© 2014, Wiley Periodicals, Inc. DOI: 10.1111/echo.12778
Echocardiography
Unusual Myocardial Late Gadolinium Enhancement in Isolated Noncompaction Cardiomyopathy Sherif Moustafa, M.B.B.Ch.,*† David J. Patton, M.D.,‡ Mansour Al Shanawani, M.Sc.,§ Michael S. Connelly, M.B.B.S.,¶ Nanette Alvarez, M.D.,¶ Timothy Prieur, M.D.,¶ and Farouk Mookadam, M.D.† *Department of Cardiovascular Diseases, Prince Salman Heart Center, King Fahad Medical City, Riyadh, Saudi Arabia; †Division of Cardiovascular Diseases, Mayo Clinic Arizona, Scottsdale, Arizona; ‡Section of Pediatric Cardiology, University of Calgary, Calgary, Alberta, Canada; §Department of Radiology, Prince Salman Heart Center, King Fahad Medical City, Riyadh, Saudi Arabia; and ¶Division of Cardiovascular Diseases, University of Calgary, Calgary, Alberta, Canada
(Echocardiography 2015;32:400–402) Key words: noncompaction cardiomyopathy, echocardiography, magnetic resonance A 19-year-old man was evaluated due to recent onset of congestive heart failure. Past and family histories were unremarkable. Physical examination was noticeable for distention of neck veins, bilateral lower limb edema, bilateral basal lung rales, and third heart sound. Electrocardiogram revealed left bundle branch block. Chest x-ray showed cardiomegaly and increased bronchovascular markings.
Transthoracic echocardiography uncovered biventricular dilatation with severe global hypokinesis, mild-to-moderate mitral regurgitation, and moderate tricuspid regurgitation. Thickened left ventricular (LV) myocardium with prominent trabeculations (end systolic ratio of noncompacted to compacted layers >2), and evidence of direct blood flow from the ventricular cavity into deep intertrabecular recesses
Figure 1. Transthoracic echocardiography apical A. four-chamber and B. two-chamber views showing thickened LV/RV myocardium with prominent trabeculations (arrows). LV mural thrombus could not be excluded. LA = left atrium; RA = right atrium.
Address for correspondence and reprint requests: Sherif Moustafa, M.B.B.Ch., M.Sc., M.R.C.P. (UK), F.A.S.E., Mayo Clinic Arizona, 13400 East Shea Boulevard, Scottsdale, AZ 85259. Fax: (480) 301-8018; E-mail: [email protected]
400
Myocardial Fibrosis in Noncompaction Cardiomyopathy
Figure 2. Cardiac magnetic resonance (CMR) apical A. four-chamber and B. two-chamber steady-state free precession images demonstrating prominent ventricular trabeculations (arrows). CMR apical C. four-chamber and D. two-chamber delayed enhancement images showing intense late gadolinium enhancement of the left ventricular trabeculations extending into the endocardial side of the compacted myocardium up to the mid wall (yellow arrows). Two small mural thrombi were noted (white arrows).
by color Doppler were found as well. The right ventricle (RV) showed increased trabeculations and thickened walls. Those findings were consistent with noncompaction cardiomyopathy. However, the presence of mural clots within the LV cavity could not be excluded due to prominent trabeculations. Echo contrast was not administered as it was not available in our facility (Fig. 1, movie clips S1–S2). Cardiac magnetic resonance (CMR) was carried out to exclude the presence of LV mural thrombi for consideration of oral anticoagulation. CMR demonstrated a severely dilated LV with severely depressed contractility (LV ejection fraction = 14%) and multiple prominent trabeculations (the ratio of the noncompacted to compacted myocardium was 2.4 in diastole). Severely dilated heavily trabeculated RV with severe systolic dysfunction was appreciated (RV ejection fraction = 19%). Delayed enhancement images illustrated intense late gadolinium
enhancement (LGE) of the LV trabeculations extending into the endocardial side of the compacted myocardium up to the mid wall. Two small mural thrombi were noted as well (Fig. 2, movie clips S3–S4). The patient received oral anticoagulation, in addition to the standard therapy for heart failure, based on the notion of mural LV thrombi revealed by the CMR. Cardiac resynchronization therapy/defibrillator was performed 6 months later due to persistence of heart failure symptoms and poor LV ejection fraction. Genetic tests, to exclude familial dilated cardiomyopathy, was not performed due to patient’s denial. An amalgamated approach utilizing both echocardiography and CMR, with delayed enhancement sequences, offers a superb image quality permitting precise detailed assessment of the cardiac chambers including tissue characterization. This is critical for decision making regarding patients’ optimal management.1–4 401
Moustafa, et al.
