Some advanced forms of malignancy
ECG: LV hypertrophy, interventricular conduction delay, atrial fibrillation, evidence of prior Q-wave infarction
Mild elevation in troponin
Elevated B-type natriuretic peptide (BNP) or pro-BNP
Elevated liver function tests
Elevated uric acid
Increased vascular markings to the upper lobes
Pleural effusions may or may not be present.
In dilated cardiomyopathy, 4-chamber enlargement and global hypokinesis are present.
In hypertrophic cardiomyopathy, severe LV hypertrophy is present.
Segmental contraction abnormalities of the LV are indicative of previous localized myocardial infarction.
May be useful to characterize certain nonischemic cardiomyopathies
Myocardial stress perfusion imaging (MPI)
Recommended in those with new-onset LV dysfunction or when ischemia is suspected
Helpful to rule out ischemic heart disease
Characterize hemodynamic severity
Pulmonary artery catheters may be reasonable in patients with refractory heart failure to help guide management.
Reduction of filling pressures
Treatment of electrolyte disturbances
Systolic failure syndromes
ACE inhibitors: All considered equally effective; initiate at low doses and titrate as tolerated to target doses (3)[A].
May need to be given IV initially and then orally as patient stabilizes
Furosemide, 40–120 mg/day or TID (3)[A]
Use with caution in acutely decompensated or low–cardiac output states.
Initiate with low doses and titrate as tolerated.
Metoprolol succinate, 12.5–200 mg/day; carvedilol, 3.125–25 mg BID; or bisoprolol, 1.25–10 mg/day (3)[A]
Patients with New York Heart Association (NYHA) II–IV heart failure, ejection fraction (EF) <35%, on standard therapy: aldosterone antagonists: spironolactone or eplerenone (3)[A]
Digoxin, 0.125–0.25 mg/day for symptomatic patients on standard therapy (3)[B]
Combination hydralazine/isosorbide dinitrate is 1st-line treatment in African American patients with class III–IV symptoms already on standard therapy and for all patients with reduced EF and symptoms incompletely responsive to ACE inhibitor and β-blocker (3)[A].
β-Blockers: low cardiac output, 2nd- or 3rd-degree heart block
Avoid use of diltiazem and verapamil in patients with systolic dysfunction.
Aldosterone antagonists: oliguria, anuria, renal dysfunction
Loop diuretics: hypokalemia, hypomagnesemia
ACE inhibitors: pregnancy, angioedema
In patients with chronic kidney disease, digoxin dosage should be ≤0.125 mg/day and drug levels followed carefully to avoid toxicity.
Closely monitor electrolytes.
ACE inhibitors: Initiate with care if BP is low. Begin with low-dose captopril, such as 6.25 mg TID.
β-Blockers: Avoid in patients with evidence of poor tissue perfusion; they may further depress systolic function.
Milrinone, dobutamine: long-term use associated with increased mortality
Medications TO AVOID
Angiotensin receptor blockers as an alternative to ACE inhibitors
Inotropic therapy (e.g., dobutamine or milrinone) for cardiogenic shock and support prior to surgery or cardiac transplantation (3)[B]
Continuous inotrope infusion may be considered in stage D outpatients for symptom control in those who are not eligible for transplantation or mechanical circulatory support (3)[B].
Prophylactic implantable cardioverter defibrillator (ICD) should be considered for patients with a left ventricular ejection fraction (LVEF) <35% and mild to moderate symptoms (3)[A].
Cardiac resynchronization therapy (CRT) is recommended and should be considered for patients in sinus rhythm with a QRS >150 msec, LVEF <35%, in FC I–III and ambulatory FC IV patients (3)[A].
Patients with severe, refractory heart failure with no reasonable expectation of improvement should not be considered for an ICD.
Consideration of an LV assist device as “permanent” or destination therapy or cardiac transplantation is reasonable in selected stage D patients.
