**5. Acquired primary and secondary cardiomyopathy**

For the purpose of this review, discussion will be limited to key features of commonly encountered acquired cardiomyopathy.

#### **5.1. Inflammatory myocarditis**

adverse prognosis [41]. In another study, 3DE derived measurement of RV volume and

Routine use of Doppler-derived strain and 2D strain may have limited application in clinically manifest disease. Application of these techniques in preclinical state and in asymptomatic family members with inherited type of DCM may identify at risk subset of patients. Observa‐ tion of intersegmental discordance in the timing of strain measures, particularly those of opposing segments identify a subset of DCM patients with LV dyssynchrony, who may benefit

Primary restrictive cardiomyopathy (RCM) predominantly affects the elderly, with a slight female predominance [43]. Clinical presentation is with signs and symptoms of systemic and pulmonary venous congestion from diastolic heart failure and pulmonary HTN [43]. As opposed to other types of primary cardiomyopathies which have distinctive morphologic abnormalities, the diagnosis of RCM is largely dependent on an altered physiology of blood flow through the heart consequent to a non compliant ventricle. The condition has no distinc‐ tive histologic features [44]. RCM should be distinguished from infiltrative disorders of the heart where, in addition to restrictive physiology which may be indistinguishable from RCM, distinctive morphologic and histopathologic changes are present. Amyloid heart disease and

Left ventricle (LV) appearance and contractility is usually normal. LV cavity size may be small. Biatrial enlargement in the absence of significant regurgitation of mitral and tricuspid valves or atrial fibrillation and with normal LV kinetics in patients with signs and symptoms of heart failure should prompt consideration of RCM (Figure-7). Impaired diastolic relaxation of the LV is encountered but a key diagnostic feature is the presence of restrictive physiology on Doppler as evidenced by an increase in E:A ratio >2 with rapid deceleration of early mitral inflow (E) velocity, usually to < 150 msec (Figure-7) [45]. This, in conjunction with reduced early mitral annular velocity (E') and elevated E/E' ratio, is confirmatory of elevated left ventricular end diastolic pressure (LVEDP). Reduced E' velocity, reflecting underlying myocardial disease, is useful in distinguishing RCM from constrictive pericarditis where mitral annular velocities are preserved [46-47]. Deformation of the LV on 2D speckle strain is constrained in the circumferential direction in constrictive pericarditis and in the longitudinal direction in RCM [48]. Flow propagation velocity on color M-mode of mitral inflow can

**4.2. Restrictive cardiomyopathy (non-hypertrophied and non-dilated)**

endomyocardial fibrosis are typical examples of the latter.

provide additional insight into diastolic dysfunction of RCM.

*4.2.2. Features of RCM on standard echocardiogram*

function was superior to conventional method [42].

from cardiac resynchronization therapy.

*4.1.3. Novel echo techniques*

14 Cardiomyopathies

*4.2.1. Introduction*

Inflammatory cardiomyopathy is defined by myocarditis in association with cardiac dysfunc‐ tion [11]. Idiopathic, autoimmune, and infectious forms of inflammatory cardiomyopathy are recognized [11]. Echo findings are of non-specific LV cavity dilatation associated with global LV dysfunction similar to that seen in idiopathic dilated cardiomyopathy. Regional variation in LV contractility is not infrequently encountered.

