Attenuation of Biventricular Pressure Gradients by Cibenzo-line in an 18-Year-Old Patient With Hypertrophic Obstructive Cardiomyopathy.

Hiasa, Go; Hamada, Mareomi; Shigematsu, Yuji; Hara, Yuji; Ohtsuka, Tomoaki; Ogimoto, Akiyoshi; Saya, Hideyuki; Suzuki, Jun; Matsunaka, Tsuyoshi; Hamada, Hironobu; Okura, Takafumi; Hiwada, Kunio

doi:10.1253/circj.66.1173

Cited by 13 publications

(10 citation statements)

References 17 publications

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“…First, we did not assess the effects of class Ia antiarrhythmic drugs such as cibenzoline. Hamada et al reported that oral administration of cibenzoline markedly attenuates the LV pressure gradient in patients with HOCM (17,18). It is noteworthy that cibenzoline also improved LV diastolic function in these patients (19).…”

Section: Discussionmentioning

confidence: 99%

Improved Hypertrophic Obstructive Cardiomyopathy by Left Ventricular Apex Epicardial Pacing

et al. 2004

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We report a case of severe hypertrophic obstructive cardiomyopahy (HOCM) that was markedly improved by left ventricular (LV) apex epicardial pacing. A 55-year-old woman with HOCM had suffered from dyspnea. Cardiac catheter examination showed a resting pressure gradient across the LV outflow tract of 198 mmHg despite combined medication. During the examination, right dual-chamber pacing could not sufficiently reduce the pressure gradient. Therefore, we treated the patient with LV apex epicardial pacing. The procedure decreased the pressure gradient to 10 mmHg. Clinical symptoms were markedly improved. LV apex epicardial pacing may be an alternative therapy for patients with HOCM who are refractory to other medical treatment. IntroductionPatients with hypertrophic obstructive cardiomyopathy (HOCM) may present with progressive incapacitating symptoms, including dyspnea, angina, and syncope. These patients have a characteristic left ventricular outflow tract (LVOT) obstruction that divides the LV into a high-pressure apical region and a lower pressure subaortic region. Medical treatment (1, 2) or dual-chamber pacing (3, 4) can reduce LVOT obstruction and diminish symptoms. Patients who do not respond positively to these therapies require percutaneous transluminal septal myocardial ablation (PTSMA) (5, 6) or surgical resection of the affected portion of the interventricular septum (7,8). However, PTSMA is an irreversible therapy and reportedly causes complete atrioventricular (AV) block in about 50% of patients, some of them requiring a permanent pacemaker (9). Surgical resection requires extracorporeal circulation and is associated with a moderate surgical risk of 5% or greater (7). Recently, the effectiveness of LV epicardial pacing was proposed (10). Matsumoto demonstrated that LV postero-lateral epicardial pacing through the coronary sinus branch effectively reduced the pressure gradient more than right AV sequential pacing in a patient with HOCM (10). The author suggested that alterations of LV wall motion in association with an elimination of systolic anterior movement (SAM) and mitral regurgitation, caused by an inversion of the LV activation sequence, might result in the improvement of hemodynamics and the amelioration of obstruction (10). We report a case of severe HOCM that did not react to right dual-chamber sequential pacing with medication, but exhibited marked improvement by LV apex pacing with an epicardial lead. Case ReportA 55-year-old woman with HOCM had suffered from dyspnea and palpitation on effort for 2 years (NYHA class III). Because her symptoms were not improved by medical treatment, she was admitted to our hospital. On admission, her blood pressure was 96/54 mmHg and heart rate was 42 beats/min. A grade 4/6 ejection murmur at the left sternal border and 2/6 holosystolic murmur at the apex were audible. A 12-lead electrocardiogram revealed LV hypertrophy with a strain pattern. Echocardiographic findings showed asymmetric septal hypertrophy, narrowing of the midzone in LV (mid-LV), SA...

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Section: Discussionmentioning

confidence: 99%

Improved Hypertrophic Obstructive Cardiomyopathy by Left Ventricular Apex Epicardial Pacing

et al. 2004

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“…On the other hand cibenzoline, which is also a class Ia antiarrhythmic agent, but with less anticholinergic effects than disopyramide, can have beneficial effects on the LVPG and diastolic function in HOCM, including cases of midventricular obstruction. 3,[11][12][13] Cibenzoline is a strong Na + channel-blocking agent, which provokes a decrease the in intracellular Ca 2+ concentration in cardiac myocytes, 14 and it also has a Ca 2+ channel blocking action. A previous report has indicated that hypercontraction and narrowing of the left ventricle play an important role in the enhancement of LVPG with sigmoid septum.…”

