© 2013, Wiley Periodicals, Inc. DOI: 10.1111/echo.12395

Echocardiography

ECHO ROUNDS Section Editor: Edmund Kenneth Kerut, M.D.

Cardiac Amyloidosis Cases with Relative Apical Sparing of Longitudinal Strain Cem Bostan, M.D., Umit Yasar Sinan, M.D., Polat Canbolat, M.D., and Serdar Kucukoglu, M.D. Department of Cardiology, Institute of Cardiology, Istanbul University, Istanbul, Turkey

(Echocardiography 2014;31:241–244)

Amyloidosis is a clinical disorder caused by extracellular deposition of insoluble abnormal fibrils, derived from aggregation of misfolded normally soluble protein.1 Cardiac amyloidosis (CA) describes clinically significant involvement of the heart by amyloid deposition, which may or may not be associated with involvement of other organs. An echocardiogram demonstrating marked left ventricle (LV) wall thickening particularly in the absence of hypertension, biatrial enlargement, thickened valve leaflets, and a pericardial effusion in the context of reduced voltage of R-waves in electrocardiogram (ECG) is highly persuasive of cardiac amyloid. If either a thickened interatrial septum or a granular highly echogenic myocardium is also present, this makes the diagnosis even more likely.2 This thickening is often referred to incorrectly as “hypertrophy” because the pathological process is infiltration, not myocyte hypertrophy. The absence of high ECG voltages in advanced disease may be more specific for infiltrative diseases, of which amyloid is the most common.3 Echocardiography in CA can detect abnormalities in systolic and diastolic function of the myocardium even before ejection fraction (EF) is impaired.4,5 Strain (S) and strain rate (SR) has been shown to be superior to tissue Doppler techniques in this respect.6 Lately, the prognostic value of S and SR was, also, shown.7 We report 2 cases with similar clinical and echocardiographic features highly suggestive of CA. First patient (patient A) was a 50-year-old male. He had history of familial Mediterranean fever for several years using cholchicum dispert. The second patient (patient B) was also male (48year old). He had type 2 diabetes mellitus controlled with oral antidiabetic drugs. Both had no history of ischemic and hypertensive heart Address for correspondence and reprint requests: Cem Bostan, M.D., Department of Cardiology, Istanbul University, Institute of Cardiology, Haseki, Fatih 34350 Istanbul, Turkey. Fax: 90 (216) 469-3796; E-mail: [email protected]

disease and had symptoms and signs of congestive heart failure. Their ECGs were similar presenting low voltage in limb leads. Patient A had also anterior and inferior pseudoinfarct pattern (Fig. 1A, B). Their transthoracic echocardiography (GE Healthcare, Horten, Norway) revealed increased wall thickness and systolic dysfunction in both LV and right ventricle (RV). (LV EF 30%, Tricuspid Annular Plane Systolic Excursion 1.3 cm) with normal chamber size, mild-to-moderate mitral and tricuspid insufficiency, mild pulmonary hypertension, and mild pericardial effusion. (Fig. 2A, B) We also performed twodimensional speckle tracking echocardiography (GE Healthcare). Longitudinal strain (LS) measurements were performed off line using automated software (EchoPAC Version 108.1.2. Advanced Analysis Technologies; GE Healthcare), using 3 standard apical views, the LV endocardium was manually identified and tissue speckles were automatically tracked frame by frame throughout the cardiac cycle. A bull’s-eye plot illustrating segmental LS values was automatically generated. Both the patients had lower global LS. ( 7.5, 4.5). The majority of segments in the basal and mid-ventricular regions had reduced LS, whereas apical segments had normal LS. Apical sparing was consistently seen in both (Fig. 3A, B). Patient A who had a salivary gland biopsy which showed non AA type amyloid deposition died 1 month after the diagnosis of congestive heart failure. Patient B had a rectal biopsy which showed AA type amyloid deposition and is still being followed. Amyloidosis should be considered in any patient older than 40 years who has nephrotic syndrome, congestive heart failure (not on an ischemic basis), idiopathic peripheral neuropathy, or unexplained hepatomegaly.8 There is an increasing realization that the prevalence of CA may be higher than previously expected and, not uncommonly, that the diagnosis can go unrecognized.9 Although a tissue biopsy is required to define the type of CA, the clinical challenge 241

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Figure 1. Electrocardiographies of patients.

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Figure 2. Parasternal long-axis transthoracic echocardiographic views of patients.

