Left ventricular outflow tract obstruction with abnormal papillary muscles

AbstractA 65-year-old man with a history of hypertension was admitted to our hospital with fainting and syncope. He had experienced recurrent syncope since 20 years of age. On admission, systolic heart murmur was audible at the apex of the heart. Echocardiography revealed anteriorly displaced papillary muscles (PMs), elongation of the anterior mitral valve leaflet (AML), and systolic anterior motion (SAM) of the AML. Color Doppler imaging showed accelerated flow with a pressure gradient (PG) of 56mmHg at the left ventricular outflow tract (LVOT). Cardiac magnetic resonance imaging revealed mild asymmetric septal hypertrophy and multiple accessory PMs. Cine images clearly demonstrated SAM and LVOT obstruction due to anteriorly displaced PMs. Based on these findings, the patient was diagnosed as having hypertrophic cardiomyopathy and LVOT obstruction due to abnormal PMs. Oral administration of bisoprolol (2.5mg/day) was initiated, because the patient rejected surgical treatment. Follow-up echocardiography revealed a gradual decrease in the LVOT-PG to 24mmHg, and no episodes of fainting or syncope have recurred for 2 years after the initiation of bisoprolol.<Learning objective: Abnormal papillary muscle (PM) is an unusual cause of left ventricular outflow tract (LVOT) obstruction, and cardiac magnetic resonance (CMR) imaging has been reported to be useful for diagnosis of abnormal PM. Abnormal PMs with LVOT obstruction are usually treated by surgical correction, and therefore, reports on medical treatment are limited. We report a case of LVOT obstruction due to abnormal PMs, which was accurately diagnosed by CMR imaging and successfully treated with a beta-blocker.

REST遺伝子変異(E116K)導入マウスを用いた心筋症発症機序の解明

金沢大学医学部附属病院研究課題/領域番号:13555218, 研究期間(年度):2001-2005出典：研究課題「REST遺伝子変異(E116K)導入マウスを用いた心筋症発症機序の解明」課題番号19790521（KAKEN：科学研究費助成事業データベース（国立情報学研究所）） （https://kaken.nii.ac.jp/ja/grant/KAKENHI-PROJECT-19790521/）を加工して作

高齢肥大型心筋症患者の左室収縮不全と左室拡張に関係する心筋ミオシン結合蛋白C遺伝子変異

取得学位 : 博士（医学）, 学位授与番号 : 医博甲第1595号, 学位授与年月日 : 平成15年6月30日, 学位授与大学 : 金沢大

Takotsubo cardiomyopathy with marked ST-segment elevation and electrical alternans complicated with hyperglycemic hyperosmolar state

金沢大学医薬保健研究域医学系This is the first report of a case of Takotsubo cardiomyopathy with a hyperglycemic hyperosmolar state (HHS). This case presented with marked ST-segment elevation and electrical alternans, uncommon findings in Takotsubo cardiomyopathy. We believe that hyperosmolarity-induced myocardial dehydration and consequent increase in intracellular calcium concentration may be the mechanism of Takotsubo cardiomyopathy and electrical alternans in HH

Polymorphic ventricular tachycardia in a patient with hypertrophic cardiomyopathy and digitalis intoxication

SummaryWe report the case of a 74-year-old woman who presented with recurrent episodes of polymorphic ventricular tachycardia (PVT) with a normal QT interval due to digitalis intoxication (serum digoxin concentration, 5.0 ng/mL) and severe hyperkalemia (serum potassium level, 8.3 mEq/L). In addition, laboratory data showed elevated levels of blood urea nitrogen (54 mg/dL) and serum creatinine (1.57 mg/dL), suggesting dehydration. She had been treated with a combination of digoxin and eplerenone for atrial fibrillation and heart failure. The PVT resolved after treatment for hyperkalemia. Cardiac magnetic resonance imaging and left ventriculography showed left ventricular hypertrophy predominantly in the apex, suggesting apical hypertrophic cardiomyopathy (HCM). We presume that the presence of HCM was related to the occurrence of PVT in this patient with digitalis intoxication and hyperkalemia.<Learning objective: PVT with a normal QT interval caused by digitalis intoxication with hyperkalemia was observed in a patient with HCM treated with digoxin and eplerenone for atrial fibrillation and heart failure. The presence of HCM may be related to the occurrence of PVT. Combination therapy with digoxin and aldosterone receptor antagonist may predispose severe hyperkalemia, and monitoring of serum digitalis concentration and potassium level should be done strictly.

Crow-Fukase syndrome associated with high-output heart failure

金沢大学医薬保健研究域医学系A 64-year-old woman was admitted with systemic edema and exertional dyspnea. High-output heart failure was diagnosed by right heart catheterization and she was treated with diuretics. After 3 weeks, her symptoms disappeared but a high cardiac output state persisted. A diagnosis of Crow-Fukase syndrome was made based on the presence of polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes. Her serum vascular endothelial growth factor (VEGF) level was markedly elevated after recovery from heart failure. We suspect that an elevated VEGF level and a high cardiac output state may play a role in the pathogenesis of heart failure in Crow-Fukase syndrome

Correlation functions of the integrable higher-spin XXX and XXZ spin chains through the fusion method

For the integrable higher-spin XXX and XXZ spin chains we present multiple-integral representations for the correlation function of an arbitrary product of Hermitian elementary matrices in the massless ground state. We give a formula expressing it by a single term of multiple integrals. In particular, we explicitly derive the emptiness formation probability (EFP). We assume $2s$-strings for the ground-state solution of the Bethe ansatz equations for the spin-$s$ XXZ chain, and solve the integral equations for the spin-$s$ Gaudin matrix. In terms of the XXZ coupling $\Delta$ we define $\zeta$ by $\Delta=\cos \zeta$, and put it in a region $0 \le \zeta < \pi/2s$ of the gapless regime: $-1 < \Delta \le 1$ ($0 \le \zeta < \pi$), where $\Delta=1$ ($\zeta=0$) corresponds to the antiferromagnetic point. We calculate the zero-temperature correlation functions by the algebraic Bethe ansatz, introducing the Hermitian elementary matrices in the massless regime, and taking advantage of the fusion construction of the $R$-matrix of the higher-spin representations of the affine quantum group.Comment: 50 pages, no figur