#### **5.2. Takotsubo cardiomyopathy (stress cardiomyopathy)**

A transient and reversible cardiomyopathy first reported in Japan by Dote, et al., in 1991 [49]. Clinical presentation may be indistinguishable from acute coronary syndrome, invariably necessitating coronary angiography for exclusion of obstructive coronary artery disease. Prevalence is about 1-2% of patients undergoing coronary angiography for acute coronary syndrome. A precipitating emotional or physical stressor is typical. Complimentary imaging modalities including echocardiography and cardiac MRI are helpful in diagnosis and in monitoring clinical recovery. In fact, LV morphology at echocardiography is characteristic as it resembles a takotsubo (Japanese octopus trap) with dilatation of the apical region of the heart and preserved contractility of basal segments (Figure-8). About a fifth of patients will have hyperdynamic contractility of basal LV segments with consequent left ventricular outflow tract obstruction and systemic hypotension. Early recognition of this by echo has marked influence on therapeutic choice [50]. Reverse pattern of LV contractile dysfunction has been described. Right ventricular involvement in takotsubo is seen in 25-30% of patients and is associated with a more complicated clinical course [51]. It is encountered in patients with more severe LV involvement [52]. However, isolated right ventricular takotsubo has been reported [53]. The condition is prone to formation of LV apical mural thrombus which should be carefully excluded in all.

**5.3. PeriPartum Cardiomyopathy (PPCM)**

**5.4. Tachycardia Induced Cardiomyopathy (TIC)**

history of PPCM.

**5.5. Ischemic cardiomyopathy**

particularly in patients with LBBB [64].

**5.6. Valvular cardiomyopathy**

The diagnosis of PPCM rests on the echocardiographic identification of new left ventricular systolic dysfunction during a limited period surrounding childbirth. Other causes of cardio‐ myopathy should be excluded [54]. Features are typically that of dilated cardiomyopathy, though LV cavity dimensions may be normal. Echocardiogram is used to monitor the effec‐ tiveness of treatment. In one study of PPCM recovery of LV function was reported in 54% of study population [55]. This was more likely to happen in women with EF of > 30% at diagnosis [55]. However, even in those with recovery of resting LV function by echo, contractile reserve on dobutamine echo is reduced [56].The condition is likely to recur during subsequent pregnancy even following recovery of LV function. The condition is associated with a worse prognosis where recovery of LV function is incomplete or did not occur after the index pregnancy [57]. Patients with LVEF of < 25% at diagnosis or in whom LV function has not normalized should be counseled against subsequent pregnancy [58]. Early and serial echo‐ cardiogram may be considered during subsequent pregnancies in all patients with prior

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There are no diagnostic features of TIC on echocardiogram. Non-specific dilated cardiomyop‐ athy may ensue from chronic tachyarrhythmia of either supraventricular or ventricular origin [59-60].TreatmentoftachyarrhythmiaisassociatedwithrecoveryofLVsystolicfunction,though some degree of adverse LV remodeling may persist [61]. Diastolic dysfunction by echo is

On standard echocardiogram findings that constitute ischemic cardiomyopathy include regional wall motion abnormalities, wall thinning with aneurysmal dilatation of the infarcted myocardial segment, left ventricular (LV) cavity dilatation and decline in LV systolic per‐

Beyond recognition of an underlying ischemic process, an issue that frequently merits clarification is that of hibernating viable myocardium and non-viable infarcted myocardium [63]. This is particularly difficult to distinguish when infarcted myocardial segments have normal or relatively normal thickness or when global LV contractile dysfunction as that seen in DCM is encountered. For this low-dose dobutamine echocardiography test is useful. Augmented contractility with dobutamine typically seen as a biphasic response is noted with hibernating myocardium. Newer methods of assessing longitudinal fiber contraction during dobutamine echo by long axis pulsed wave Doppler and M-mode is considered to be superior,

It is defined as ventricular dysfunction that is out of proportion to the abnormal loading conditions of the heart [11]. Left ventricle is affected by regurgitant lesions of the mitral and

encountered which may not reverse after normalization of LV systolic function [62].

formance that is out of proportion to the degree of underlying CAD (Figure-9).

**Figure 8.** Diastolic and systolic frame of the LV in acute phase of takotsubo cardiomyopathy (panels A and B, respec‐ tively) and during recovery (panels C and D, respectively). Apical systolic expansion is noted during acute illness (panel B). Apical endocardial border is highlighted in red. Normalization of apical systolic morphology and function is noted upon recovery (panel D).