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Longitudinal Strain in Cardiac Amyloidosis

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Figure 3. Bull’s-eye plots illustrating segmental longitudinal strain values of patients.

often lies in identifying the disease early and differentiating it from other causes of increased wall thickness. Biopsy of the rectum has been considered by many investigators to be the gold standard for screening with a sensitivity of 80%.10,11 Combining features of history, physical examination, ECG, and echocardiography have been shown to be reliable at differentiating cardiac amyloid from other diagnoses.5 Murtagh et al.12 provide the largest report to date of ECG findings in a population of patients with amyloidosis and biopsy-proven cardiac involvement. They found that low ECG voltage and pseudoinfarct patterns were the most common abnormalities. Although no single noninvasive test is pathognomonic of cardiac amyloid, a combination of a typical echocardiographic appearance and low-voltage ECG complexes is highly suggestive of disease.13 There is now a growing interest in the use of strain imaging to characterize patients with suspected CA, with several studies illustrating that a decrease in peak systolic global LS can be identified even before any of the traditional echocardiographic parameters become abnormal.14 Relative sparing of LS in the LV apex as assessed using two-dimensional speckle tracking is highly sensitive and specific for the diagnosis of CA among patients with increased LV wall thickness.7,14 Both patients had similar symptoms, ECG and echocardiographic findings as well as low global LS ( 7.5 and 4.5) and relative apical sparing of LS suggestive of CA. In conclusion, cardiac amyloid is characterized by regional variations in LS from base to apex. A relative apical sparing pattern of LS is an easily

recognizable, accurate, and reproducible method of differentiating CA from other causes of left ventricular hypertrophy. Even if cardiac systolic functions seem normal with echocardiography regional wall-motion abnormalities and regional differences can be diagnosed by strain imaging even in early stages of CA. References 1. Merlini G, Westermark P: The systemic amyloidoses: Clearer understanding of the molecular mechanisms offers hope for more effective therapies. J Intern Med 2004;255:159–178. 2. Bhandari AK, Nanda NC: Myocardial texture characterization by two-dimensional echocardiography. Am J Cardiol 1983;51:817–825. 3. Burroughs EI, Aronson AE, Duffy JR, et al: Speech disorders in systemic amyloidosis. Br J Disord Commun 1991;26:201–206. 4. Koyama J, Ray-Sequin PA, Davidoff R, et al: Usefulness of pulsed tissue Doppler imaging for evaluating systolic and diastolic left ventricular function in patients with AL (primary) amyloidosis. Am J Cardiol 2002;89:1067– 1071. 5. Bellavia D, Pellikka PA, Abraham TP, et al: Evidence of impaired left ventricular systolic function by Doppler myocardial imaging in patients with systemic amyloidosis and no evidence of cardiac involvement by standard two-dimensional and Doppler echocardiography. Am J Cardiol 2008;101:1039– 1045. 6. Koyama J, Ray-Sequin PA, Falk RH: Longitudinal myocardial function assessed by tissue velocity, strain, and strain rate tissue Doppler echocardiography in patients with AL (primary) cardiac amyloidosis. Circulation 2003;107: 2446–2452. 7. Koyama J, Falk RH: Prognostic significance of strain Doppler imaging in light-chain amyloidosis. JACC Cardiovasc Imaging 2010;3:333e42 8. Gertz MA, Lacy MQ, Dispenzieri A: Amyloidosis: Recognition, confirmation, prognosis, and therapy. Mayo Clin Proc 1999;74:490–494.

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9. Guan J, Mishra S, Falk RH, et al: Current perspectives on cardiac amyloidosis. Am J Physiol Heart Circ Physiol 2012;302:H544–H552. 10. Kyle RA, Gertz MA: Primary systemic amyloidosis: Clinical and laboratory features in 474 cases. Semin Hematol 1995;32:45–59. 11. Janssen S, van Rijswijk MH, Meijer S, et al: Systemic amyloidosis: A clinical survey of 144 cases. Neth J Med 1986;29:376–385. 12. Murtagh B, Hammill SC, Gertz MA, et al: Electrocardiographic findings in primary systemic amyloidosis and

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biopsy-proven cardiac involvement. Am J Cardiol 2005;95:535–537. 13. Selvanayagam JB, Hawkins PN, Paul B, et al: Evaluation and management of the cardiac amyloidosis. J Am Coll Cardiol 2007;50:2101–2110. 14. Phelan D, Collier P, Thavendiranathan P, et al: Relative apical sparing of longitudinal strain using two-dimensional speckle-tracking echocardiography is both sensitive and specific for the diagnosis of cardiac amyloidosis. Heart 2012;98:1442–1448.

Cardiac amyloidosis cases with relative apical sparing of longitudinal strain.

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