References 1. Jenni R, Oechslin E, Schneider J, et al: Echocardiographic and pathoanatomical characteristics of isolated LV non-compaction: A step towards classification as a distinct cardiomyopathy. Heart 2001;86:666–671. 2. Kurita T, Matsuoka K, Hoshida K, et al: Unique myocardial fibrosis pattern by late gadolinium enhanced magnetic resonance imaging in a patient with isolated noncompaction of the ventricular myocardium. Circ J 2010;74:381– 382. 3. Petersen SE, Selvanayagam JB, Wiesmann F, et al: Left ventricular non-compaction: Insights from cardiovascular magnetic resonance imaging. J Am Coll Cardiol 2005;46:101–105. 4. Eilen D, Peterson N, Karkut C, et al: Isolated noncompaction of the left ventricular myocardium: A case report and literature review. Echocardiography 2008;25:755–761.
402
Supporting Information Additional Supporting Information may be found in the online version of this article: Movie clips S1–S2. TTE apical (1) fourchamber and (2) two-chamber views revealing severe biventricular systolic dysfunction with prominent trabeculations. LV mural thrombus could not be excluded. Movie clips S3–S4. CMR apical (3) four-chamber and (4) two-chamber cine images demonstrating severe biventricular systolic dysfunction with prominent trabeculations. Mitral and tricuspid regurgitation as well as pericardial/pleural effusion were appreciated.
Copyright of Echocardiography is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Echocardiography
Unusual Myocardial Late Gadolinium Enhancement in Isolated Noncompaction Cardiomyopathy Sherif Moustafa, M.B.B.Ch.,*† David J. Patton, M.D.,‡ Mansour Al Shanawani, M.Sc.,§ Michael S. Connelly, M.B.B.S.,¶ Nanette Alvarez, M.D.,¶ Timothy Prieur, M.D.,¶ and Farouk Mookadam, M.D.† *Department of Cardiovascular Diseases, Prince Salman Heart Center, King Fahad Medical City, Riyadh, Saudi Arabia; †Division of Cardiovascular Diseases, Mayo Clinic Arizona, Scottsdale, Arizona; ‡Section of Pediatric Cardiology, University of Calgary, Calgary, Alberta, Canada; §Department of Radiology, Prince Salman Heart Center, King Fahad Medical City, Riyadh, Saudi Arabia; and ¶Division of Cardiovascular Diseases, University of Calgary, Calgary, Alberta, Canada
(Echocardiography 2015;32:400–402) Key words: noncompaction cardiomyopathy, echocardiography, magnetic resonance A 19-year-old man was evaluated due to recent onset of congestive heart failure. Past and family histories were unremarkable. Physical examination was noticeable for distention of neck veins, bilateral lower limb edema, bilateral basal lung rales, and third heart sound. Electrocardiogram revealed left bundle branch block. Chest x-ray showed cardiomegaly and increased bronchovascular markings.
Transthoracic echocardiography uncovered biventricular dilatation with severe global hypokinesis, mild-to-moderate mitral regurgitation, and moderate tricuspid regurgitation. Thickened left ventricular (LV) myocardium with prominent trabeculations (end systolic ratio of noncompacted to compacted layers >2), and evidence of direct blood flow from the ventricular cavity into deep intertrabecular recesses
Figure 1. Transthoracic echocardiography apical A. four-chamber and B. two-chamber views showing thickened LV/RV myocardium with prominent trabeculations (arrows). LV mural thrombus could not be excluded. LA = left atrium; RA = right atrium.