#### **5.3. PeriPartum Cardiomyopathy (PPCM)**

modalities including echocardiography and cardiac MRI are helpful in diagnosis and in monitoring clinical recovery. In fact, LV morphology at echocardiography is characteristic as it resembles a takotsubo (Japanese octopus trap) with dilatation of the apical region of the heart and preserved contractility of basal segments (Figure-8). About a fifth of patients will have hyperdynamic contractility of basal LV segments with consequent left ventricular outflow tract obstruction and systemic hypotension. Early recognition of this by echo has marked influence on therapeutic choice [50]. Reverse pattern of LV contractile dysfunction has been described. Right ventricular involvement in takotsubo is seen in 25-30% of patients and is associated with a more complicated clinical course [51]. It is encountered in patients with more severe LV involvement [52]. However, isolated right ventricular takotsubo has been reported [53]. The condition is prone to formation of LV apical mural thrombus which should be carefully

**Figure 8.** Diastolic and systolic frame of the LV in acute phase of takotsubo cardiomyopathy (panels A and B, respec‐ tively) and during recovery (panels C and D, respectively). Apical systolic expansion is noted during acute illness (panel B). Apical endocardial border is highlighted in red. Normalization of apical systolic morphology and function is noted

excluded in all.

16 Cardiomyopathies

upon recovery (panel D).

The diagnosis of PPCM rests on the echocardiographic identification of new left ventricular systolic dysfunction during a limited period surrounding childbirth. Other causes of cardio‐ myopathy should be excluded [54]. Features are typically that of dilated cardiomyopathy, though LV cavity dimensions may be normal. Echocardiogram is used to monitor the effec‐ tiveness of treatment. In one study of PPCM recovery of LV function was reported in 54% of study population [55]. This was more likely to happen in women with EF of > 30% at diagnosis [55]. However, even in those with recovery of resting LV function by echo, contractile reserve on dobutamine echo is reduced [56].The condition is likely to recur during subsequent pregnancy even following recovery of LV function. The condition is associated with a worse prognosis where recovery of LV function is incomplete or did not occur after the index pregnancy [57]. Patients with LVEF of < 25% at diagnosis or in whom LV function has not normalized should be counseled against subsequent pregnancy [58]. Early and serial echo‐ cardiogram may be considered during subsequent pregnancies in all patients with prior history of PPCM.

#### **5.4. Tachycardia Induced Cardiomyopathy (TIC)**

There are no diagnostic features of TIC on echocardiogram. Non-specific dilated cardiomyop‐ athy may ensue from chronic tachyarrhythmia of either supraventricular or ventricular origin [59-60].TreatmentoftachyarrhythmiaisassociatedwithrecoveryofLVsystolicfunction,though some degree of adverse LV remodeling may persist [61]. Diastolic dysfunction by echo is encountered which may not reverse after normalization of LV systolic function [62].

#### **5.5. Ischemic cardiomyopathy**

On standard echocardiogram findings that constitute ischemic cardiomyopathy include regional wall motion abnormalities, wall thinning with aneurysmal dilatation of the infarcted myocardial segment, left ventricular (LV) cavity dilatation and decline in LV systolic per‐ formance that is out of proportion to the degree of underlying CAD (Figure-9).

Beyond recognition of an underlying ischemic process, an issue that frequently merits clarification is that of hibernating viable myocardium and non-viable infarcted myocardium [63]. This is particularly difficult to distinguish when infarcted myocardial segments have normal or relatively normal thickness or when global LV contractile dysfunction as that seen in DCM is encountered. For this low-dose dobutamine echocardiography test is useful. Augmented contractility with dobutamine typically seen as a biphasic response is noted with hibernating myocardium. Newer methods of assessing longitudinal fiber contraction during dobutamine echo by long axis pulsed wave Doppler and M-mode is considered to be superior, particularly in patients with LBBB [64].

#### **5.6. Valvular cardiomyopathy**

It is defined as ventricular dysfunction that is out of proportion to the abnormal loading conditions of the heart [11]. Left ventricle is affected by regurgitant lesions of the mitral and

*5.6.2. Aortic regurgitation*

*5.6.3. Mitral regurgitation*

dilated cardiomyopathy.