I42.9 Cardiomyopathy, unspecified
I42.2 Other hypertrophic cardiomyopathy
I42.1 Obstructive hypertrophic cardiomyopathy
I43 Cardiomyopathy in diseases classified elsewhere
I42.4 Endocardial fibroelastosis
I42.5 Other restrictive cardiomyopathy
I42.8 Other cardiomyopathies
I42.6 Alcoholic cardiomyopathy
I42.3 Endomyocardial (eosinophilic) disease
I42.7 Cardiomyopathy due to drug and external agent
I42.0 Dilated cardiomyopathy
425.4 Other primary cardiomyopathies
425.18 Other hypertrophic cardiomyopathy
425.11 Hypertrophic obstructive cardiomyopathy
425.8 Cardiomyopathy in other diseases classified elsewhere
425.5 Alcoholic cardiomyopathy
425.7 Nutritional and metabolic cardiomyopathy
425.9 Secondary cardiomyopathy, unspecified
85898001 Cardiomyopathy (disorder)
233873004 Hypertrophic cardiomyopathy (disorder)
45227007 Hypertrophic obstructive cardiomyopathy (disorder)
195029002 Cardiomyopathy associated with another disorder (disorder)
83521008 Dilated cardiomyopathy secondary to alcohol (disorder)
399020009 Congestive cardiomyopathy (disorder)
415295002 Restrictive cardiomyopathy (disorder)
195023001 Nutritional and metabolic cardiomyopathies (disorder)
89461002 Primary cardiomyopathy
Cardiomyopathy represents the end-stage of a large number of disease processes involving the heart muscle.
Ischemic, hypertensive, postviral, familial, alcoholic, and incessant tachycardia-induced are the most common cardiomyopathy varieties seen in the United States.
Core therapy for heart failure applies: salt restriction, diuretics, ACE inhibitors, β-blockers, digoxin, and electrical treatments, such as cardiac resynchronization and implantable defibrillators, as appropriate.
Septal amyloidosis shows characteristic apple green birefringence of amyloid on Congo red stain.
FIGURE 28.2. Radiographs illustrating the different stages of sarcoidosis. A: Stage I. Bilateral hilar adenopathy and paratracheal adenopathy with normal lung fields. B: Stage II. Bilateral hilar adenopathy with interstitial lung field involvement. C: Stage III. Lung field involvement only. D: Stage IV. Severely fibrotic lungs with volume loss and cyst formation.
FIGURE 28.2. Radiographs illustrating the different stages of sarcoidosis. A: Stage I. Bilateral hilar adenopathy and paratracheal adenopa...
<bold>FIG. 79.1.</bold> A: Mitral valve from a patient with mitral stenosis from rheumatic fever (viewed from atrium). Notice the thickened calcific leaflets and thickened chordae tendineae compared with a normal mitral valve (B). (Courtesy of W.D. Edwards, M.D., Mayo Clinic.)
<bold>FIG. 79.1.</bold> A: Mitral valve from a patient with mitral stenosis from rheumatic fever (viewed from atrium). Notice ...
<bold>FIG. 79.2.</bold> A: Erythema marginatum in an adult with rheumatic fever. B: Closer view of rash. (Courtesy of P. Witman, M.D., Mayo Clinic.)
<bold>FIG. 79.2.</bold> A: Erythema marginatum in an adult with rheumatic fever. B: Closer view of rash. (Courtesy of P. Witma...
<bold>FIG. 93.1.</bold> A: Electron micrograph of amyloid surrounding a cardiac myocyte (×20, 125). B: High-magnification image showing typical fi illar nature of amyloid deposit (×68,125).
<bold>FIG. 93.1.</bold> A: Electron micrograph of amyloid surrounding a cardiac myocyte (×20, 125). B: High-magnification imag...
<bold>FIG. 93.16.</bold> Two-dimensional echocardiogram for the same subject as depicted in Fig. 93.6. Classic features of amyloid cardiomyopathy are thickened intraventricular septum <italic>(IVS)</bold>, left ventricular posterior wall <italic>(PW)</bold>, and enlarged left atrium <italic>(LA)</bold>.
<bold>FIG. 93.16.</bold> Two-dimensional echocardiogram for the same subject as depicted in Fig. 93.6. Classic features of amy...
