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A 13-year-old girl was referred for closer examination of electrocardiographic abnormalities. She had a Levine 2/6 systolic murmur (SM) and a fourth heart sound. Electrocardiography findings showed poor R progression from V1 to V4 and negative T waves in the II, III, and aVF leads. Approximately 3.2 years later, her SM increased to Levine 3–4/6. Echocardiography indicated mitral regurgitation and left ventricular pressure gradient (LVPG) of 18.5 mmHg, together with a notch on the systolic wave in the apexcardiogram. We concluded that the electrocardiographic abnormalities were caused by hypertrophic cardiomyopathy. Approximately 6 months later, her SM further increased to Levine 4–5/6, and the voltage on the electrocardiogram increased. In carotid pulse tracing, steep upstroke and deflated percussion wave due to mitral regurgitation were noted. The LVPG was approximately 102 mmHg, and systolic anterior movement was confirmed. After the oral administration of 200 mg of cibenzoline, the LVPG decreased to approximately 48 mmHg. Subsequently, 300 mg/day of cibenzoline was administered. The LVPG further decreased, and her symptoms improved. Clinical symptoms and left ventricular pressure gradient decrease can be ameliorated by cibenzoline therapy in patients with hypertrophic obstructive cardiomyopathy. This finding applies not only to adult patients but also to teen-aged patients. [ABSTRACT FROM AUTHOR]

An 84-year-old woman was urgently referred to our hospital owing to persistent malaise and chest pain. Takotsubo cardiomyopathy was diagnosed following transthoracic echocardiography, emergency coronary angiography, and left ventriculography. A left ventricular pressure tracing during cardiac catheterization revealed a 72 mmHg-intraventricular pressure gradient (LVPG). Although β-blockers are effective at reducing LVPG in takotsubo cardiomyopathy, such treatment was contraindicated in our patient owing to her history of bronchial asthma. Therefore, we administered intravenous cibenzoline succinate to attenuate her LVPG. The LVPG decreased to 18 mmHg within 10 min after intravenous cibenzoline succinate administration. During her subsequent hospitalization, the patient showed excellent functional recovery, without any complications. Left ventriculography, performed 21 days after admission, showed normal wall motion and a left ventricular ejection fraction of 68%. < Learning objective: The prognosis of takotsubo cardiomyopathy is generally good, however, fatal complications have been reported. Our case demonstrated a LVPG of 72 mmHg, and β-blockers were contraindicated because of bronchial asthma. Intravenous cibenzoline administration resulted in successful attenuation of the patient's LVPG. The present case suggests that cibenzoline might be effective for the treatment of takotsubo cardiomyopathy, especially in those contraindicated for β-blocker therapy.> [ABSTRACT FROM AUTHOR]

Summary: A 54-year-old woman visited our hospital because of palpitation and dizziness. Systolic ejection murmur, Levine IV/VI, was heard at the left sternal border and atrial gallop was auscultated at apex. Echocardiogram demonstrated diffuse hypertrophy of the left ventricular walls extending to the apex. The left ventricular walls increased symmetrically; the septal and the posterior wall measured approximately 26mm and 30mm in thickness, respectively. Apical two-chamber views revealed a narrow outflow of the left ventricular cavity at apex with a distinct convergence zone, where color flow Doppler imaging showed turbulent jet flow from mid to late systole. The leukocyte α-galactosidase A activity was lower than normal. Percutaneous endomyocardial biopsy showed specific change for Fabry''s disease by electron microscopy. On cardiac catheterization, mid-ventricular resting pressure gradient of approximately 90mmHg was shown. This pressure gradient dramatically disappeared after intravenous administration of 70mg of cibenzoline. We describe a rare case of Fabry''s disease that revealed diffuse hypertrophy having dynamic obstruction at mid-left ventricle, like mid-ventricular obstruction, and the administration of cibenzoline had beneficial effects on pressure gradient of mid-left ventricle. [Copyright &y& Elsevier]