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CASE REPORT
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Systolic Flow Reversal in a Case of Mid-Ventricular Obstructive Hypertrophic Cardiomyopathy Ikuo Misumi 1, Tsuyoshi Honda 1, Hirofumi Kurokawa 1, Yuji Kubota 1, Masanobu Ishii 2, Ryota Sato 2, Hiroshige Yamabe 2, Hisayo Yasuda 2, Koichi Kaikita 2, Seiji Hokimoto 2 and Hisao Ogawa 2
Abstract A 69-year-old man presented to our hospital with chest pain. Two-dimensional transthoracic echocardiography showed hypertrophy of the left ventricle, mid-ventricular obstruction and an apical aneurysm. Color-flow imaging at the obstruction site on the apical four-chamber view demonstrated systolic flow reversal in addition to a paradoxical jet flow. The systolic flow reversal may have been caused by a decreased apical contractility and pressure during systole. Key words: systolic flow reversal, mid-ventricular obstruction, Doppler echocardiography (Intern Med 54: 1765-1769, 2015) (DOI: 10.2169/internalmedicine.54.4153)
Introduction Apical aneurysms may be present in patients with midventricular obstructive hypertrophic cardiomyopathy (MVOCM) (1) and are frequently associated with an unusual type of intraventricular isovolumetric relaxation flow called a paradoxical jet flow (2). We herein report a rare case of MVOCM associated with additional systolic flow reversal from the base of the ventricle (LV) to the apex.
Case Report A 69-year-old man presented to our hospital after experiencing chest pain and a transient loss of consciousness while driving a taxi. His blood pressure was 140/95 mmHg and his pulse rate was 87 beats/min. Auscultation showed a Levine 2/6 systolic murmur at the right upper sternal border, while a 12-lead electrocardiogram (ECG) showed a sinus rhythm with complete right bundle branch block and negative T waves. In addition, a chest radiograph disclosed a cardiothoracic ratio of 0.52, and two-dimensional transthoracic echocardiography revealed hypertrophy of the left LV (inter-
ventricular septum: 14 mm thick, and LV posterior wall: 13 mm thick) as well as mid-ventricular obstruction and an apical aneurysm (Fig. 1). Continuous-wave Doppler echocardiography of the site of obstruction (Fig. 2) and color-flow imaging in the apical four-chamber view (Fig. 3) demonstrated systolic mid-ventricular obstruction and a paradoxical jet flow typical of MVOCM. Furthermore, there was a systolic flow reversal from the LV base to the apex between the systolic and paradoxical jet flow (Fig. 2, 3), and cardiac MRI imaging showed massive LV hypertrophy and midventricular obstruction (Fig. 4, right panel, arrows). The patient underwent cardiac catheterization. Although no significant coronary artery stenosis was observed, coronary spasms were induced by the intracoronary injection of acetylcholine. The patient’s hemodynamic data showed a mean pulmonary capillary wedge pressure of 12 mmHg, LV end-diastolic pressure of 25 mmHg, cardiac output of 5.5 L/ min and cardiac index of 2.6 L/min/m2. There were no significant peak systolic pressure gradients in the LV apex, LV base or aorta (Fig. 5). The patient was subsequently treated with a calcium channel blocker and angiotensin II receptor blocker and discharged from our hospital.
１
Department of Cardiology, Kumamoto Saisyunsou Hospital, Japan and ２Department of Cardiovascular Medicine, Kumamoto University School of Medicine, Japan Received for publication September 24, 2014; Accepted for publication November 19, 2014 Correspondence to Dr. Ikuo Misumi, [email protected]
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Intern Med 54: 1765-1769, 2015
DOI: 10.2169/internalmedicine.54.4153
Figure 1. Apical four-chamber view in diastole (left panel) and systole (right panel) shows an apical aneurysm (An.) and mid-ventricular obstruction (arrows).
Figure 2. Continuous Doppler echocardiography at the site of left ventricular obstruction shows mid-systolic flow reversal (a) and a paradoxical jet flow (b).