<bold>FIG. 93.17.</bold> Electrocardiogram of a patient with amyloid cardiomyopathy. Low voltage is a prominent feature, and the standard, but erroneous, interpretation as "septal infarct, age indeterminate" is common.
<bold>FIG. 93.17.</bold> Electrocardiogram of a patient with amyloid cardiomyopathy. Low voltage is a prominent feature, and t...
Figure 5.62. Sarcoidosis-related retinal phlebitis with perivascular exudates known as "candle-wax drippings."
FIGURE 82.1. Chest radiograph of older child with congestive heart failure. Note cardiac enlargement and evidence of pulmonary venous congestion.
FIGURE 82.1. Chest radiograph of older child with congestive heart failure. Note cardiac enlargement and evidence of pulmonary venous cong...
<bold>Figure 31.19</bold> Hemodynamics of amyloidosis, a restrictive cardiomyopathy. The elevated right atrial pressure, Kussmaul sign, and prominent Y descents <bold>(A)</bold> as well as the dip and plateau in the right ventricular tracing <bold>(B)</bold> are indicative of severe right ventricular dysfunction and not constriction. Prominent V waves <bold>(C)</bold> are present in the wedge tracing despite the lack of mitral regurgitation and reflect the volume overload in the stiff atrium, characteristic of amy...
<bold>Figure 31.19</bold> Hemodynamics of amyloidosis, a restrictive cardiomyopathy. The elevated right atrial pressure, Kussm...
<bold>Figure 31.20</bold> Electrocardiogram of a patient with amyloid. Classic findings include low limb lead voltage but preserved precordial voltage, nonspecific ST changes, P wave prominence, and pseudoinfarct pattern with precordial Q waves.
<bold>Figure 31.20</bold> Electrocardiogram of a patient with amyloid. Classic findings include low limb lead voltage but pres...
<bold>Figure 31.22</bold> Endomyocardial biopsy of patient with amyloid. A. Even without congo red staining, amyloid is suggested by separation of myofibrils and infiltration of blood vessels by pink amorphous proteinaceous material. Hypertrophy notably absent as well. <bold>B.</bold> At autopsy, left atrial walls appear leathery and waxy, consistent with endocardial deposition of amyloid protein.
<bold>Figure 31.22</bold> Endomyocardial biopsy of patient with amyloid. A. Even without congo red staining, amyloid is sugges...
Homeless diabetic man with painful foot showing gangrene and dystrophic nails
<bold>Fig C 2-1 Congestive heart failure.</bold> Diffuse bilateral symmetric infiltration of the central portion of the lungs along with relative sparing of the periphery produces the butterfly, or bat's wing, pattern. The margins of the edematous lung are sharply defined. The consolidation is fairly homogeneous and is associated with a well-defined air bronchogram on both sides.<sup>6</sup>
<bold>Fig C 2-1 Congestive heart failure.</bold> Diffuse bilateral symmetric infiltration of the central portion of the lungs ...
<bold>Fig CA 18-1 Essential (idiopathic) hypertension.</bold> (A) Frontal and (B) lateral views of the chest demonstrate characteristic tortuosity of the aorta (arrows), especially the ascending portion. Because the elevated blood pressure has caused left ventricular hypertrophy without dilatation, the radiographic appearance of the cardiac silhouette remains normal.
<bold>Fig CA 18-1 Essential (idiopathic) hypertension.</bold> (A) Frontal and (B) lateral views of the chest demonstrate chara...
<bold>Fig CA 4-7 Alcoholic cardiomyopathy</bold>. Generalized cardiac enlargement that involves all chambers but has a left ventricular predominance. There is pulmonary vascular congestion and a right pleural effusion.
<bold>Fig CA 4-7 Alcoholic cardiomyopathy</bold>. Generalized cardiac enlargement that involves all chambers but has a left ve...
<bold><italic>FIGURE 6.64.</bold></bold> Apical four-chamber view recorded in an elderly patient with long-standing mild systemic hypertension and congestive heart failure due to predominantly diastolic dysfunction. Note the evidence of significant atrial enlargement, which is a marker of long-standing diastolic dysfunction. LA, Left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle
<bold><italic>FIGURE 6.64.</bold></bold> Apical four-chamber view recorded in an elderly patient with long-standin...
