International Journal of Cardiology 184 (2015) 394–396
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Letter to the Editor
Hypertrophic cardiomyopathy with apical aneurysm Jing Ping Sun, Xing Sheng Yang, Ka-Tak Wong, Cheuk-Man Yu ⁎ Prince of Wales Hospital, Chinese University of Hong Kong, Hong Kong
a r t i c l e
i n f o
Article history: Received 13 January 2015 Accepted 24 February 2015 Available online 26 February 2015 Keywords: Hypertrophic cardiomyopathy Aneurysm Echocardiography MRI
A 66 year-old man, presented with shortness of breath on exertion. Electrocardiogram: Sinus rhythm with a right bundle branch block pattern and old anterior myocardial infarction (V4-6 QS pattern). Angiogram: The coronary arteries were normal. Echocardiography: There was significant hypertrophy of the midventricular septum and posterior wall (1.4 vs. 1.6 cm) causing midventricular cavity obliteration. There was no obstruction in left ventricular outflow tract (mean pressure gradient was 1.7 mm Hg). The LV apex was thin with dyskinesia compatible with an aneurysm (Figs. 1, 2A, Videos 1 and 2). The left atrium was dilated. Mild to moderate mitral and tricuspid regurgitation was present. The peak velocity of tricuspid regurgitation was 3.69 m/s, with an estimated pulmonary artery pressure of 65 mm Hg. Magnetic resonance imaging (MRI): 4-chamber view demonstrates LV hypertrophy with mid-ventricular obstruction and the apex is thin with dyskinesia compatible with LV aneurysm (Fig. 2B; Video 3). Asymmetrical LV hypertrophy (septal wall = 1.7 cm, lateral wall = 1.4 cm), most pronounced at mid-ventricular level was noted causing mid-ventricular obstruction, perfusion scan showing gadolinium enhancement involving the interventricular septum, anterior, posterior and apical wall (Fig. 2C & D, arrows). Delayed gadolinium enhancement was seen in LV apex (transmural), anteroseptal/inferoseptal and inferior walls from basal to apical level
⁎ Corresponding author at: Division of Cardiology, Department of Medicine and Therapeutics, Prince of Wales Hospital, Li Ka Shing Institute of Health and Sciences, The Chinese University of Hong Kong, Hong Kong. E-mail address: [email protected] (C.-M. Yu).
http://dx.doi.org/10.1016/j.ijcard.2015.02.093 0167-5273/© 2015 Elsevier Ireland Ltd. All rights reserved.
(mid-layer) (Fig. 3A,B,C & D). LV systolic function is moderately impaired with a LVEF of 40%. Mild degree of RV apical hypertrophy was also seen. Overall features are compatible with hypertrophy cardiomyopathy with LV apical aneurysm formation and multifocal scarring. The distribution of patchy stress induced myocardial ischemia is more in favor of micro vascular disease rather than coronary artery stenosis. Hypertrophic cardiomyopathy (HCM) is a primary disease that affects the muscles of the heart. With HCM, the sarcomeres (contractile elements) in the heart replicate causing heart muscle cells to increase in size, which results in the thickening of the heart muscle. In addition, the normal alignment of muscle cells is disrupted, a phenomenon known as myocardial disarray. Hypertrophic cardiomyopathy is a genetically determined disease with diverse clinical manifestations and pathophysiological substrates. Patients with HCM and an LV apical aneurysm are an under-recognized but clinically important subset within the broad HCM disease spectrum. Mid-ventricular obstruction is an uncommon variant of LV obstructive HCM. It may lead to the development of an apical aneurysm, creating two distinct (basal and apical) LV chambers. The prevalence of LV apical aneurysms was 2% among 28 cases in one report [1]. The clinical course of patients with HCM and those with LV apical aneurysms vary but is largely unfavorable overall. Patients with HCM and large LV apical aneurysms (N4 cm diameter) are more likely to experience adverse complications than patients with small aneurysms (≤4 cm diameter) [1]. More than 40% of the patients with an LV apical aneurysm presented with bursts of nonsustained monomorphic VT on Holter ECGs; which has been reported to be a determinant of increased risk for sudden