International Journal of Cardiology 179 (2015) 188–189
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Letter to the Editor
Apical hypertrophic cardiomyopathy: An echocardiography challenging case report Athanasios Angelis, Constantina Aggeli, Eleftherios Tsiamis, Dimitris Tousoulis ⁎ 1st Cardiology Department, Hippokration Hospital, Athens University Medical School, Greece
a r t i c l e
i n f o
Article history: Received 5 November 2014 Accepted 6 November 2014 Available online 8 November 2014 Keywords: Apical hypertrophic cardiomyopathy Contrast echocardiography Left ventriculography
Apical hypertrophic cardiomyopathy (AHCM) is a rare form of hypertrophic cardiomyopathy (HCM) that usually involves the apex of the left ventricle however right ventricular apex may be involved too. The mean age at presentation is 41.4 ± 14.5 years and is more frequent in Japan where it represents the 15% of the HCM population whereas in the western world a minor prevalence of 2–5% has been reported. Although low in prevalence, it is frequently accompanied by morbid conditions such as ventricular arrhythmias, atrial fibrillation, myocardial infraction and formation of apical aneurysms [1,2]. Electrocardiography and echocardiography are basic diagnostic modalities for the detection of the disease in the daily clinical practice. However, even based on characteristic patterns, its recognition frequently becomes obscured making diagnosis truly challenging [3–5]. 1. Case report We present the case of a 65 year old white female referred to our hospital for evaluation of effort angina symptoms occurring in the past month. She reports a history of well controlled primary hypertension for the last 5 years under pharmacological treatment with a combination of ramipril and hydrochlorothiazide. She has been repeatedly evaluated with 2D-echocardiography as a routine follow-up process for a hypertensive heart disease since a diagnosis of mild hypertrophy of both septal and posterior walls in a 2D transthoracic echocardiography study was established. There was no history of family heart disease, sudden cardiac death or other cardiovascular risk factors. The patient did not report any symptoms of dizziness, palpitation or syncope. On physical examination her blood pressure was 125/75 mm Hg and both S1 and S2 heart sounds were finely audible. Peripheral pulses were well palpated with no carotid or femoral auscultated murmur. On the surface 12 lead ECG, sinus rhythm was confirmed with normal QRS axis, prolonged P waves, normal QRS duration and negative T waves up to 0.5 mV in the ⁎ Corresponding author at: Athens University Medical School, Hippokration Hospital, Vasilissis Sofias 114, 115 28 Athens, Greece. E-mail address: [email protected] (D. Tousoulis).
http://dx.doi.org/10.1016/j.ijcard.2014.11.081 0167-5273/© 2014 Published by Elsevier Ireland Ltd.
precordial leads V3 to V6. No ECG signs of left ventricular hypertrophy were observed (RaVL b 11 mm, SV1 + RV5 b 35 mm, RaVL + SV3 b 20 mm) [6]. A 2D echocardiography examination revealed a mild hypertrophy of the left ventricle's walls and the papillary muscles, with a globally well preserved systolic function and no presence of valvular dysfunction (Fig. 1a). However, on the apical views, formation of the apex during systole raised suspicion of an excessively hypertrophied wall segment (Fig. 1b). Contrast echocardiography, a documented technique of left ventricle's cavity enhancement and delineation of regional dysfunction, thrombi and masses [3], confirmed a spade-like apex at systole, pathognomonic of a hypertrophic apical cardiomyopathy disease (Fig. 1c, d). Further diagnostic processing with coronary angiography and left ventriculography did not reveal any significant coronary artery disease but showed a characteristic imaging of an ace of spade pattern (Fig. 2). A remarkable tortuosity of the coronary vessels still could be attributed to chronic pressure overload as well as to an augmented myocardial mass [7,8]. Morphologically, AHCM can be divided into subgroups based on whether isolated asymmetric apical hypertrophy is the dominant phenotype (pure AHCM) or an interventricular septum hypertrophy co-exists (mixed AHCM) [2]. Nevertheless, clinical diagnosis relies upon