*5.8.1. Amyloid heart disease*

**5.8. Metabolic cardiomyopathy**

≤ 60% is used to time surgical intervention [68].

**5.7. Hypertensive heart disease and cardiomyopathy**

LV cavity enlargement is present, which can be marked. Systolic function is initially preserved but declines with advanced disease. Increase in LV end-diastolic pressure may blunt the color Doppler signal of aortic regurgitation. Serial estimation of LV cavity dimension and volume is necessary for aortic valve replacement prior to irreversible contractile dysfunction. LVEF of ≤ 50%, LV end-diastolic dimension of ≥ 70 mm and LV end-systolic dimension of ≥ 50 mm are echo criteria for surgical intervention in asymptomatic individuals [66]. Abnormal longitudi‐ nal and circumferential strain is noted in the preclinical phase [67]. Incremental value of these

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LV volume overload is well tolerated with preserved LV systolic function early in the disease. Progressive decline in LV systolic function can be underestimated when using LV ejection fraction as a marker of systolic performance. Left atrial enlargement is followed by LV cavity enlargement. Progressive increase in left atrial pressure may decrease the color Doppler signal of mitral regurgitation in advanced cases. LV end-systolic dimension of ≥ 45 mm or LVEF of

There is an increase in LV mass consequent to concentric hypertrophy of LV. Systolic function is preserved. Variable degree of diastolic function is noted. In patients with severe LV hypertrophy echocardiographic differentiation from other disease states with LV hypertrophy is challenging. Restrictive filling pattern is seen in severe disease. Progressive disease is associated with LV cavity dilatation and decline in LV systolic function similar to that seen in

Cardiac amyloidosis is an infiltrative disorder of the heart which on echo is seen as thick-walled left and right ventricles with normal left ventricular cavity dimension and systolic function. Advanced disease is associated with decline in left ventricular systolic function. Increased echogenicity from thickening of heart valves, biatrial enlargement and thickened interatrial septum are other morphologic features of established disease. Pericardial effusion is seen in more than half of patients [69] (Figure-10). Increased granular appearance of the heart in earlier description of cardiac amyloidosis is not distinct on modern echo hardware and image processing [70]. Assessment of transmitral flow and mitral annular velocities by Doppler reveals impaired diastolic function. Restrictive diastolic filling pattern is noted in advanced cases. Unlike in restrictive filling pattern, reduced mitral A- velocity may be seen with normal mitral E-deceleration time. This finding suggests atrial myopathy and reduced contractility from amyloid infiltration [71]. Some overlapping clinical and echocardiographic features of

strain parameters for therapeutic intervention is not well established.

**Figure 9.** Apical LV aneurysm (white arrow) is seen in a patient with ischemic cardiomyopathy. In another patient api‐ cal LV aneurysm is associated with a large LV mural thrombus (red arrow).

aortic valves and by increased afterload of aortic stenosis. Primary valve abnormality can be readily identified though findings can be blunted in the failing heart.

#### *5.6.1. Aortic stenosis*

Increased afterload of aortic stenosis results in concentric left ventricular (LV) hypertrophy. LV cavity size is normal and systolic function preserved. With progressive disease LV dilatation and impaired systolic function ensues. In patients with severe systolic dysfunction assessment of aortic stenosis by Doppler can be challenging due to low flow velocity. Inotropic augmentation of contractility and flow with dobutamine can be used in such cases [65].

#### *5.6.2. Aortic regurgitation*

LV cavity enlargement is present, which can be marked. Systolic function is initially preserved but declines with advanced disease. Increase in LV end-diastolic pressure may blunt the color Doppler signal of aortic regurgitation. Serial estimation of LV cavity dimension and volume is necessary for aortic valve replacement prior to irreversible contractile dysfunction. LVEF of ≤ 50%, LV end-diastolic dimension of ≥ 70 mm and LV end-systolic dimension of ≥ 50 mm are echo criteria for surgical intervention in asymptomatic individuals [66]. Abnormal longitudi‐ nal and circumferential strain is noted in the preclinical phase [67]. Incremental value of these strain parameters for therapeutic intervention is not well established.

