Infiltrate Cardiomyopathy

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Restrictive

Arrhythmogenic Right Ventricular Cardiomyopathy

Idiopathic Genetic

Autosomal dominant Autosomal recessive Naxos disease (mal de Meleda)

Unclassified

Primary idiopathic Loeffler's Endomyocardial fibrosis Secondary Infiltrative Amyloidosis Sarcoidosis Storage Diseases Hemochromatosis Glycogen storage Lysosomal storage

Fig. 1. Cardiomyopathies: classification.

Left ventricular noncompaction Endocardial fibroelastosis Mitochondrial diseases

Specific

Ischemic Hypertensive Valvular Metaboliic Connective tissue disease General system disease Muscular/

neuromuscular Toxic

Stress-related Takotsubo Myocardial Stunning

Infiltrative Cardiomyopathy

Fig. 2. Cardiomyopathies: three major functional types. Two-dimensional and Doppler echocardiography play central roles in the identification of the three major functional types of the cardiomyopathies. Major distinguishing features of these classes are listed in Table 1. Ao, aorta; LA, left atrium; LV, left ventricle.

Fig. 2. Cardiomyopathies: three major functional types. Two-dimensional and Doppler echocardiography play central roles in the identification of the three major functional types of the cardiomyopathies. Major distinguishing features of these classes are listed in Table 1. Ao, aorta; LA, left atrium; LV, left ventricle.

was present and all peripheral pulses were palpable, although of reduced volume. Sinus tachycardia at 112 bpm, frequent premature ventricular contractions and 1.0 mm ST depression in V6, left axis deviation, left atrial enlargement, poor R-wave progression and lateral ST/T-wave abnormalities were all noted on his electrocardiogram (ECG).

Echocardiography revealed a dilated cardiomyopathy. Some of his images are shown in Figs. 3 and 4 (please see companion DVD for corresponding video).

Table 1

Cardiomyopathies: Three Major Functional Types

Dilated cardiomyopathy Hypertrophic cardiomyopathy Restrictive cardiomyopathy

Clinical presentation and frequency

Cardiac chamber dimensions

Wall dimensions

Systolic indices and ejection fraction (normal >55%)

Diastolic function

Valvular function: MR; TR

Dyspnea on exertion; congestive heart failure

Dilated cardiac chambers, esp. the left ventricle; dilated atria; right sided chambers can be dilated as well Normal, mildly increased, or decreased

Reduced systolic function; decreased EF, <30%

Impaired; may reflect volume overload MR secondary to annular dilation and apical tethering; TR frequent

Often asymptomatic; syncope; sudden death; genetic mutation (~1 in 500) Reduced left ventricular cavity size; dilated atria

Asymmetric septal hypertrophy most common; other variants seen (Fig. 8) Normal or increased systolic function; dynamic LVOT obstruction and intracavitary gradients Impaired; asynchronous relaxation MR with SAM of mitral leaflets

Progressive dyspnea, right-sided heart failure; features of underlying disorder

Reduced ventricular cavity size; dilated atria—marked; dilated right sided chambers

Usually increased, and may involve all cardiac chambers

Normal systolic function in early phases; reduced with advanced disease

Impaired with progressive restrictive physiology

MR and TR frequent

Table modified from Warner Stevenson L. Diseases of the myocardium. In: Cecil Textbook of Medicine, 22nd ed. Goldman L, Ausiello D., eds., Saunders, Philadelphia: 2004:441-454.

EF, ejection fraction; LVOT, left ventricular outflow tract; MR, mitral regurgitation; SAM, systolic anterior motion; TR, tricuspid regurgitation.

Dilated cardiomyopathy is characterized by a dilated poorly functional left ventricle. The echocardiography appearance of dilated cardiomyopathy can be remarkably similar despite the multiple etiologies (Table 2). Although regional wall motion abnormalities can be extremely suggestive of an ischemic etiology, some patients with nonischemic cardiomyopathy have regional dysfunction.

Dilated cardiac chambers characterize advanced stages of a dilated cardiomyopathy, predominantly the left ventricle, associated with accompanying systolic dysfunction. All indices of ventricular systolic function—left ventricular volumes, ejection fraction, stroke volume, cardiac output, and others—are generally reduced (Table 3; see also Chapter 4, Tables 4-7). Nevertheless, some patients may demonstrate minimal dilatation with significant ventricular dysfunction.

Wall thickness in dilated cardiomyopathy is usually within normal limits, but may be increased or decreased. Overall cardiac mass is invariably increased (Fig. 5). Dilated cardiomyopathy often leads to dilated mitral annulus, papillary muscle displacement resulting in poor mitral leaflet coaptation, both of which contribute to functional mitral regurgitation. These are best visualized using parasternal and apical windows. (Fig. 6; please see companion DVD for corresponding video; see also Chapter 14, Figs. 5 and 6). Dilated cardiomyopathy may present with relative sparing of regional wall function, especially of the basal and inferior walls (Fig. 3; please see companion DVD for corresponding video), or with multiple regional wall motion abnormalities, although, if nonischemic in etiology, not usually in a coronary distribution. Distinguishing between ischemic and nonischemic etiology by echocardiography can be challenging.

A dilated akinetic-hypokinetic left ventricular chamber increases the risk of intracardiac thrombus, which is more prone to form in areas of relative stasis. The majority of thrombi that form are mural; therefore, careful examination of the ventricular walls, using techniques to improve visualization of spontaneous echocontrast or thrombus (such as using a high-frequency transducer or myocardial contrast agents) should be employed. Intracavitary thrombi are more common in the left ventricular apex (see Chapter 7, Figs. 11 and 12).