criteria of asymmetric LV hypertrophy confined predominantly to the apex, on echocardiography or magnetic resonance imaging techniques [2,8,9]. Although the net values of an apical wall diastolic thickness of ≥15 mm and a ratio of maximal apical to posterior wall thickness ≥1.5 mm have been proposed [1,9], atypical phenotypes may confound diagnosis. Common ECG findings are negative T-waves in the precordial leads which are found in 93% of patients, followed by LV hypertrophy in 65% of patients. Twave negativity with a depth N 10 mV is found in 43% of patients with AHCM. Diastolic dysfunction that usually presents is responsible for the left atrial enlargement and consequently for the prolonged P waves [2,5,9,10]. Differential diagnosis includes apical cardiac tumors, apical thrombus, isolated ventricular non-compaction, hypertensive heart disease, endomyocardial fibrosis and coronary artery disease [2]. Regarding clinical presentation and in spite of a variety of symptoms such as palpitation, dyspnea, dizziness and syncope that relate to AHCM, the majority of patients (75%) present with angina [3]. In our case, there was not a typical diagnostic image of the disease. Apex hypertrophy during diastole was mild as well as left ventricle wall thickness. Consequently, on the surface 12 lead ECG negative T waves were not exceeding 5 mV, making even easier confounding with hypertensive or coronary heart disease. On a contrary, angina symptom was typically present since it may be caused by a variety of mechanisms including excess of hypertrophy, microvascular disease, diastolic dysfunction and epicardial obstructive coronary artery disease, all producing an imbalance between oxygen supply and demand in hypertrophic cardiomyopathy [3,11]. Another reason to miss the echocardiography diagnosis is the apical localization of hypertrophy that is not clearly seen as it interferes with reverberation artifacts from the chest wall and ribs. A possible solution is the use of second harmonic imaging although problematic too in patients with a thin chest wall since ultrasound has to travel some distance before harmonic frequencies are generated [8,11]. Contrast echocardiography, offers advantages on the abovementioned technical issues and furthermore is a bedside technique with findings analogous to left ventriculography on demonstrating a spade-like left ventricular cavity. In cases of a still diagnostic dilemma, magnetic resonance would offer a proper answer [12]. When angina symptoms are present, a coronary angiography with left ventriculography as a part of an integrated diagnostic procedure, will add critical information concerning coronary anatomy and myocardial structure [3,8]. In conclusion apical hypertrophic cardiomyopathy might become a challenging echocardiography diagnosis for non-typical phenotypes. In those cases when symptoms or ECG findings raise clinical dilemma on the disease's nature, use of an echo-contrast material as part of a bedside evaluation process helps improve accuracy and establish diagnosis. Magnetic resonance is a very suitable alternative imaging modality for identifying AHCM and offers critical information in cases of non-diagnostic echocardiography. Finally, when
A. Angelis et al. / International Journal of Cardiology 179 (2015) 188–189
189
Fig. 1. a) Apical 4 chamber view during diastole showing a mild hypertrophy of the left ventricle walls without excessive hypertrophy of the apex. Left atrial dilation is present. b) Apical 2 chamber view with mild hypertrophy of the left ventricle walls and the papillary muscles. c) After echo-contrast material intravenous injection there is still no typical disease pattern on diastole. d) Systole: The echo-contrast material reveals the hypertrophied apex.
References
Fig. 2. A left ventricle RAO projection during diastole. A hypertrophy of the cavity's walls with a normal internal diameter and formation of a spade pattern on the apex (arrow) was observed. angina symptoms are reported, coronary angiography will integrate the diagnostic procedure. Left ventriculography as a part of the catheterization protocol will confirm the typical pattern of the apex therefore establishing the proper diagnostic answer.
Conflict of interest None declared.