Parasternal long (A) and apical four-chamber (B) and mitral inflow (C) in advanced cardiac amyloidosis. Note that the left ventricular size is normal with markedly thickened ventricular walls and has its characteristic granular sparkling appearance. DT, deceleration time; LA, left atrium; LV, left ventricle; PE, pericardial effusion; PL EFF, pleural effusion; pm, papillary muscle; RA, right atrium; RV, right ventricle.
Parasternal long (A) and apical four-chamber (B) and mitral inflow (C) in advanced cardiac amyloidosis. Note that the left ventricular siz...
Schaumann body consists of fragmented irregular calcified basophilic refractile structure in the cytoplasm of a multinucleated giant cell in a sarcoidosis granuloma.
Schaumann body consists of fragmented irregular calcified basophilic refractile structure in the cytoplasm of a multinucleated giant cell ...
Morphologic features of the main types of cardiomyopathies. The normal geometry of the LV is shown. In comparison, there is enlargement and dilatation of the LV in DCM. In HCM, there is marked thickening of the LV wall, often asymmetric, with the septum being even thicker than the free wall of the LV. In restrictive cardiomyopathy (RCM), the ventricular wall may be normal, hypertrophic, or slightly dilated, but the main feature is that the restriction to diastolic compliance of the ventricl...
Morphologic features of the main types of cardiomyopathies. The normal geometry of the LV is shown. In comparison, there is enlargement an...
<bold><italic>Figure 14.60.</bold></bold> Pretibial diabetic skin changes: necrobiosis lipoidica diabeticorum.
FIG. 11 Diabetic patient with acute spontaneous onset of low back pain and fever. <bold>A,B:</bold> Dramatic elements of two-level spondylodiscitis, L3-5, with secondary communicating psoas abscess component.
FIG. 11 Diabetic patient with acute spontaneous onset of low back pain and fever. <bold>A,B:</bold> Dramatic elements of two-l...
<bold>Figure 4.27. Kerley lines in patients with congestive heart failure. A,</bold> AP radiograph shows prominent interstitial markings in both bases with a fine interlacing pattern. <bold>B,</bold> detail view shows the linear horizontal Kerley B-lines in the periphery. <bold>C</bold> and <bold>D,</bold> detail views of two other patients show similar findings.
<bold>Figure 4.27. Kerley lines in patients with congestive heart failure. A,</bold> AP radiograph shows prominent interstitia...
<bold>Figure 5.33. Congestive heart failure and pulmonary edema. A.</bold> Frontal view shows mild pulmonary edema and pulmonary venous engorgement. <bold>B.</bold> Detail view shows the edema and prominent septal (Kerley) lines. <bold>C.</bold> Detail view in another patient shows the prominent horizontal Kerley lines.
<bold>Figure 5.33. Congestive heart failure and pulmonary edema. A.</bold> Frontal view shows mild pulmonary edema and pulmona...
<bold>Figure 11.93. Neuropathic (Charcot) joints in two diabetic patients. A.</bold> Radiographs of both feet show bilateral neuropathic joints at the first metatarsophalangeal joints (<italic>arrows</bold>). On the left there is periarticular osteopenia, indicating superimposed infection. Frontal <bold>(B)</bold> and lateral <bold>(C)</bold> knee radiographs of another patient show disintegration of the knee joint with considerable bony debris (" bag of bones") and a large joint effusion. In the past, involvement o...
<bold>Figure 11.93. Neuropathic (Charcot) joints in two diabetic patients. A.</bold> Radiographs of both feet show bilateral n...
Necrobiosis lipoidica diabeticorum. Atrophic patch present x 6 mo.
<bold>FIGURE 11-44. Cardiac amyloidosis.</bold><bold>A.</bold> A section of myocardium stained with Congo red shows interstitial, pink-staining deposits of amyloid. <bold>B.</bold> Under polarized light, the same section displays the characteristic green birefringence of amyloid fibrils.
<bold>FIGURE 11-44. Cardiac amyloidosis.</bold><bold>A.</bold> A section of myocardium stained with Congo red show...