death in HCM [2]. The dyskinetic/akinetic apical aneurysm in HCM can also provide a structural basis for intracavitary thrombus formation. The overall rate of HCM with apical aneurysm-related adverse cardiovascular events such as sudden cardiac death, cardiac arrest, or progressive cardiac failure consequences was 10.5% per year, significantly higher than that reported in the general HCM population [1]. Echocardiography has been used extensively in diagnosis of HCM; however, it is likely to underestimate the true prevalence of apical aneurysms in the overall HCM population. MRI has higher spatial resolution and detection ability. Gadolinium-enhanced MRI has been validated for the detection of irreversible injury in myocardial infarction. In fibrosis and extracellular expansion, there is a greater extracellular space for gadolinium-DTPA accumulation, and the distribution kinetics is slower than normal myocardium [3]. In our case, the findings of echocardiography and MRI were compatible with the diagnosis of hypertrophic cardiomyopathy with apical aneurysm. Coronary angiogram was normal, thus the apical aneurysm would have been a result of microvascular disease. Microvascular dysfunction is a common feature of hypertrophic cardiomyopathy. Moreover,
J.P. Sun et al. / International Journal of Cardiology 184 (2015) 394–396
395
Fig. 1. Apical 3-chamber view showing left ventricle hypertrophy, most pronounced at mid-ventricular level causing LV obstruction in end systole (right), the apical aneurysm (arrow) can be noted during the whole cardiac cycle.
structural abnormalities of small vessels have been described in patients with hypertrophic cardiomyopathy and are thought to represent a primary abnormality [4]. A few studies have reported successful surgical therapy in patients with HCM and an LV apical aneurysm. Optimal treatment would prevent further episodes of arrhythmia and normalize LV shape and geometry. Supplementary data to this article can be found online at http://dx. doi.org/10.1016/j.ijcard.2015.02.093.
Disclosures None. Conflict of interest The authors report no relationships that could be construed as a conflict of interest.
Fig. 2. Transthoracic echocardiography apical 4-chamber view showing left ventricle hypertrophy, most pronounced at mid-ventricular level causing LV obstruction at early diastole (A). The cardiovascular magnetic resonance 4-chamber view demonstrates LV hypertrophy with mid-ventricular obstruction and an apical aneurysm (B), 4-chamber (C), 2-chamber views (D) showing gadolinium enhancement involving the interventricular septum, anterior, inferior and apical wall (arrows). LA, left atrium; VS, ventricular septum, LV, left ventricle; RV right ventricle; RA, right ventricle.
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J.P. Sun et al. / International Journal of Cardiology 184 (2015) 394–396
Fig. 3. Four-chamber, 2-chamber and short axis of mid-ventricular level views (A), (B) (C) showing late gadolinium enhancement involving the cardiac apex (transmural), interventricular septum and inferior wall (mid layer) (white arrows) consistent with fibrosis (D) Myocardial perfusion imaging demonstrating impaired perfusion in the anteroseptal wall and inferior wall (arrowheads). LA, left atrium; VS, ventricular septum, LV, left ventricle; RV right ventricle; RA, right ventricle.
References [1] M.S. Maron, J.J. Finley, J.M. Bos, T.H. Hauser, W.J. Manning, T.S. Haas, et al., Prevalence, clinical significance, and natural history of left ventricular apical aneurysms in hypertrophic cardiomyopathy, Circulation 118 (2008) 1541–1549. [2] B.J. Maron, Hypertrophic cardiomyopathy: a systematic review, JAMA 287 (2002) 1308–1320.
[3] S.J. Flacke, S.E. Fischer, C.H. Lorenz, Measurement of the gadopentetate dimeglumine partition coefficient in human myocardium in vivo normal distribution and elevation in acute and chronic infarction, Radiology 218 (3) (2001) 703–710. [4] B.J. Maron, J.K. Wolfson, S.E. Epstein, W.C. Roberts, Intramural (“small vessel”) coronary artery disease in hypertrophic cardiomyopathy, J. Am. Coll. Cardiol. 8 (1986) 545–557.