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Intern Med 54: 1765-1769, 2015
DOI: 10.2169/internalmedicine.54.4153
Figure 3. Color-flow imaging in the apical four-chamber view in systole (left panel), late-systole (middle panel) and isovolumetric relaxation (right panel) during the cardiac cycle. Systolic flow reversal is observed between the systolic forward flow and paradoxical jet flow.
Figure 4. Cardiac MRI imaging shows massive LV hypertrophy with mid-ventricular obstruction (arrows).
Discussion MVOCM may be accompanied by apical aneurysm formation. Although the precise pathogenesis of apical aneurysms is unknown, these lesions may be caused by high apical pressure due to mid-ventricular obstruction (3). Another proposed mechanism underlying aneurysm formation is myocardial ischemia. The oxygen supply may decrease as a result of an impaired coronary flow reserve, de-
creased coronary perfusion pressure, squeezing of the coronary arteries and coronary spasms (4, 5). The oxygen demand is also increased by high apical pressure and wall stress (6). Moreover, enlargement of the apex and thinning of the wall caused by aneurysm formation may increase wall stress, which subsequently induces positive feedback, thus leading to a vicious cycle (Fig. 6). This case may be the second reported case demonstrating additional transient mid-systolic flow reversal between the
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Intern Med 54: 1765-1769, 2015
DOI: 10.2169/internalmedicine.54.4153
Figure 5. Pressure recording during cardiac catheterization (lower panels). The upper panels show the positions of pressure recording. There were no significant peak pressure gradients in the LV apex (left panel), LV base or aorta.
Figure 6. Pathogenesis underlying the formation of a left ventricular apical aneurysm in the setting of mid-ventricular obstructive hypertrophic cardiomyopathy (MVOCM).
systolic flow and a paradoxical jet flow. We previously encountered a case of atrial fibrillation in a patient with MVOCM and systolic flow reversal, suggesting that atrial fibrillation contributes to the development of this unusual flow (7). However, the present patient had a sinus rhythm. We therefore consider that the systolic flow reversal may
have been caused by a decrease in the systolic apical pressure equal to the LV basal pressure as a result of hypokinesis of the apex (Fig. 6, left lower). In conclusion, we herein reported a rare case of systolic flow reversal in a patient with MVOCM exhibiting an apical aneurysm.
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Intern Med 54: 1765-1769, 2015
DOI: 10.2169/internalmedicine.54.4153
The authors state that they have no Conflict of Interest (COI).
References 1. Maron MS, Finley JJ, Bos JM, et al. Prevalence, clinical significance, and natural history of left ventricular apical aneurysms in hypertrophic cardiomyopathy. Circulation 118: 1541-1549, 2008. 2. Hanaoka Y, Misumi I, Rokutanda T, et al. Simultaneous pressure recording in mid-ventricular obstructive hypertrophic cardiomyopathy. Intern Med 51: 387-390, 2012. 3. Matsubara K, Nakamura T, Kuribayashi T, et al. Sustained cavity obliteration and apical aneurysm formation in apical hypertrophic cardiomyopathy. J Am Coll Cardiol 42: 288-295, 2003.
4. Sato Y, Matsumoto N, Matsuo S, Yoda S, Kunimoto S, Saito S. Mid-ventricular hypertrophic obstructive cardiomyopathy presenting with acute myocardial infarction. Tex Heart Inst J 34: 475478, 2007. 5. Harada K, Shimizu T, Sugishita Y, et al. Hypertrophic cardiomyopathy with midventricular obstruction and apical aneurysm: a case report. Jpn Circ J 65: 915-919, 2001. 6. Grossman W, Jones D, McLaurin LP. Wall stress and patterns of hypertrophy in the human left ventricle. J Clin Invest 56: 56-64, 1975. 7. Ito M, Misumi I, Rokutanda T, et al. Mid-systolic flow reversal in a patient with mid-ventricular obstructive hypertrophic cardiomyopathy. J Echocardiogr 12: 78-80, 2014.
