Key Words Aneurysms Cardiomyopathies, hypertrophic Death, sudden Defibrillators, implantable Ventricular arrhythmia

Transcription

1 J Cardiol 2001 Dec; 38 6 : Mid-Ventricular Obstructive Hypertrophic Cardiomyopathy Associated With an Apical Aneurysm and Sustained Ventricular Tachycardia: A Case Report Koichi Teruo Kazuyoshi Shigeko Itaru Goro Rieko Hiroshi Kouichi Shigeo Hiroaki KONO, MD HIGASHI, MD HARA, MD MORI, MD ITO, MD SHINBO, MD ANZAI, MD YAGI, MD TAMANO, MD HORINAKA, MD MATSUOKA, MD, FJCC Abstract A 60-year-old woman presented with mid-ventricular obstructive hypertrophic cardiomyopathy associated with an apical aneurysm and sustained ventricular tachycardia. She was admitted because of drug refractory ventricular tachycardia. She had been treated with several antiarrhythmic agents, including amiodarone, but symptomatic episodes had continued. Echocardiography, magnetic resonance imaging, and left ventriculography showed mid-ventricular obstructive hypertrophic cardiomyopathy with an apical aneurysm. Electrophysiological study easily reproduced sustained pleomorphic ventricular tachycardia, polymorphic ventricular tachycardia, and ventricular fibrillation. The patient underwent implantation of a cardioverter-defibrillator. The relationship between mid-ventricular hypertrophic cardiomyopathy and apical aneurysm is unknown, but mid-ventricular hypertrophic cardiomyopathy is one of the causes of severe ventricular arrhythmias and sudden death. J Cardiol 2001 Dec ; 38 6 : Key Words Aneurysms Cardiomyopathies, hypertrophic Death, sudden Defibrillators, implantable Ventricular arrhythmia : Department of Hypertension and Cardiorenal Medicine, Dokkyo University School of Medicine, Tochigi Address for correspondence : KONO K, MD, Department of Hypertension and Cardiorenal Medicine, Dokkyo University School of Medicine, Kitakobayashi 880, Mibu-machi, Shimotsuga-gun, Tochigi Manuscript received April 10, 2001; revised July 16, 2001; accepted July 17,

2 344 Fig. 1 Twelve-lead electrocardiograms during normal sinus rhythm A and during spontaneous ventricular tachycardia B, C 60 : :50 59 : : : 110/66 mmhg60/min 4 Levine / 12 : QRS 0.14a F, 3 6 ST T Fig. 1 A 2Figs. 1 B, C Fig. 2 : 9mm 9mm 26 mm 22 mm 38mmHg Fig. 3 : 99 m Tc-methoxy-isobutyl isonitrile Fig. 4 : radioisotope : RIRI J Cardiol 2001 Dec; 38 6 :

3 345 Fig. 2 Color Doppler echocardiogram A and continuous wave Doppler recording B of the left mid-ventricular obstruction velocity Note disappearance of Doppler flow during mid-systole arrow. Peak flow velocity was more than 3.1 m/sec. LV left ventricle ; LA left atrium. Fig. 3 Magnetic resonance imaging at enddiastole showing left mid-ventricular obstructive hypertrophic cardiomyopathy with an apical aneurysm of the left ventricle Ao ascending thoracic aorta ; RA right atrium ; ANT anterior ; SEPT septal ; INF inferior ; LAT lateral. Other abbreviation as in Fig. 2. J Cardiol 2001 Dec; 38 6 :

4 346 Fig. 4 Technetium-99 m methoxy-isobutyl isonitrile scintigram at rest showing hypertrophic change of the left mid-ventricle, and perfusion defect in the left ventricular apex Impaired perfusion was also demonstrated in the anterior wall of the basal area and infero-posterior wall. Mid-d distal of the mid-ventricle; Mid-p proximal of the mid-ventricle. Other abbreviations as in Fig. 3. Fig. 5 Left ventriculograms in right anterior oblique projection at endsystole A and enddiastole B Arrows indicate the mid-ventricular obstruction with hypertrophy. Dotted lines indicate the outlines of the apical aneurysm of the left ventricle. RI : Fig. 5 2 : 200 mg/day6 Fig. 6 J Cardiol 2001 Dec; 38 6 :

5 347 Fig. 6 Twelve-lead electrocardiograms showing sustained monomorphic ventricular tachycardias induced with programmed ventricular stimulation during the electrophysiologic study The tachycardia cycle lengths were VT 1: 311 msec, VT 2: 338 msec, VT 3: 342 msec, VT 4: 356 msec, VT 5: 380msec, VT 6 : 442 msec. VT ventricular tachycardia. implantable cardioverter defibrillator : ICD, Medtronic Micro Jewel II model 7223Cx, Medtronic, Inc / 8,12 14Inoue 9 1 / J Cardiol 2001 Dec; 38 6 :