#### *5.6.3. Mitral regurgitation*

LV volume overload is well tolerated with preserved LV systolic function early in the disease. Progressive decline in LV systolic function can be underestimated when using LV ejection fraction as a marker of systolic performance. Left atrial enlargement is followed by LV cavity enlargement. Progressive increase in left atrial pressure may decrease the color Doppler signal of mitral regurgitation in advanced cases. LV end-systolic dimension of ≥ 45 mm or LVEF of ≤ 60% is used to time surgical intervention [68].

#### **5.7. Hypertensive heart disease and cardiomyopathy**

There is an increase in LV mass consequent to concentric hypertrophy of LV. Systolic function is preserved. Variable degree of diastolic function is noted. In patients with severe LV hypertrophy echocardiographic differentiation from other disease states with LV hypertrophy is challenging. Restrictive filling pattern is seen in severe disease. Progressive disease is associated with LV cavity dilatation and decline in LV systolic function similar to that seen in dilated cardiomyopathy.

#### **5.8. Metabolic cardiomyopathy**

#### *5.8.1. Amyloid heart disease*

aortic valves and by increased afterload of aortic stenosis. Primary valve abnormality can be

**Figure 9.** Apical LV aneurysm (white arrow) is seen in a patient with ischemic cardiomyopathy. In another patient api‐

Increased afterload of aortic stenosis results in concentric left ventricular (LV) hypertrophy. LV cavity size is normal and systolic function preserved. With progressive disease LV dilatation and impaired systolic function ensues. In patients with severe systolic dysfunction assessment of aortic stenosis by Doppler can be challenging due to low flow velocity. Inotropic augmentation of contractility and flow with dobutamine can be used in such cases [65].

readily identified though findings can be blunted in the failing heart.

cal LV aneurysm is associated with a large LV mural thrombus (red arrow).

*5.6.1. Aortic stenosis*

18 Cardiomyopathies

Cardiac amyloidosis is an infiltrative disorder of the heart which on echo is seen as thick-walled left and right ventricles with normal left ventricular cavity dimension and systolic function. Advanced disease is associated with decline in left ventricular systolic function. Increased echogenicity from thickening of heart valves, biatrial enlargement and thickened interatrial septum are other morphologic features of established disease. Pericardial effusion is seen in more than half of patients [69] (Figure-10). Increased granular appearance of the heart in earlier description of cardiac amyloidosis is not distinct on modern echo hardware and image processing [70]. Assessment of transmitral flow and mitral annular velocities by Doppler reveals impaired diastolic function. Restrictive diastolic filling pattern is noted in advanced cases. Unlike in restrictive filling pattern, reduced mitral A- velocity may be seen with normal mitral E-deceleration time. This finding suggests atrial myopathy and reduced contractility from amyloid infiltration [71]. Some overlapping clinical and echocardiographic features of hypertrophic cardiomyopathy are seen in 5% of cases. In contrast to a hypertrophied ventricle, low voltage in precordial leads is seen on ECG, and systolic anterior motion of mitral valve which is a frequent observation in hypertrophic cardiomyopathy on echo, is uncommon in patients with cardiac amyloidosis. By novel echo techniques longitudinal strain and strain rate show systolic dysfunction despite preserved radial contraction as determined by fractional shortening. The value of strain parameters in early diagnosis and in prognosis is being evaluated [72]. Furthermore, strain measurement by 2D speckle tracking shows variation in longitudinal strain from base to apex with relative preservation of apical strain. This finding can be helpful in distinguishing cardiac amyloidosis from hypertrophic cardiomyopathy and hypertrophy associated with increased afterload state of aortic stenosis [73].