Address for correspondence and reprint requests: Sherif Moustafa, M.B.B.Ch., M.Sc., M.R.C.P. (UK), F.A.S.E., Mayo Clinic Arizona, 13400 East Shea Boulevard, Scottsdale, AZ 85259. Fax: (480) 301-8018; E-mail: [email protected]
400
Myocardial Fibrosis in Noncompaction Cardiomyopathy
Figure 2. Cardiac magnetic resonance (CMR) apical A. four-chamber and B. two-chamber steady-state free precession images demonstrating prominent ventricular trabeculations (arrows). CMR apical C. four-chamber and D. two-chamber delayed enhancement images showing intense late gadolinium enhancement of the left ventricular trabeculations extending into the endocardial side of the compacted myocardium up to the mid wall (yellow arrows). Two small mural thrombi were noted (white arrows).
by color Doppler were found as well. The right ventricle (RV) showed increased trabeculations and thickened walls. Those findings were consistent with noncompaction cardiomyopathy. However, the presence of mural clots within the LV cavity could not be excluded due to prominent trabeculations. Echo contrast was not administered as it was not available in our facility (Fig. 1, movie clips S1–S2). Cardiac magnetic resonance (CMR) was carried out to exclude the presence of LV mural thrombi for consideration of oral anticoagulation. CMR demonstrated a severely dilated LV with severely depressed contractility (LV ejection fraction = 14%) and multiple prominent trabeculations (the ratio of the noncompacted to compacted myocardium was 2.4 in diastole). Severely dilated heavily trabeculated RV with severe systolic dysfunction was appreciated (RV ejection fraction = 19%). Delayed enhancement images illustrated intense late gadolinium
enhancement (LGE) of the LV trabeculations extending into the endocardial side of the compacted myocardium up to the mid wall. Two small mural thrombi were noted as well (Fig. 2, movie clips S3–S4). The patient received oral anticoagulation, in addition to the standard therapy for heart failure, based on the notion of mural LV thrombi revealed by the CMR. Cardiac resynchronization therapy/defibrillator was performed 6 months later due to persistence of heart failure symptoms and poor LV ejection fraction. Genetic tests, to exclude familial dilated cardiomyopathy, was not performed due to patient’s denial. An amalgamated approach utilizing both echocardiography and CMR, with delayed enhancement sequences, offers a superb image quality permitting precise detailed assessment of the cardiac chambers including tissue characterization. This is critical for decision making regarding patients’ optimal management.1–4 401
Moustafa, et al.
References 1. Jenni R, Oechslin E, Schneider J, et al: Echocardiographic and pathoanatomical characteristics of isolated LV non-compaction: A step towards classification as a distinct cardiomyopathy. Heart 2001;86:666–671. 2. Kurita T, Matsuoka K, Hoshida K, et al: Unique myocardial fibrosis pattern by late gadolinium enhanced magnetic resonance imaging in a patient with isolated noncompaction of the ventricular myocardium. Circ J 2010;74:381– 382. 3. Petersen SE, Selvanayagam JB, Wiesmann F, et al: Left ventricular non-compaction: Insights from cardiovascular magnetic resonance imaging. J Am Coll Cardiol 2005;46:101–105. 4. Eilen D, Peterson N, Karkut C, et al: Isolated noncompaction of the left ventricular myocardium: A case report and literature review. Echocardiography 2008;25:755–761.
402
Supporting Information Additional Supporting Information may be found in the online version of this article: Movie clips S1–S2. TTE apical (1) fourchamber and (2) two-chamber views revealing severe biventricular systolic dysfunction with prominent trabeculations. LV mural thrombus could not be excluded. Movie clips S3–S4. CMR apical (3) four-chamber and (4) two-chamber cine images demonstrating severe biventricular systolic dysfunction with prominent trabeculations. Mitral and tricuspid regurgitation as well as pericardial/pleural effusion were appreciated.
Copyright of Echocardiography is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