[1] M.J. Eriksson, B. Sonneberg, A. Woo, et al., Long term outcome in patients with apical hypertrophic cardiomyopathy, J. Am. Coll. Cardiol. 39 (2002) 638–645. [2] S.W. Yusuf, J.D. Bathina, J. Banchs, E.N. Mouhayar, I.N. Daher, Apical hypertrophic cardiomyopathy, World J. Cardiol. 3 (7) (2011) 256–259. [3] P. Soman, J. Swinburn, M. Callister, N.G. Stefens, R. Senior, Apical hypertrophic cardiomyopathy: bedside diagnosis by Intravenous contrast echocardiography, J. Am. Soc. Echocardiogr. 14 (2001) 311–313. [4] J.T. Levis, ECG diagnosis: apical hypertrophic cardiomyopathy, Perm. J. 17 (2) (Spring 2013) 84. [5] V. Reddy, C.L. Korcarz, L. Weinert, J. Al-Sadir, K.T. Spencer, R.M. Lang, Apical hypertrophic cardiomyopathy, Circulation 98 (1998) 2354. [6] M. Gerch, The ECG. A Two-step Approach to Diagnosis, Springer, 2004. 53. [7] M. Jacob, D. Spasojevic, O.N. Krogmann, H. Wiher, R. Hug, O.M. Heiss, Tortuosity of coronary arteries in chronic pressure and volume overload, Cathet. Cardiovasc. Diagn. 38 (1996) 25–31. [8] M. Ates, R.Y. Kwong, M.J. Lipton, S. Tatli, Apical hypertrophic cardiomyopathy diagnosed by cardiac magnetic resonance imaging, Imaging Cardiovasc. Med. 33 (2006) 408–409. [9] L.K. Williams, M.P. Frenneaux, R.R. Steeds, Echocardiography in hypertrophic cardiomyopathy diagnosis, prognosis and role management, Eur. J. Echocardiogr. 10 (2009) 9–14. [10] L.C. Alfonso, J. Bernal, J.J. Bax, T.P. Abraham, Echocardiography in hypertrophic cardiomyopathy. The role of conventional and emerging technologies, JACC Cardiovasc. Imaging 6 (2008) 787–800. [11] J. Wynne, E. Braunwald, Heart disease: a textbook of cardiovascular medicine, The Cardiomyopathies and Myocarditides, 5th ed.WB Saunders, Philadelphia, 1999, pp. 1404–1464. [12] J.C. Moon, N.G. Fisher, W.J. McKenna, D.J. Pennell, Detection of apical hypertrophic cardiomyopathy by cardiovascular magnetic resonance in patients with nondiagnostic echocardiography, Heart 6 (2004) 645–649.
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Letter to the Editor
Apical hypertrophic cardiomyopathy: An echocardiography challenging case report Athanasios Angelis, Constantina Aggeli, Eleftherios Tsiamis, Dimitris Tousoulis ⁎ 1st Cardiology Department, Hippokration Hospital, Athens University Medical School, Greece
a r t i c l e
i n f o
Article history: Received 5 November 2014 Accepted 6 November 2014 Available online 8 November 2014 Keywords: Apical hypertrophic cardiomyopathy Contrast echocardiography Left ventriculography
Apical hypertrophic cardiomyopathy (AHCM) is a rare form of hypertrophic cardiomyopathy (HCM) that usually involves the apex of the left ventricle however right ventricular apex may be involved too. The mean age at presentation is 41.4 ± 14.5 years and is more frequent in Japan where it represents the 15% of the HCM population whereas in the western world a minor prevalence of 2–5% has been reported. Although low in prevalence, it is frequently accompanied by morbid conditions such as ventricular arrhythmias, atrial fibrillation, myocardial infraction and formation of apical aneurysms [1,2]. Electrocardiography and echocardiography are basic diagnostic modalities for the detection of the disease in the daily clinical practice. However, even based on characteristic patterns, its recognition frequently becomes obscured making diagnosis truly challenging [3–5]. 1. Case report We present the case of a 65 year old white female referred to our hospital for evaluation of effort angina symptoms occurring in the past month. She reports a history of well controlled primary hypertension for the last 5 years under pharmacological treatment with a combination of ramipril and hydrochlorothiazide. She has been repeatedly evaluated with 2D-echocardiography as a routine follow-up process for a hypertensive heart disease since a diagnosis of mild hypertrophy of both septal and posterior walls in a 2D transthoracic echocardiography study was established. There was no history of family heart disease, sudden cardiac death or other cardiovascular risk factors. The patient did not report any symptoms of dizziness, palpitation or syncope. On physical examination her blood pressure was 125/75 mm Hg and both S1 and S2 heart sounds were finely audible. Peripheral pulses were well palpated with no carotid or femoral auscultated murmur. On the surface 12 lead ECG, sinus rhythm was confirmed with normal QRS axis, prolonged P waves, normal QRS duration and negative T waves up to 0.5 mV in the ⁎ Corresponding author at: Athens University Medical School, Hippokration Hospital, Vasilissis Sofias 114, 115 28 Athens, Greece. E-mail address: [email protected] (D. Tousoulis).