Mitral Valve Intra Cardiac Echo

Fig. 3. Dilated cardiomyopathy. Left ventricular, left atrial, and right ventricular dilatation are seen in the parasternal long-axis view (A). Wall thicknesses were within normal limits. Severe hypokinesis with akinetic segments, poor wall thickening are seen. Reduced systolic function leads to poor aortic valve opening, premature closure secondary to reduced stroke volume, and reduced anterior motion of aortic root during systole (B). M-mode at the mitral valve level shows increased E-point septal separation. Note poor mitral valve closure, the akinetic septum, and the relatively preserved postero-basal segment (C). M-mode more just distal to the mitral leaflets (D) shows dilated ventricular chambers with minimal excursion of the ventricular walls, little difference between systole and diastole, and calculated ejection fraction of 15% (Teichholz method, see Chapter 4). (Please see companion DVD for corresponding video.)

Fig. 3. Dilated cardiomyopathy. Left ventricular, left atrial, and right ventricular dilatation are seen in the parasternal long-axis view (A). Wall thicknesses were within normal limits. Severe hypokinesis with akinetic segments, poor wall thickening are seen. Reduced systolic function leads to poor aortic valve opening, premature closure secondary to reduced stroke volume, and reduced anterior motion of aortic root during systole (B). M-mode at the mitral valve level shows increased E-point septal separation. Note poor mitral valve closure, the akinetic septum, and the relatively preserved postero-basal segment (C). M-mode more just distal to the mitral leaflets (D) shows dilated ventricular chambers with minimal excursion of the ventricular walls, little difference between systole and diastole, and calculated ejection fraction of 15% (Teichholz method, see Chapter 4). (Please see companion DVD for corresponding video.)

Dilated Cardiomyopathy Hiv
Fig. 4.

Table 2

Causes of a Dilated Cardiomyopathy on Echocardiography

Idiopathic (most common) Ischemic

Valvular heart disease Chronic hypertension Tachyarrythmias (including SVTs, VTs) Toxins (alcohol, anthracycline, e.g., daunorubicin)

Infections (HIV and viral, bacterial, parasitic, including Chagas) Metabolic

Systemic and connective tissue disease Other

SVT, supraventricular tachycardia; VT, ventricular tachycardia.

Fig. 5. Dilated cardiomyopathy: explanted heart. Gross explanted heart specimen (minus atria) from 56-yr-old male with end-stage dilated cardiomyopathy who received a heart transplant. Note the marked increase in total heart mass (heart weighed 780 g; normal < 360 g). Bilateral atrial enlargement was present, but portions of both atrium remained within transplant recipient. No intracavitary thrombus was seen.

Table 3

Dilated Cardiomyopathy: Echocardiographic Findings

Modality

Description

Two-dimensional Dilated left ventricle3 (sine qua non) findings Dilated right ventricle3 (frequent)

Dilated atria (frequent) Low left ventricle ejection fraction3 Low right ventricle ejection fraction3 reduced indices of systolic function (see Chapter 4) Normal wall thickness, sometimes decreased or increased

Sometimes spontaneous echo contrast ± intracavitary thrombus

Dilated cardiac chambers

M-Mode

Doppler findings increased E-point septal separation

Diastolic dysfunction3—initial impaired relaxation ± later restrictive pattern (see Chapter 5);

mitral regurgitation (functional): secondary to apical tethering ("tenting") leading to restricted leaflet closure, annular dilatation, and papillary muscle displacement

"Association with adverse outcomes.

Fig. 5. Dilated cardiomyopathy: explanted heart. Gross explanted heart specimen (minus atria) from 56-yr-old male with end-stage dilated cardiomyopathy who received a heart transplant. Note the marked increase in total heart mass (heart weighed 780 g; normal < 360 g). Bilateral atrial enlargement was present, but portions of both atrium remained within transplant recipient. No intracavitary thrombus was seen.

Additional Parameters

Evaluation of right heart function, including the presence of right-sided chamber dilatation, tricuspid regurgitation, and pulmonary artery pressures, should be assessed in patients with cardiomyopathy, and can have prognostic importance (see Chapter 4). High tri-cuspid regurgitation velocities have been associated with worse outcomes.

Patients with dilated cardiomyopathy frequently exhibit some degree of diastolic dysfunction. Diastolic dysfunction almost always accompanies systolic dysfunction. A very rapid mitral deceleration time (DT) is indicative of so-called restrictive physiology, which can be seen on pulse-wave Doppler evaluation of mitral inflow. A "restrictive" Doppler pattern has been associated with worse prognosis in patients with heart failure (Fig. 7). Mitral DT less than approx 140 ms is associated with poor prognosis.

Fig. 4. (Opposite p3ge) Dilated cardiomyopathy. Parasternal short-axis views at the papillary muscle level shows dilated cardiac chambers. Note the thinned septal regions (A). Apical four-chamber view (B) shows four-chamber dilatation and apical tethering (tenting) of mitral valve leaflets—factors contributing to mitral regurgitation. Mitral inflow pulse Doppler velocity profile appears normal (C), but when integrated with the reduced velocities and reversed pattern seen on Doppler tissue imaging (D), should be interpreted as "pseudonormal"—both evidence of diastolic dysfunction. (Please see companion DVD for corresponding video.)

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