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1. 特発性血球減少症（Idiopathic cytopenias of undetermined significance［ICUS］）、clonal hematopoiesis of indeterminate potential (CHIP)、clonal cytopenias of undetermined significance (CCUS)idiopathic cytopenias of undetermined significance icus clonal hematopoiesis of indeterminate potential chip and clonal cytopenias of undetermined significance ccus [show details]
… SRSF2, MLL2, SETD2, SETDB1, GNAS, PPM1D, and BCORL1 . CHIP has also been called age-related clonal hematopoiesis (ARCH) because of the higher prevalence in older persons, although it can be seen at any …
2. 胸部大動脈瘤・胸部大動脈解離への開胸手術の概要overview of open surgical repair of the thoracic aorta [show details]
… initially, then curves to form the aortic arch, and descends caudally adjacent to the spine. The ascending thoracic aorta gives off the coronary arteries, and the aortic arch branches are typically the brachiocephalic …
3. 胸部大動脈瘤の臨床症状および診断clinical manifestations and diagnosis of thoracic aortic aneurysm [show details]
… associated with thoracic aortic aneurysm include bicuspid aortic valve, intracranial aneurysm, inguinal hernia, aortic arch branching anomalies (eg, bovine aortic arch,… silhouette ; Enlargement of the aortic knob; Displacement of the trachea from midline ; Other features include displaced aortic calcification, aortic kinking,…
4. 急性大動脈解離の臨床的特徴および診断clinical features and diagnosis of acute aortic dissection [show details]
… dissected aortic wall may not be seen with noninvasive imaging. In one study of 181 patients who underwent repair of the ascending aorta or aortic arch, nine (5 percent) had a subtle aortic dissection …
5. 心血管疾患の危険因子の概要overview of possible risk factors for cardiovascular disease [show details]
…of the aortic arch is associated with a greater risk of CVD and total mortality. In one study of 139,849 members of a health maintenance organization followed for a median of 28 years, aortic arch calcification …

English Journal

Pulmonary arteriopexy to prevent pulmonary artery kinking in orthotopic heart transplantation.

Toscano G, Guariento A, Spigolon L, Blitzer D, Gallo M, Gerosa G.
Journal of cardiac surgery. 2019 May;().
Heart transplantation is still the best treatment for patients with end-stage heart failure unresponsive to medical therapy or those treated with mechanical circulatory support. The surgical technique for heart transplantation is fraught with potential complications. One of these potential complicat
PMID 31111545

Time-resolved 3-dimensional magnetic resonance phase contrast imaging (4D Flow MRI) reveals altered blood flow patterns in the ascending aorta of patients with valve-sparing aortic root replacement.

Oechtering TH, Sieren MM, Hunold P, Hennemuth A, Huellebrand M, Scharfschwerdt M, Richardt D, Sievers HH, Barkhausen J, Frydrychowicz A.
The Journal of thoracic and cardiovascular surgery. 2019 Apr;().
The aim of this study was to compare aortic flow patterns in patients after David valve-sparing aortic root replacement with physiologically shaped sinus prostheses or conventional tube grafts in healthy volunteers. Twelve patients with sinus prostheses (55 ± 15 years), 6 patients with tube graf
PMID 31078313

Single-center experience with an inner branched arch endograft.

Tsilimparis N, Detter C, Law Y, Rohlffs F, Heidemann F, Brickwedel J, von Kodolitsch Y, Debus ES, Kölbel T.
Journal of vascular surgery. 2019 Apr;69(4)977-985.e1.
Whereas open repair is the "gold standard" for most aortic arch diseases, a subgroup of patients might benefit from an endovascular approach. The introduction of branched stent grafts with dedicated design to address the challenges of the ascending aorta and the aortic arch has opened an entirely ne
PMID 30477941

Japanese Journal

Long-segment Coarctation に対して大動脈再建術を施行した1例

中尾充貴,黄義浩,木ノ内勝士,阿部貴行,野村耕司
日本心臓血管外科学会雑誌 46(2), 66-69, 2017
<p>縮窄距離の長い大動脈縮窄症に対する血行再建では狭窄の残存，上行大動脈の屈曲，気道狭窄が起きうる．また，吻合が届かない場合は人工血管を介在させる再建法を行う必要があるが，将来再手術が不可避である．われわれはend-to-end anastomosisに鎖骨下動脈フラップ法を組み合わせることにより自己組織のみによる縮窄解除を施行し，良好な術後経過をたどった症例を経験したため報告する． …
NAID 130005548869

大動脈弁狭窄症および偽性大動脈縮窄症に合併した胸部大動脈瘤に対して clamshell 切開により同時手術を施行した1例

渋川貴規,田内祐也,奥田直樹,山田光倫,佐藤尚司,松田暉
日本心臓血管外科学会雑誌 43(6), 336-339, 2014
症例は胸痛を主訴とする64歳男性．心エコーで大動脈弁狭窄症と診断され，外科治療目的で紹介された．術前CTで大動脈弓部の低形成，遠位弓部での狭窄，狭窄後の下行大動脈の瘤形成（52 mm）と蛇行を認めた．大動脈弁狭窄を合併する偽性大動脈縮窄＋胸部大動脈瘤と診断し，clamshell切開での一期的手術を行った．大動脈弁は2尖弁で機械弁による置換を行い，大動脈については上行大動脈も将来の瘤化も考え上行から …
NAID 130004706645

偽性大動脈縮窄症に伴う若年者胸部大動脈瘤

有木弘,水野俊一,恒川智宏,土井智章
日本血管外科学会雑誌 15(1), 35-38, 2006
症例は, 13歳男性で, 健診にて胸部X線で縦隔の異常陰影を指摘され精査目的で当院を紹介された. MRアンギオにて峡部での大動脈の異常な屈曲と蛇行, およびこれに続く嚢状の大動脈瘤が認められた. 上下肢の間で血圧の較差はほとんどなく, rib notchingなどの側副血行路の発達を示す所見もなく, 以上より偽性大動脈縮窄症に合併した胸部下行大動脈瘤と診断した. 低体温, 循環停止を併用した体外循 …
NAID 130004490139

「先天性大動脈弓部蛇行症」

　　[★]

英: kinking of aortic arch

参考

Congenital Kinking of the Aorta (Pseudocoarctation)

John F. Griffin, M.D.
The New England journal of medicine.1964 Oct 1;271:726-8.
KINKING of the descending arch of the aorta is a condition that may cause serious difficulty in the interpretation of the routine posteroanterior chest film and lead to unnecessary exploratory thoracotomy when not recognized. This congenital anomaly may also present the clinician with a sufficient number of physical findings to suggest the diagnosis of coarctation of the aorta. Although it is considered to be a rare malformation, the frequency with which it has been reported during the past decade warrants wider appreciation of its occurrence. Once the diagnosis is suspected it becomes relatively simple to establish its presence and clarify . . . I am indebted to Dr. Oscar Starobin, of the Worcester Memorial Hospital, for performing the angiography.
PMID 14173676