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Letter to the Editor
Hypertrophic cardiomyopathy with apical aneurysm Jing Ping Sun, Xing Sheng Yang, Ka-Tak Wong, Cheuk-Man Yu ⁎ Prince of Wales Hospital, Chinese University of Hong Kong, Hong Kong
a r t i c l e
i n f o
Article history: Received 13 January 2015 Accepted 24 February 2015 Available online 26 February 2015 Keywords: Hypertrophic cardiomyopathy Aneurysm Echocardiography MRI
A 66 year-old man, presented with shortness of breath on exertion. Electrocardiogram: Sinus rhythm with a right bundle branch block pattern and old anterior myocardial infarction (V4-6 QS pattern). Angiogram: The coronary arteries were normal. Echocardiography: There was significant hypertrophy of the midventricular septum and posterior wall (1.4 vs. 1.6 cm) causing midventricular cavity obliteration. There was no obstruction in left ventricular outflow tract (mean pressure gradient was 1.7 mm Hg). The LV apex was thin with dyskinesia compatible with an aneurysm (Figs. 1, 2A, Videos 1 and 2). The left atrium was dilated. Mild to moderate mitral and tricuspid regurgitation was present. The peak velocity of tricuspid regurgitation was 3.69 m/s, with an estimated pulmonary artery pressure of 65 mm Hg. Magnetic resonance imaging (MRI): 4-chamber view demonstrates LV hypertrophy with mid-ventricular obstruction and the apex is thin with dyskinesia compatible with LV aneurysm (Fig. 2B; Video 3). Asymmetrical LV hypertrophy (septal wall = 1.7 cm, lateral wall = 1.4 cm), most pronounced at mid-ventricular level was noted causing mid-ventricular obstruction, perfusion scan showing gadolinium enhancement involving the interventricular septum, anterior, posterior and apical wall (Fig. 2C & D, arrows). Delayed gadolinium enhancement was seen in LV apex (transmural), anteroseptal/inferoseptal and inferior walls from basal to apical level
⁎ Corresponding author at: Division of Cardiology, Department of Medicine and Therapeutics, Prince of Wales Hospital, Li Ka Shing Institute of Health and Sciences, The Chinese University of Hong Kong, Hong Kong. E-mail address: [email protected] (C.-M. Yu).
http://dx.doi.org/10.1016/j.ijcard.2015.02.093 0167-5273/© 2015 Elsevier Ireland Ltd. All rights reserved.
(mid-layer) (Fig. 3A,B,C & D). LV systolic function is moderately impaired with a LVEF of 40%. Mild degree of RV apical hypertrophy was also seen. Overall features are compatible with hypertrophy cardiomyopathy with LV apical aneurysm formation and multifocal scarring. The distribution of patchy stress induced myocardial ischemia is more in favor of micro vascular disease rather than coronary artery stenosis. Hypertrophic cardiomyopathy (HCM) is a primary disease that affects the muscles of the heart. With HCM, the sarcomeres (contractile elements) in the heart replicate causing heart muscle cells to increase in size, which results in the thickening of the heart muscle. In addition, the normal alignment of muscle cells is disrupted, a phenomenon known as myocardial disarray. Hypertrophic cardiomyopathy is a genetically determined disease with diverse clinical manifestations and pathophysiological substrates. Patients with HCM and an LV apical aneurysm are an under-recognized but clinically important subset within the broad HCM disease spectrum. Mid-ventricular obstruction is an uncommon variant of LV obstructive HCM. It may lead to the development of an apical aneurysm, creating two distinct (basal and apical) LV chambers. The prevalence of LV apical aneurysms was 2% among 28 cases in one report [1]. The clinical course of patients with HCM and those with LV apical aneurysms vary but is largely unfavorable overall. Patients with HCM and large LV apical aneurysms (N4 cm diameter) are more likely to experience adverse complications than patients with small aneurysms (≤4 cm diameter) [1]. More than 40% of the patients with an LV apical aneurysm presented with bursts of nonsustained monomorphic VT on Holter ECGs; which has been reported to be a determinant of increased risk for sudden death