http://dx.doi.org/10.1016/j.ijcard.2014.11.081 0167-5273/© 2014 Published by Elsevier Ireland Ltd.
precordial leads V3 to V6. No ECG signs of left ventricular hypertrophy were observed (RaVL b 11 mm, SV1 + RV5 b 35 mm, RaVL + SV3 b 20 mm) [6]. A 2D echocardiography examination revealed a mild hypertrophy of the left ventricle's walls and the papillary muscles, with a globally well preserved systolic function and no presence of valvular dysfunction (Fig. 1a). However, on the apical views, formation of the apex during systole raised suspicion of an excessively hypertrophied wall segment (Fig. 1b). Contrast echocardiography, a documented technique of left ventricle's cavity enhancement and delineation of regional dysfunction, thrombi and masses [3], confirmed a spade-like apex at systole, pathognomonic of a hypertrophic apical cardiomyopathy disease (Fig. 1c, d). Further diagnostic processing with coronary angiography and left ventriculography did not reveal any significant coronary artery disease but showed a characteristic imaging of an ace of spade pattern (Fig. 2). A remarkable tortuosity of the coronary vessels still could be attributed to chronic pressure overload as well as to an augmented myocardial mass [7,8]. Morphologically, AHCM can be divided into subgroups based on whether isolated asymmetric apical hypertrophy is the dominant phenotype (pure AHCM) or an interventricular septum hypertrophy co-exists (mixed AHCM) [2]. Nevertheless, clinical diagnosis relies upon criteria of asymmetric LV hypertrophy confined predominantly to the apex, on echocardiography or magnetic resonance imaging techniques [2,8,9]. Although the net values of an apical wall diastolic thickness of ≥15 mm and a ratio of maximal apical to posterior wall thickness ≥1.5 mm have been proposed [1,9], atypical phenotypes may confound diagnosis. Common ECG findings are negative T-waves in the precordial leads which are found in 93% of patients, followed by LV hypertrophy in 65% of patients. Twave negativity with a depth N 10 mV is found in 43% of patients with AHCM. Diastolic dysfunction that usually presents is responsible for the left atrial enlargement and consequently for the prolonged P waves [2,5,9,10]. Differential diagnosis includes apical cardiac tumors, apical thrombus, isolated ventricular non-compaction, hypertensive heart disease, endomyocardial fibrosis and coronary artery disease [2]. Regarding clinical presentation and in spite of a variety of symptoms such as palpitation, dyspnea, dizziness and syncope that relate to AHCM, the majority of patients (75%) present with angina [3]. In our case, there was not a typical diagnostic image of the disease. Apex hypertrophy during diastole was mild as well as left ventricle wall thickness. Consequently, on the surface 12 lead ECG negative T waves were not exceeding 5 mV, making even easier confounding with hypertensive or coronary heart disease. On a contrary, angina symptom was typically present since it may be caused by a variety of mechanisms including excess of hypertrophy, microvascular disease, diastolic dysfunction and epicardial obstructive coronary artery disease, all producing an imbalance between oxygen supply and demand in hypertrophic cardiomyopathy [3,11]. Another reason to miss the echocardiography diagnosis is the apical localization of hypertrophy that is not clearly seen as it interferes with reverberation artifacts from the chest wall and ribs. A possible solution is the use of second harmonic imaging although problematic too in patients with a thin chest wall since ultrasound has to travel some distance before harmonic frequencies are generated [8,11]. Contrast echocardiography, offers advantages on the abovementioned technical issues and furthermore is a bedside technique with findings analogous to left ventriculography on demonstrating a spade-like left ventricular cavity. In cases of a still diagnostic dilemma, magnetic resonance would offer a proper answer [12]. When angina symptoms are present, a coronary angiography with left ventriculography as a part of an integrated diagnostic procedure, will add critical information concerning coronary anatomy and myocardial structure [3,8]. In conclusion apical hypertrophic cardiomyopathy might become a challenging echocardiography diagnosis for non-typical phenotypes. In those cases when symptoms or ECG findings raise clinical dilemma on the disease's nature, use of an echo-contrast material as part of a bedside evaluation process helps improve accuracy and establish diagnosis. Magnetic resonance is a very suitable alternative imaging modality for identifying AHCM and offers critical information in cases of non-diagnostic echocardiography. Finally, when
A. Angelis et al. / International Journal of Cardiology 179 (2015) 188–189
189
Fig. 1. a) Apical 4 chamber view during diastole showing a mild hypertrophy of the left ventricle walls without excessive hypertrophy of the apex. Left atrial dilation is present. b) Apical 2 chamber view with mild hypertrophy of the left ventricle walls and the papillary muscles. c) After echo-contrast material intravenous injection there is still no typical disease pattern on diastole. d) Systole: The echo-contrast material reveals the hypertrophied apex.