Ⓒ 2015 The Japanese Society of Internal Medicine http://www.naika.or.jp/imonline/index.html
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CASE REPORT
□
Systolic Flow Reversal in a Case of Mid-Ventricular Obstructive Hypertrophic Cardiomyopathy Ikuo Misumi 1, Tsuyoshi Honda 1, Hirofumi Kurokawa 1, Yuji Kubota 1, Masanobu Ishii 2, Ryota Sato 2, Hiroshige Yamabe 2, Hisayo Yasuda 2, Koichi Kaikita 2, Seiji Hokimoto 2 and Hisao Ogawa 2
Abstract A 69-year-old man presented to our hospital with chest pain. Two-dimensional transthoracic echocardiography showed hypertrophy of the left ventricle, mid-ventricular obstruction and an apical aneurysm. Color-flow imaging at the obstruction site on the apical four-chamber view demonstrated systolic flow reversal in addition to a paradoxical jet flow. The systolic flow reversal may have been caused by a decreased apical contractility and pressure during systole. Key words: systolic flow reversal, mid-ventricular obstruction, Doppler echocardiography (Intern Med 54: 1765-1769, 2015) (DOI: 10.2169/internalmedicine.54.4153)
Introduction Apical aneurysms may be present in patients with midventricular obstructive hypertrophic cardiomyopathy (MVOCM) (1) and are frequently associated with an unusual type of intraventricular isovolumetric relaxation flow called a paradoxical jet flow (2). We herein report a rare case of MVOCM associated with additional systolic flow reversal from the base of the ventricle (LV) to the apex.
Case Report A 69-year-old man presented to our hospital after experiencing chest pain and a transient loss of consciousness while driving a taxi. His blood pressure was 140/95 mmHg and his pulse rate was 87 beats/min. Auscultation showed a Levine 2/6 systolic murmur at the right upper sternal border, while a 12-lead electrocardiogram (ECG) showed a sinus rhythm with complete right bundle branch block and negative T waves. In addition, a chest radiograph disclosed a cardiothoracic ratio of 0.52, and two-dimensional transthoracic echocardiography revealed hypertrophy of the left LV (inter-
ventricular septum: 14 mm thick, and LV posterior wall: 13 mm thick) as well as mid-ventricular obstruction and an apical aneurysm (Fig. 1). Continuous-wave Doppler echocardiography of the site of obstruction (Fig. 2) and color-flow imaging in the apical four-chamber view (Fig. 3) demonstrated systolic mid-ventricular obstruction and a paradoxical jet flow typical of MVOCM. Furthermore, there was a systolic flow reversal from the LV base to the apex between the systolic and paradoxical jet flow (Fig. 2, 3), and cardiac MRI imaging showed massive LV hypertrophy and midventricular obstruction (Fig. 4, right panel, arrows). The patient underwent cardiac catheterization. Although no significant coronary artery stenosis was observed, coronary spasms were induced by the intracoronary injection of acetylcholine. The patient’s hemodynamic data showed a mean pulmonary capillary wedge pressure of 12 mmHg, LV end-diastolic pressure of 25 mmHg, cardiac output of 5.5 L/ min and cardiac index of 2.6 L/min/m2. There were no significant peak systolic pressure gradients in the LV apex, LV base or aorta (Fig. 5). The patient was subsequently treated with a calcium channel blocker and angiotensin II receptor blocker and discharged from our hospital.
１
Department of Cardiology, Kumamoto Saisyunsou Hospital, Japan and ２Department of Cardiovascular Medicine, Kumamoto University School of Medicine, Japan Received for publication September 24, 2014; Accepted for publication November 19, 2014 Correspondence to Dr. Ikuo Misumi, [email protected]
1765
Intern Med 54: 1765-1769, 2015
DOI: 10.2169/internalmedicine.54.4153
Figure 1. Apical four-chamber view in diastole (left panel) and systole (right panel) shows an apical aneurysm (An.) and mid-ventricular obstruction (arrows).
Figure 2. Continuous Doppler echocardiography at the site of left ventricular obstruction shows mid-systolic flow reversal (a) and a paradoxical jet flow (b).