in HCM [2]. The dyskinetic/akinetic apical aneurysm in HCM can also provide a structural basis for intracavitary thrombus formation. The overall rate of HCM with apical aneurysm-related adverse cardiovascular events such as sudden cardiac death, cardiac arrest, or progressive cardiac failure consequences was 10.5% per year, significantly higher than that reported in the general HCM population [1]. Echocardiography has been used extensively in diagnosis of HCM; however, it is likely to underestimate the true prevalence of apical aneurysms in the overall HCM population. MRI has higher spatial resolution and detection ability. Gadolinium-enhanced MRI has been validated for the detection of irreversible injury in myocardial infarction. In fibrosis and extracellular expansion, there is a greater extracellular space for gadolinium-DTPA accumulation, and the distribution kinetics is slower than normal myocardium [3]. In our case, the findings of echocardiography and MRI were compatible with the diagnosis of hypertrophic cardiomyopathy with apical aneurysm. Coronary angiogram was normal, thus the apical aneurysm would have been a result of microvascular disease. Microvascular dysfunction is a common feature of hypertrophic cardiomyopathy. Moreover,
J.P. Sun et al. / International Journal of Cardiology 184 (2015) 394–396
395
Fig. 1. Apical 3-chamber view showing left ventricle hypertrophy, most pronounced at mid-ventricular level causing LV obstruction in end systole (right), the apical aneurysm (arrow) can be noted during the whole cardiac cycle.
structural abnormalities of small vessels have been described in patients with hypertrophic cardiomyopathy and are thought to represent a primary abnormality [4]. A few studies have reported successful surgical therapy in patients with HCM and an LV apical aneurysm. Optimal treatment would prevent further episodes of arrhythmia and normalize LV shape and geometry. Supplementary data to this article can be found online at http://dx. doi.org/10.1016/j.ijcard.2015.02.093.
Disclosures None. Conflict of interest The authors report no relationships that could be construed as a conflict of interest.
Fig. 2. Transthoracic echocardiography apical 4-chamber view showing left ventricle hypertrophy, most pronounced at mid-ventricular level causing LV obstruction at early diastole (A). The cardiovascular magnetic resonance 4-chamber view demonstrates LV hypertrophy with mid-ventricular obstruction and an apical aneurysm (B), 4-chamber (C), 2-chamber views (D) showing gadolinium enhancement involving the interventricular septum, anterior, inferior and apical wall (arrows). LA, left atrium; VS, ventricular septum, LV, left ventricle; RV right ventricle; RA, right ventricle.
396
J.P. Sun et al. / International Journal of Cardiology 184 (2015) 394–396
Fig. 3. Four-chamber, 2-chamber and short axis of mid-ventricular level views (A), (B) (C) showing late gadolinium enhancement involving the cardiac apex (transmural), interventricular septum and inferior wall (mid layer) (white arrows) consistent with fibrosis (D) Myocardial perfusion imaging demonstrating impaired perfusion in the anteroseptal wall and inferior wall (arrowheads). LA, left atrium; VS, ventricular septum, LV, left ventricle; RV right ventricle; RA, right ventricle.
References [1] M.S. Maron, J.J. Finley, J.M. Bos, T.H. Hauser, W.J. Manning, T.S. Haas, et al., Prevalence, clinical significance, and natural history of left ventricular apical aneurysms in hypertrophic cardiomyopathy, Circulation 118 (2008) 1541–1549. [2] B.J. Maron, Hypertrophic cardiomyopathy: a systematic review, JAMA 287 (2002) 1308–1320.
[3] S.J. Flacke, S.E. Fischer, C.H. Lorenz, Measurement of the gadopentetate dimeglumine partition coefficient in human myocardium in vivo normal distribution and elevation in acute and chronic infarction, Radiology 218 (3) (2001) 703–710. [4] B.J. Maron, J.K. Wolfson, S.E. Epstein, W.C. Roberts, Intramural (“small vessel”) coronary artery disease in hypertrophic cardiomyopathy, J. Am. Coll. Cardiol. 8 (1986) 545–557.
