当院における経皮的心肺補助装置の導入状況と予後について： 症例集積研究

京都府立医科大学附属北部医療センター循環器内科京都府立医科大学附属北部医療センター臨床工学科Department of Cardiovascular Medicine, North Medical Center, Kyoto Prefectural University of MedicineDepartment of Clinical Engineering, North Medical Center, Kyoto Prefectural University of Medicine経皮的心肺補助は、重症心不全（急性心筋梗塞、心筋症、劇症型心筋炎など）、開心術後の低拍出症候群、大血管手術（胸部下行大動脈瘤、胸腹部大動脈瘤）による補助循環や重症呼吸不全などの病態に用いられているが、近年救急医療の現場、とくに心肺停止患者への適用が急増している。しかしながら、装置が高価であることや導入にマンパワーを要する治療法であることから、導入をためらっている施設も少なくない。また、適用基準や使用方法についても各施設によってさまざまである。経皮的心肺補助装置は2008 年に当院に導入され、2018 年3 月までの約10 年間で16 例に用いられた。年齢の中央値は65.5 歳で、男性が12 例だった。原因疾患として急性冠症候群/ 急性心筋梗塞が11 例、劇症型を含む心筋炎が4 例だった。そのうち2 例は現在も当院外来に通院中であり、長期生存率は12.5% であった。高齢化率の高い丹後医療圏におけるPCPS の適用基準にまつわる問題点を挙げ、使用の心得を記述し、京都府下の病院におけるPCPS 導入状況を俯瞰する

仮性動脈瘤による静脈圧排が原因と考えられた下肢浮腫の1例

京都府立医科大学附属北部医療センター循環器内科Department of Cardiovascular Medicine, Kyoto Prefectural University of Medicine North Medical Center症例は７３歳女性。安静時胸痛を認め、不安定狭心症を疑い、冠動脈造影検査にて３枝病変を認めた。カテーテル治療を選択し、左大腿動脈より６Fr シースを挿入し、経皮的冠動脈ステント留置術を施行した。止血デバイスを用い止血を行い、穿刺部に問題なく術翌日に退院した。退院7 日後より左下肢の腫脹を認め、血管エコー検査にて穿刺部に仮性動脈瘤を認めた。カテーテル後の下肢浮腫の原因として仮性動脈瘤に留意すべきと考える

PTRF/Cavin-1 Deficiency Causes Cardiac Dysfunction Accompanied by Cardiomyocyte Hypertrophy and Cardiac Fibrosis

<div><p>Mutations in the <i>PTRF/Cavin-1</i> gene cause congenital generalized lipodystrophy type 4 (CGL4) associated with myopathy. Additionally, long-QT syndrome and fatal cardiac arrhythmia are observed in patients with CGL4 who have homozygous <i>PTRF</i>/<i>Cavin-1</i> mutations. PTRF/Cavin-1 deficiency shows reductions of caveolae and caveolin-3 (Cav3) protein expression in skeletal muscle, and Cav3 deficiency in the heart causes cardiac hypertrophy with loss of caveolae. However, it remains unknown how loss of PTRF/Cavin-1 affects cardiac morphology and function. Here, we present a characterization of the hearts of <i>PTRF</i>/<i>Cavin-1</i>-null (<i>PTRF</i>^−/−) mice. Electron microscopy revealed the reduction of caveolae in cardiomyocytes of <i>PTRF</i>^−/− mice. <i>PTRF</i>^−/− mice at 16 weeks of age developed a progressive cardiomyopathic phenotype with wall thickening of left ventricles and reduced fractional shortening evaluated by echocardiography. Electrocardiography revealed that <i>PTRF</i>^−/− mice at 24 weeks of age had low voltages and wide QRS complexes in limb leads. Histological analysis showed cardiomyocyte hypertrophy accompanied by progressive interstitial/perivascular fibrosis. Hypertrophy-related fetal gene expression was also induced in <i>PTRF</i>^−/− hearts. Western blotting analysis and quantitative RT-PCR revealed that Cav3 expression was suppressed in <i>PTRF</i>^−/− hearts compared with that in wild-type (WT) ones. ERK1/2 was activated in <i>PTRF</i>^−/− hearts compared with that in WT ones. These results suggest that loss of PTRF/Cavin-1 protein expression is sufficient to induce a molecular program leading to cardiomyocyte hypertrophy and cardiomyopathy, which is partly attributable to Cav3 reduction in the heart.</p></div

Echocardiographic analysis of WT and <i>PTRF</i>^−/− female mice at 16 weeks of age.