1766
Intern Med 54: 1765-1769, 2015
DOI: 10.2169/internalmedicine.54.4153
Figure 3. Color-flow imaging in the apical four-chamber view in systole (left panel), late-systole (middle panel) and isovolumetric relaxation (right panel) during the cardiac cycle. Systolic flow reversal is observed between the systolic forward flow and paradoxical jet flow.
Figure 4. Cardiac MRI imaging shows massive LV hypertrophy with mid-ventricular obstruction (arrows).
Discussion MVOCM may be accompanied by apical aneurysm formation. Although the precise pathogenesis of apical aneurysms is unknown, these lesions may be caused by high apical pressure due to mid-ventricular obstruction (3). Another proposed mechanism underlying aneurysm formation is myocardial ischemia. The oxygen supply may decrease as a result of an impaired coronary flow reserve, de-
creased coronary perfusion pressure, squeezing of the coronary arteries and coronary spasms (4, 5). The oxygen demand is also increased by high apical pressure and wall stress (6). Moreover, enlargement of the apex and thinning of the wall caused by aneurysm formation may increase wall stress, which subsequently induces positive feedback, thus leading to a vicious cycle (Fig. 6). This case may be the second reported case demonstrating additional transient mid-systolic flow reversal between the
1767
Intern Med 54: 1765-1769, 2015
DOI: 10.2169/internalmedicine.54.4153
Figure 5. Pressure recording during cardiac catheterization (lower panels). The upper panels show the positions of pressure recording. There were no significant peak pressure gradients in the LV apex (left panel), LV base or aorta.
Figure 6. Pathogenesis underlying the formation of a left ventricular apical aneurysm in the setting of mid-ventricular obstructive hypertrophic cardiomyopathy (MVOCM).
systolic flow and a paradoxical jet flow. We previously encountered a case of atrial fibrillation in a patient with MVOCM and systolic flow reversal, suggesting that atrial fibrillation contributes to the development of this unusual flow (7). However, the present patient had a sinus rhythm. We therefore consider that the systolic flow reversal may
have been caused by a decrease in the systolic apical pressure equal to the LV basal pressure as a result of hypokinesis of the apex (Fig. 6, left lower). In conclusion, we herein reported a rare case of systolic flow reversal in a patient with MVOCM exhibiting an apical aneurysm.
1768
Intern Med 54: 1765-1769, 2015
DOI: 10.2169/internalmedicine.54.4153
The authors state that they have no Conflict of Interest (COI).
References 1. Maron MS, Finley JJ, Bos JM, et al. Prevalence, clinical significance, and natural history of left ventricular apical aneurysms in hypertrophic cardiomyopathy. Circulation 118: 1541-1549, 2008. 2. Hanaoka Y, Misumi I, Rokutanda T, et al. Simultaneous pressure recording in mid-ventricular obstructive hypertrophic cardiomyopathy. Intern Med 51: 387-390, 2012. 3. Matsubara K, Nakamura T, Kuribayashi T, et al. Sustained cavity obliteration and apical aneurysm formation in apical hypertrophic cardiomyopathy. J Am Coll Cardiol 42: 288-295, 2003.
4. Sato Y, Matsumoto N, Matsuo S, Yoda S, Kunimoto S, Saito S. Mid-ventricular hypertrophic obstructive cardiomyopathy presenting with acute myocardial infarction. Tex Heart Inst J 34: 475478, 2007. 5. Harada K, Shimizu T, Sugishita Y, et al. Hypertrophic cardiomyopathy with midventricular obstruction and apical aneurysm: a case report. Jpn Circ J 65: 915-919, 2001. 6. Grossman W, Jones D, McLaurin LP. Wall stress and patterns of hypertrophy in the human left ventricle. J Clin Invest 56: 56-64, 1975. 7. Ito M, Misumi I, Rokutanda T, et al. Mid-systolic flow reversal in a patient with mid-ventricular obstructive hypertrophic cardiomyopathy. J Echocardiogr 12: 78-80, 2014.
Ⓒ 2015 The Japanese Society of Internal Medicine http://www.naika.or.jp/imonline/index.html
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