Section: Circulation Journal Vol68 October 2004mentioning

confidence: 99%

Beneficial Effect of Cibenzoline on Left Ventricular Pressure Gradient With Sigmoid Septum

Konishi¹,

Shiraishi²,

Muraguchi³

et al. 2004

Circ J

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sigmoid-shaped ventricular septum is one of the causes of left ventricular outflow tract (LVOT) obstruction in older patients and because it is rarely related to clinical symptoms, 1,2 medical therapy for the reduction of the left ventricular pressure gradient (LVPG) associated with sigmoid septum has not been established. It was recently reported that the class Ia antiarrhythmic drug, cibenzoline, attenuates the LVPG in patients with hypertrophic obstructive cardiomyopathy (HOCM), 3 and we describe a patient with LVPG caused by a sigmoid-shaped septum who improved after administration of cibenzoline. Case ReportA 83-year-old-female was admitted to hospital with episodes of dyspnea on effort after having breakfast. She had had hypertension for 20 years prior to admission. Her blood pressure was 154/74 mmHg, and her pulse rate was 100 beats/min and regular. Her physical examination was unremarkable, except for a grade 4/6 systolic murmur at the left sternal border in the third to fourth intercostal space. Routine blood tests were normal except for brain natriuretic peptide (BNP) of 455 pg/ml. An electrocardiography recorded 18 years prior to admission had been normal, whereas that recorded in the current admission showed normal sinus rhythm at a rate of 75 beats/min and left ventricular hypertrophy with ST-segment depression and inverted T waves characteristic of the strain pattern (Fig 1). Chest X-ray showed cardiomegaly with a cardiothoracic ratio of 56%. Two-dimensional transthoracic echocardiography revealed a sigmoid-shaped base of the interventricular septum markedly protruding into the left ventricle and calcification of both posterior mitral leaflet and aortic valve (Fig 2A,B), whereas that performed 18 years prior to admission had been normal (Fig 3). The remainder of the septum and the left ventricular free wall were concentrically hypertrophied, but no enlargement of the left ventricular cavity was observed. The angle between the mid line axis of the ascending aorta and that of the interventricular septum was 97.5°(normal range, 145±7°). M-mode echocardiography demonstrated a systolic anterior motion of the mitral valve (SAM) with fractional shortening (FS) of 39% ( Fig 4A). Color Doppler echocardiography showed turbulent systolic flow through the LVOT with a pressure gradient of 121.8 mmHg, mild aortic stenosis, and moderate mitral regurgitation (Figs 2C,4B). Cardiac catheterization was performed and angiography indicated an intermediate stenosis in the mid portion of the right coronary artery. The left ventricular end-diastolic pressure was 18 mmHg and the peak-to peak LVPG at the LVOT was 146 mmHg. These findings confirmed the diagnosis of LVOT obstruction caused by the sigmoid septum, concentric left ventricular hypertrophy and SAM.To reduce the LVOT obstruction, 60 mg/day of metoprolol was administered, but despite that treatment the LVPG remained at approximately 100 mmHg. Hence, 200 mg/day of cibenzoline, a class Ia antiarrhythmic agent, was administered in addition to 40 mg/day of metoprolol. A...

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“…Occasionally, predominant right ventricular disease can be seen. [1][2][3][4][5][6][7] A 52-year-old male with a history of alcohol and psychotropic drug abuse was referred to our department with New York Heart Association class III symptoms of heart failure after a resuscitated cardiac arrest. An ECG showed left atrial abnormality, left axis deviation, and left ventricular strain pattern ( Figure 1 The patient received beta-adrenolytic medical therapy and remains in New York Heart Association class II/III.…”

mentioning

confidence: 99%

Predominant, Severe Right Ventricular Outflow Tract Obstruction in Hypertrophic Cardiomyopathy

Kręcki

Lipiec²,

Piotrowska-Kownacka³

et al. 2007

Circulation

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Attenuation of Biventricular Pressure Gradients by Cibenzo-line in an 18-Year-Old Patient With Hypertrophic Obstructive Cardiomyopathy.

Cited by 13 publications

References 17 publications

Improved Hypertrophic Obstructive Cardiomyopathy by Left Ventricular Apex Epicardial Pacing

Improved Hypertrophic Obstructive Cardiomyopathy by Left Ventricular Apex Epicardial Pacing

Beneficial Effect of Cibenzoline on Left Ventricular Pressure Gradient With Sigmoid Septum

Predominant, Severe Right Ventricular Outflow Tract Obstruction in Hypertrophic Cardiomyopathy

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