<p>Echocardiographic analysis of WT and <i>PTRF</i>^−/− female mice at 16 weeks of age.</p

Electrocardiogram of <i>PTRF</i>^−/− mice.

<p>(A) Left, representative ECG of WT and <i>PTRF</i>^−/− female mice at 24 weeks of age. Right, magnified waveforms of ECG in lead II. (B) ECG parameters in lead II of WT and <i>PTRF</i>^−/− female mice at 24 weeks of age. HR, heart rate; bpm, beats per minute. Values are expressed as means ± SEM. **<i>P</i> < 0.01 compared with WT mice.</p

Morphometric analysis of WT and <i>PTRF</i>^−/− female mice at 16 weeks of age.

<p>Morphometric analysis of WT and <i>PTRF</i>^−/− female mice at 16 weeks of age.</p

mRNA and protein expression in the heart of <i>PTRF</i>^−/− mice.

<p>(A) mRNA expression of caveolins and cavins in WT and <i>PTRF</i>^−/− female hearts at 16 weeks of age. (B) mRNA expression of cardiac hypertrophy-related fetal genes and fibrosis-related genes in WT and <i>PTRF</i>^−/− female hearts at 16 weeks of age. (C) Expression of caveola-associated proteins in WT and <i>PTRF</i>^−/− female hearts at 16 weeks of age. Left, representative immunoblotting of heart lysates from WT and <i>PTRF</i>^−/− mice. Right, bar graph showing protein expression of WT and <i>PTRF</i>^−/− hearts. (D) Phosphorylation levels of MAPKs and Akt in WT and <i>PTRF</i>^−/− female hearts at 16 weeks of age. Left, representative immunoblotting of heart lysates from WT and <i>PTRF</i>^−/− mice. Right, bar graph showing phosphorylation levels of MAPKs and Akt in WT and <i>PTRF</i>^−/− hearts. *<i>P</i> < 0.05 and **<i>P</i> < 0.01.</p

Variability of Jupiter’s Main Auroral Emission in Response to Magnetospheric Hot Plasma Injections

We present observations of Jupiter’s FUV aurora acquired by the Hubble Space Telescope during a two-week interval in January 2014. The variability of the main auroral emission was studied using latitudinal profiles of intensity. The main oval intensity was found to be reduced when bright patches of diffuse emission were present at lower latitudes. These low latitude emissions are interpreted as the signatures of hot plasma injections from the outer magnetosphere, a process which has previously been related to interchange between the flux tubes from the outer magnetosphere and outward-moving flux tubes loaded with iogenic plasma. The main emission was also observed to broaden and shift in latitude, and occasionally display a double peak structure. These observations are interpreted with reference to the expected changes in auroral field-aligned currents associated with the replacement of the radially-stretched, mass-loaded flux tubes in the middle magnetosphere by more dipolar flux tubes containing rarefied hot plasma

Morphological changes in the heart of <i>PTRF</i>^−/− mice.

<p>(A) Left, representative electron microscopic images of WT and <i>PTRF</i>^−/− hearts from 12-week-old female mice. Caveolae were identified by their characteristic flask shape and location at the plasma membrane. White arrowheads indicate caveolae. Right, quantification of caveolae per μm of plasma membrane in atrial and ventricular cardiomyocytes of WT and <i>PTRF</i>^−/− hearts. Multiple electron micrographs were obtained for each heart, and both the number of caveolae and the total length of the plasma membrane were quantified in each image. Caveolae were counted as omega-shaped membrane profiles open at the cell surface. (B) Myocyte cross-sectional area of WT and <i>PTRF</i>^−/− hearts. Left, representative H&E staining sections of hearts from WT and <i>PTRF</i>^−/− female mice at 16 weeks of age. Right, bar graph showing cross-sectional area of cardiomyocytes of WT and <i>PTRF</i>^−/− hearts. (C) Fibrotic area of WT and <i>PTRF</i>^−/− female hearts. Left, representative Masson’s trichrome staining sections of hearts from WT and <i>PTRF</i>^−/− female mice at 16 weeks of age. Right, bar graph showing fibrotic area in WT and <i>PTRF</i>^−/− hearts. **<i>P</i> < 0.01.</p