Assessment of Pathophysiology Based on the Left Ventricular Shape in Five Patients With Midventricular Obstructive Hypertrophic Cardiomyopathy

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1 : 5 Assessment of Pathophysiology Based on the Left Ventricular Shape in Five Patients With Midventricular Obstructive Hypertrophic Cardiomyopathy Mayumi Kazumi Hiromi Noriko Takao Kenjiro AOKI, MD UEKITA, MD OBATA, MD MAKIGUCHI, MD MITSUOKA, MD KIKUCHI, MD, FJCC Abstract Objectives. The pathophysiology of midventricular obstructive hypertrophic cardiomyopathymvois unknown. Patients with MVO and MVO-like cardiomyopathy were classified into three groups based on the cardioimaging morphological characteristics of the left ventricle to investigate their complications and treatment. Methods. Four patients with MVO and one patient with disease-like MVO were admitted in our hospital from 1999 to Group A consisted of one patient with indications of pressure gradient at mid-ventricle without apical aneurysm, Group B consisted of three patients with indications of pressure gradient and apical aneurysm, and Group C consisted of one patient with hour-glass appearance with apical aneurysm and decreased left ventricular systolic function without pressure gradient. Results. The diagnosis was established during examination for sustained ventricular tachycardiasvt, three patients, paroxysmal atrial fibrillationone patient, and coronary artery diseaseone patient. Cardiogenic embolization was observed in all cases which originated from atrial fibrillationone caseand apical aneurysmtwo cases. No embolic event occurred in any patient after warfarin therapy. SVT occurred in patients in Groups B and C. SVT refractory to beta-blocker and mexiletine was treated by amiodarone. Apical aneurysmectomy and cryoablation could prevent recurrent SVT with drug resistance. Conclusions. Four of the five patients with MVO had arrhythmiaatrial fibrillation, SVTand three had cardiogenic embolization. MVO could be classified into three groups depending on the morphological characteristics and complications. Treatment of MVO should be based on these characteristics. J Cardiol 2007 Jul; 501: 2938 Key Words Cardiomyopathies, hypertrophicmidventricular obstructive Echocardiography, transthoracic Ventricular tachycardia Complicationscardioembolic stroke Atrial fibrillation midventricular obstructive hypertrophic cardiomyopathy : MVO 1% 1 MVO : ; Department of Cardiology, Obihiro National Hospital, Hokkaido ; Division of Cardiology, Nephrology, Pulmonology and Neurology, Asahikawa Medical College, Hokkaido Address for correspondence : AOKI M, MD, Department of Cardiology, Obihiro National Hospital, Nishi 18, Kita 2, Obihiro, Hokkaido ; mayu11@rose.ocn.ne.jp Manuscript received September 21, 2006 ; revised April 18, 2007 ; accepted April 23,

2 30 Fig. 1 Case 4. Color Doppler imaging showing diastolic paradoxical jet flow The jetopen arrowoccurred from the apex to the base during early diastolic phase. The peak value of the pressure gradient of the paradoxical jet at mid-ventricle was 44.4 mmhg. LV left ventricle ; LA left atrium ; Ao aorta. hour-glass appearance Fig MVO 4 MVO MVO 24 MVO 5 MVO MVO 4 MVO 1 MVO 1MVO 5 A1; Fig. 2 B 24; Fig. 3 C5; Fig. 43 ALOKA ProSound SSD SSD-2200 magnetic resonance imaging : MRI computed tomography : CT

3 31 Fig. 2 Case 1. Pulmonary artery angiograms in the return phase A : End-diastolic phase. B: End-systolic phase. Left ventricular hypertrophy and apical small cavityarrowswere observed in the end-systolic phase classified as Group A. Fig. 3 Case 4. Left ventriculograms A : End-diastolic phase. B: End-systolic phase. Obstruction in the mid-ventricle and apical aneurysmarrowswere observed, classified as Group B. Table 1 Table % 3 5 sustained ventricular tachycar-

4 32 Fig. 4 Case 5. Left ventriculograms A: End-diastolic phase. B : End-systolic phase. Hour-glass appearancearrowsand decreased left ventricular systolic function were observed. Table 1Patient characteristics Case No. Age yr Sex Chief complaints Observation periods yr Electrocardiogram Q wave ST elevation Complications Cardioembolic PAF SVT CHF stroke Therapy Warfarin AMD Ope 1 59 Male Palpitation 2 72 Male Chest oppression 3 60 Male Dizziness 4 70 Male Palpitation Dizziness 5 67 Male Palpitation PAFparoxysmal atrial fibrillation; SVTsustained ventricular tachycardia; CHFcongestive heart failure; AMDamiodarone; Opeapical aneurysmectomy and cryoablation. dia : SVT 4 MVO SVT Tl mmhg 1 SVT wide QRS Fig. 5 5

5 33 Table 2Morphological characteristics by echocardiography and left ventriculography Case No. Diastolic paradoxical jet flow Pressure gradient at mid-ventricle (mmhg) Apical aneurysm Decreased left ventricular systolic function (A) (B) Fig. 5 Case 3. Twelve-lead electrocardiogram during sustained ventricular tachycardia Sustained ventricular tachycardia with right bundle branch block and superior axis were observed with heart rate of 150 beats/min. 3 6 Q STFig SVT3 5SVT 3 6 SVT 4 SVTFig. 7 SVT MVO 1% MVO 6 MVO 5

6 34 (A) (B) Fig. 6 Case 5. Twelve-lead electrocardiogram A : Abnormal Q wave was present in the and 3 6 leads. B : ST elevation was found in the and 4 6 leads after 6 years. Fig. 7 Case 4. Cardiac magnetic resonance images A : End-diastolic phase. B : End-systolic phase. Remarkable myocardial hypertrophy and thin wall apical aneurysm were observed. 4 MVO MVO 3 Fig. 8 MVO MVO

7 35 Fig. 8 Relationship between left ventricular shape and complications Cardiogenic embolization and PAF/AF occurred in every group. Sustained ventricular tachycardia was recognized in Groups B and C, and congestive heart failure may occur in Group C. HOCM hypertrophic obstructive cardiomyopathy ; HCM hypertrophic cardiomyopathy ; MVO obstructive hypertrophic cardiomyopathy ; AF atrial fibrillation. Other abbreviations as in Table 1. MRI 64 MDCT Tl 1 5,7,9 4 MRI MVOQ R ST 2,5,10 R Q ST 5 Q ST SVT SVT SVTmacroreentry 9,15 B C SVT SVT 14B SVTMVO 16 SVT SVT 1721 SVT MVO 9,23 B C A MVO A 5

8 36 C a DDD a DDD MVO 24 MVO 5 MVO Fig. 8 A B C MVO A 2729 B C 1 3 MVO MVO 5 MVO4 SVT3 MVO 3 SVT B C SVT MVO : MVO MVO 3 : MVO 4 MVO 1 5 A 1 B 3 C1 :53 SVT1 1 MVO12 SVT BC 3 1

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