References
Fig. 2. A left ventricle RAO projection during diastole. A hypertrophy of the cavity's walls with a normal internal diameter and formation of a spade pattern on the apex (arrow) was observed. angina symptoms are reported, coronary angiography will integrate the diagnostic procedure. Left ventriculography as a part of the catheterization protocol will confirm the typical pattern of the apex therefore establishing the proper diagnostic answer.
Conflict of interest None declared.
[1] M.J. Eriksson, B. Sonneberg, A. Woo, et al., Long term outcome in patients with apical hypertrophic cardiomyopathy, J. Am. Coll. Cardiol. 39 (2002) 638–645. [2] S.W. Yusuf, J.D. Bathina, J. Banchs, E.N. Mouhayar, I.N. Daher, Apical hypertrophic cardiomyopathy, World J. Cardiol. 3 (7) (2011) 256–259. [3] P. Soman, J. Swinburn, M. Callister, N.G. Stefens, R. Senior, Apical hypertrophic cardiomyopathy: bedside diagnosis by Intravenous contrast echocardiography, J. Am. Soc. Echocardiogr. 14 (2001) 311–313. [4] J.T. Levis, ECG diagnosis: apical hypertrophic cardiomyopathy, Perm. J. 17 (2) (Spring 2013) 84. [5] V. Reddy, C.L. Korcarz, L. Weinert, J. Al-Sadir, K.T. Spencer, R.M. Lang, Apical hypertrophic cardiomyopathy, Circulation 98 (1998) 2354. [6] M. Gerch, The ECG. A Two-step Approach to Diagnosis, Springer, 2004. 53. [7] M. Jacob, D. Spasojevic, O.N. Krogmann, H. Wiher, R. Hug, O.M. Heiss, Tortuosity of coronary arteries in chronic pressure and volume overload, Cathet. Cardiovasc. Diagn. 38 (1996) 25–31. [8] M. Ates, R.Y. Kwong, M.J. Lipton, S. Tatli, Apical hypertrophic cardiomyopathy diagnosed by cardiac magnetic resonance imaging, Imaging Cardiovasc. Med. 33 (2006) 408–409. [9] L.K. Williams, M.P. Frenneaux, R.R. Steeds, Echocardiography in hypertrophic cardiomyopathy diagnosis, prognosis and role management, Eur. J. Echocardiogr. 10 (2009) 9–14. [10] L.C. Alfonso, J. Bernal, J.J. Bax, T.P. Abraham, Echocardiography in hypertrophic cardiomyopathy. The role of conventional and emerging technologies, JACC Cardiovasc. Imaging 6 (2008) 787–800. [11] J. Wynne, E. Braunwald, Heart disease: a textbook of cardiovascular medicine, The Cardiomyopathies and Myocarditides, 5th ed.WB Saunders, Philadelphia, 1999, pp. 1404–1464. [12] J.C. Moon, N.G. Fisher, W.J. McKenna, D.J. Pennell, Detection of apical hypertrophic cardiomyopathy by cardiovascular magnetic resonance in patients with nondiagnostic echocardiography, Heart 6 (2004) 645–649.