peutic regimens. Dose dependent cardiotoxicity from anthracyclines is reversible if detected early and upon institution of effective heart failure therapy [76]. Serial assessment of left ventricle systolic function, preferably by echo, is routine in such cases. Impaired tissue kinetics by measures of myocardial strain and strain rate is noted prior to gross impairment of left

Value of echocardiography in the diagnosis, prognosis and monitoring of therapy in patients with cardiomyopathy is discussed in the preceding review. 3DE, Doppler and speckle strain and left ventricular torsion may have a role in preclinical disease states. Incorporation of these diagnostic methods in routine clinical assessment of patients with cardiomyopathy is depend‐

2 Faculty of Medicine and Health Sciences, United Arab Emirates University, Al Ain, United

[1] Jenkins, C, Bricknell, K, Hanekom, L, & Marwick, T. H. Reproducibility and accuracy of echocardiographic measurements of left ventricular parameters using real-time

[2] Sonne, C, Sugeng, L, Takeuchi, M, et al. Real-Time 3-Dimensional Echocardiographic Assessment of Left Ventricular Dyssynchrony: Pitfalls in Patients with Dilated Cardi‐

[3] Nagueh, S. F, Middleton, K. J, Kopelen, H. A, Zoghbi, W. A, & Quinones, M. A. Dop‐ pler tissue imaging: a noninvasive technique for evaluation of left ventricular relaxa‐ tion and estimation of filling pressures. *J Am Coll Cardiol*. (1997). , 30, 1527-1533.

[4] Ommen, S. R, Nishimura, R. A, Appleton, C. P, Miller, F. A, Oh, J. K, Redfield, M. M, & Tajik, A. J. Clinical utility of Doppler echocardiography and tissue Doppler imag‐

three-dimensional echocardiography, J Am Coll Cardiol , 44-2004.

omyopathy. J Am Coll Cardiol Img. (2009). , 2(7), 802-812.

and Anwer Qureshi1\*

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21

ventricle systolic function. This may have a role in influencing management [77].

ent on emerging data on the usefulness and reproducibility of these techniques.

, Abdullah Shehab2

1 Division of Cardiology, Tawam Hospital, Al Ain, United Arab Emirates

\*Address all correspondence to: aqureshi@tawamhospital.ae

**6. Conclusion**

**Author details**

, Ahmed Abbas1

Gohar Jamil1

Arab Emirates

**References**

**Figure 10.** Severe concentric left ventricular wall thickening and small pericardial effusion is present in this patient with cardiac amyloidosis (panel A). Impaired mitral annular velocity is indicative of abnormal diastolic function (re‐ duced mitral annular velocities with reversal of E' to A' ratio) (panel B).

#### *5.8.2. Hemochromatosis*

There are no specific morphologic features on echocardiogram. Dilated cardiomyopathy is seen in advanced stages of hemochromatosis [74]. Non-invasive diagnosis of cardiac involve‐ ment is dependent on demonstration of myocardial iron deposit on cardiac MRI [75]. In cases with established cardiac involvement, assessment of myocardial kinetics by Doppler and tissue strain may reveal functional impairment prior to development of overt cardiomyopathy. The value of these new techniques in determining prognosis and in serial follow up of patients following therapeutic intervention has been the subject of recent studies.

#### **5.9. Toxic cardiomyopathy: Alcohol and anthracyclines**

Echo findings are non-specific. Dilated cardiomyopathy from cardiotoxicity of alcohol cannot be distinguished from idiopathic dilated cardiomyopathy. Impairment of left ventricle systolic function is a concern for both anthracycline and some non-anthracycline based chemothera‐ peutic regimens. Dose dependent cardiotoxicity from anthracyclines is reversible if detected early and upon institution of effective heart failure therapy [76]. Serial assessment of left ventricle systolic function, preferably by echo, is routine in such cases. Impaired tissue kinetics by measures of myocardial strain and strain rate is noted prior to gross impairment of left ventricle systolic function. This may have a role in influencing management [77].