6 QRSsubstrate ICD 2ICD 3 ICD ICD 3 ICD 25% DDD 15,16 Hintringer 17 DDD 1 single chamber ICD ICD dual chamber ICD 60 J Cardiol 2001 Dec; 38 6 : Fananapazir L, Chang AC, Epstein SE, McAreavey D : Prognostic determinants in hypertrophic cardiomyopathy: Prospective evaluation of a therapeutic strategy based on clinical, Holter, hemodynamic, and electrophysiological findings. Circulation 1992; 86 : Kuck KH : Arrhythmias in hypertrophic cardiomyopathy. Pacing Clin Electrophysiol 1997; 20 : Maron BJ, Shen WK, Link MS, Epstein AE, Almquist AK, Daubert JP, Bardy GH, Favale S, Rea RF, Boriani G, Estes NA, Spirito P : Efficacy of implantable cardioverterdefibrillators for the prevention of sudden death in patients with hypertrophic cardiomyopathy. N Engl J Med 2000; 342: Sakamoto T, Tei C, Murayama M, Ichiyasu H, Hada Y : Giant T wave inversion as a manifestation of asymmetrical apical hypertrophy AAH of the left ventricle : Echocardiographic and ultrasono-cardiographic study. Jpn Heart J 1976; 17 : Yamaguchi H, Ishimura T, Nishiyama S, Nagasaki F, Nakanishi S, Takatsu F, Nishijo T, Umeda T, Machii K : Hypertrophic nonobstructive cardiomyopathy with giant negative T waves apical hypertrophy : Ventriculographic and echocardiographic features in 30 patients. Am J Cardiol 1979; 44 : Maron BJ, Bonow RO, Seshagiri TNR, Roberts WC, Epstein SE : Hypertrophic cardiomyopathy with ventricular septal hypertrophy localized to the apical region of the left ventricle apical hypertrophic cardiomyopathy. Am J Cardiol 1982; 49 : Falicov RF, Resnekov L, Bharati S, Lev M: Mid-ventricular obstruction: A variant of obstructive cardiomyopathy. Am J Cardiol 1976; 37: Fighali S, Krajcer Z, Edelman S, Leachman RD : Progression of hypertrophic cardiomyopathy into a hypokinetic left ventricle : Higher incidence in patients with midventricular obstruction. J Am Coll Cardiol 1987 ; 9 : Inoue T, Sunagawa O, Tohma T, Shinzato U, Fukiyama K: Apical hypertrophic cardiomyopathy followed by midventricular obstruction and apical aneurysm : A case report. J J Cardiol 2001 Dec; 38 6 :

7 349 Cardiol 1999; 33 : in Jpn with Eng abstr 10 Barbaresi F, Longhini C, Brunazzi C, Caneva M, Cotogni A, Musacci GF, Rigatelli G, Volta G: Idiopathic apical left ventricular aneurysm in hypertrophic cardiomyopathy : Report of 3 cases, and review of the literature. Jpn Heart J 1985; 26 : Akutsu Y, Shinozuka A, Huang TY, Watanabe T, Yamada T, Yamanaka H, Saitou T, Geshi E, Takenaka H, Takeyama Y, Munechika H, Ban Y, Katagiri T : Hypertrophic cardiomyopathy with apical left ventricular aneurysm. Jpn Circ J 1998; 62 : Sutton MG, Tajik AJ, Smith HC, Ritman EL : Angina in idiopathic hypertrophic subaortic stenosis : A clinical correlate of regional left ventricular dysfunction: A videometric and echocardiographic study. Circulation 1980 ; 61 : Waller BF, Maron BJ, Epstein SE, Roberts WC : Transmural myocardial infarction in hypertrophic cardiomyopathy: A cause of conversion from left ventricular asymmetry to symmetry and from normal-sized to dilated left ventricular cavity. Chest 1981; 79: Kitazume H, Kramer JR, Krauthamer D, El Tobgi S, Proudfit WL, Sones FM: Myocardial bridges in obstructive hypertrophic cardiomyopathy. Am Heart J 1983 ; 106 : Rishi F, Hulse JE, Auld DO, McRae G, Kaltman J, Kanter K, Williams W, Campbell RM : Effects of dual-chamber pacing for pediatric patients with hypertrophic obstructive cardiomyopathy. J Am Coll Cardiol 1997; 29 : Gadler F, Linde C, Daubert C, McKenna W, Meisel E, Aliot E, Chojnowska L, Guize L, Gras D, Jeanrenaud X, Kappenberger L, for the Pacing In Cardiomyopathy PIC study group: Significant improvement of quality of life following atrioventricular synchronous pacing in patients with hypertrophic obstructive cardiomyopathy : Data from 1 year of follow-up. Eur Heart J 1999; 20 : Hintringer F, Nesser HJ, Niel J, Baumgartner G, Aichinger J: Pacing in distal left ventricular hypertrophic cardiomyopathy. PACE 1998 ; 21 : J Cardiol 2001 Dec; 38 6 :