今日の臨床サポート

急性大動脈解離

著者: 圷 宏一 日本医科大学 循環器内科

監修: 今井靖 自治医科大学 薬理学講座臨床薬理学部門・内科学講座循環器内科学部門

著者校正/監修レビュー済:2021/12/01
参考ガイドライン:
患者向け説明資料

概要・推奨   

  1. 大動脈解離急性期には、β遮断薬を使用することが推奨される(推奨度1)。
  1. ACE阻害薬が臨床的に奏効する可能性は十分である(推奨度2)。
  1. ARBに関してはMarfan症候群の大動脈径拡大予防効果に関しては大規模研究も行われているが一定の結論が得られていない(推奨度2)。大動脈解離そのものへの有用性を示した報告はない(推奨度2)。
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  1. 閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要とな
  1. 閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必
  1. 閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要と
薬剤監修について:
オーダー内の薬剤用量は日本医科大学付属病院 薬剤部 部長 伊勢雄也 以下、林太祐、渡邉裕次、井ノ口岳洋、梅田将光による疑義照会のプロセスを実施、疑義照会の対象については著者の方による再確認を実施しております。
※薬剤中分類、用法、同効薬、診療報酬は、エルゼビアが独自に作成した薬剤情報であり、
著者により作成された情報ではありません。
尚、用法は添付文書より、同効薬は、薬剤師監修のもとで作成しております。
※薬剤情報の(適外/適内/⽤量内/⽤量外/㊜)等の表記は、エルゼビアジャパン編集部によって記載日時にレセプトチェックソフトなどで確認し作成しております。ただし、これらの記載は、実際の保険適用の査定において保険適用及び保険適用外と判断されることを保証するものではありません。また、検査薬、輸液、血液製剤、全身麻酔薬、抗癌剤等の薬剤は保険適用の記載の一部を割愛させていただいています。
(詳細はこちらを参照)
著者のCOI(Conflicts of Interest)開示:
圷 宏一 : 未申告[2021年]
監修:今井靖 : 講演料(第一三共株式会社)[2021年]

改訂のポイント:
  1. 最新のガイドラインの追加・変更点に従って改定した。
  1. 論文等により診療方針が変わってきている点について改定した。
  1. 前回作成の文章の補足・修正を行った。

病態・疫学・診察

疾患情報(疫学・病態)  
急性大動脈解離は死亡率の高い循環器救急疾患であり、胸痛患者の最も重要な鑑別疾患である。本疾患において特異度の高い生化学マーカーは存在せず、確定診断はCTによってなされる。症状が多彩であるため、誤診率も高く、また見落としが患者の生死につながる疾患であるため、胸痛患者を帰宅させる際には本疾患の除外が必須である。
 
  1. 大動脈解離とは、“血液が大動脈内膜の破綻部分から外膜方向に向かって入り込み、中膜のおよそ外1/3のレベルで大動脈を長軸方向に引き裂きながら進行した結果、大動脈が2腔になった状態”を指す。
 
大動脈解離の発症、進行と偽腔の形成

エントリーから流入した血流は偽腔を形成しながら末梢側へ進行していく。ときとして中枢側へ進行することもある。

出典

 
大動脈解離の病態

エントリーから流入した血液は中膜のレベルで大動脈を割きながら進行し、結果として偽腔を形成する。よってflap(中隔)の構成成分は内膜と中膜の一部である。

出典

 
  1. 本疾患の病態における問題点は、①破裂 ②分枝血流障害である。
  1. 破裂は、急性期において胸腔内または腹腔内へは少なく、上行大動脈を覆っている心膜腔への破裂、すなわち心タンポナーデが多い。心タンポナーデは血行動態の急速な破綻を来し、急性期の死因の第1位である[1]
 
急性大動脈解離の死因

出典

img1:  著者提供
 
 
 
  1. 分枝血流障害に関しては、①偽腔による近位の真腔への圧排が遠位の血流を低下させること、②分岐部における大動脈の真腔が狭小化していること、③あるいは解離が分枝に直接及ぶこと――などによって生じる。心筋虚血、脳虚血、上肢虚血、対麻痺、腸管虚血、腎不全、下肢虚血などがあるが、特に心筋虚血[2]、脳虚血[3]、腸管虚血[4]は重要な予後不良因子である[4]
  1. 本疾患の急性期死亡率は15.2%(2017年東京都大動脈スーパーネットワーク)であり、急性心筋梗塞の死亡率6.4%程度をしのぐ[5]。また、急性期の診断率が43%との報告もあり[6]、胸(背部)痛を主とする患者に対し、必ず本疾患を鑑別の対象とすることが重要である。
問診・診察のポイント  
問診のポイント:
  1. 1.痛みに関しての詳細な質問:

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文献 

著者: Igor Zindovic, Tomas Gudbjartsson, Anders Ahlsson, Simon Fuglsang, Jarmo Gunn, Emma C Hansson, Vibeke Hjortdal, Kati Järvelä, Anders Jeppsson, Ari Mennander, Christian Olsson, Emily Pan, Johan Sjögren, Anders Wickbom, Arnar Geirsson, Shahab Nozohoor
雑誌名: J Thorac Cardiovasc Surg. 2019 Apr;157(4):1324-1333.e6. doi: 10.1016/j.jtcvs.2018.10.134. Epub 2018 Nov 16.
Abstract/Text OBJECTIVES: To evaluate the effect of preoperative malperfusion on 30-day and late mortality and postoperative complications using data from the Nordic Consortium for Acute Type A Aortic Dissection (ATAAD) registry.
METHODS: We studied 1159 patients who underwent ATAAD surgery between January 2005 and December 2014 at 8 Nordic centers. Multivariable logistic and Cox regression analyses were performed to identify independent predictors of 30-day and late mortality.
RESULTS: Preoperative malperfusion was identified in 381 of 1159 patients (33%) who underwent ATAAD surgery. Thirty-day mortality was 28.9% in patients with preoperative malperfusion and 12.1% in those without. Independent predictors of 30-day mortality included any malperfusion (odds ratio, 2.76; 95% confidence interval [CI], 1.94-3.93), cardiac malperfusion (odds ratio, 2.37; 95% CI, 1.34-4.17), renal malperfusion (odds ratio, 2.38; 95% CI, 1.23-4.61) and peripheral malperfusion (odds ratio, 1.95; 95% CI, 1.26-3.01). Any malperfusion (hazard ratio, 1.72; 95% CI, 1.21-2.43), cardiac malperfusion (hazard ratio, 1.89; 95% CI, 1.24-2.87) and gastrointestinal malperfusion (hazard ratio, 2.25; 95% CI, 1.18-4.26) were predictors of late mortality. Malperfusion was associated with significantly poorer survival at 1, 3, and 5 years (95.0% ± 0.9% vs 88.7% ± 1.9%, 90.1% ± 1.3% vs 84.0% ± 2.4%, and 85.4% ± 1.7% vs 80.8% ± 2.7%; log rank P = .009).
CONCLUSIONS: Malperfusion has a significant influence on early and late outcomes in ATAAD surgery. Management of preoperative malperfusion remains a major challenge in reducing mortality associated with surgical treatment of ATAAD.

Copyright © 2018 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.
PMID 30578066  J Thorac Cardiovasc Surg. 2019 Apr;157(4):1324-1333.e6.・・・
著者: Marco Di Eusanio, Himanshu J Patel, Christoph A Nienaber, Daniel M Montgomery, Amit Korach, Thoralf M Sundt, Carlo Devincentiis, Matthias Voehringer, Mark D Peterson, Truls Myrmel, Gianluca Folesani, Magnus Larsen, Nimesh D Desai, Joseph E Bavaria, Jehangir J Appoo, Teresa M Kieser, Rossella Fattori, Kim Eagle, Roberto Di Bartolomeo, Santi Trimarchi
雑誌名: J Thorac Cardiovasc Surg. 2013 Mar;145(3 Suppl):S213-21.e1. doi: 10.1016/j.jtcvs.2012.11.054.
Abstract/Text OBJECTIVES: The management strategy remains controversial for patients presenting with type A acute aortic dissection with cerebrovascular accident or coma. The present study aimed to help guide surgeons treating these high-risk patients.
METHODS: Of 1873 patients with type A acute aortic dissection enrolled in the International Registry for Acute Dissection, 87 (4.7%) presented with cerebrovascular accident and 54 (2.9%) with coma. The hospital and 5-year results were stratified by the presence and type of brain injury (no injury vs stroke vs coma) and management type (medical vs surgical). Independent predictors of short- and mid-term survival were identified.
RESULTS: Presentation with shock, hypotension, or tamponade (46.8% vs 25.2%; P < .001) and arch vessel involvement (55.0% vs 36.1%; P < .001) was more likely in patients with brain injury. Surgical management was avoided more often in patients with coma (33.3%) or cerebrovascular accident (24.1%) than in those without brain injury (11.1%; P < .001). The overall hospital mortality was 22.7% without brain injury, 40.2% with cerebrovascular accident, and 63.0% with coma (P < .001). Mortality varied among the management types for both cerebrovascular accident (76.2% medical vs 27.0% surgical; P < .001) and coma (100% medical vs 44.4% surgical; P < .001). Postoperatively, cerebrovascular accident and coma resolved in 84.3% and 78.8% of cases, respectively. On logistic regression analysis, surgery was protective against mortality in patients presenting with brain injury (odds ratio 0.058; P < .001). The 5-year survival of patients presenting with cerebrovascular accident and coma was 23.8% and 0% after medical management versus 67.1% and 57.1% after surgery (log rank, P < .001), respectively.
CONCLUSIONS: Brain injury at presentation adversely affects hospital survival of patients with type A acute aortic dissection. In the present observational study, the patients selected to undergo surgery demonstrated improved late survival and frequent reversal of neurologic deficits.

Copyright © 2013 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.
PMID 23410778  J Thorac Cardiovasc Surg. 2013 Mar;145(3 Suppl):S213-21・・・
著者: D C Miller, R S Mitchell, P E Oyer, E B Stinson, S W Jamieson, N E Shumway
雑誌名: Circulation. 1984 Sep;70(3 Pt 2):I153-64.
Abstract/Text A 20 year (1963 to 1982) surgical experience including 175 consecutive patients with aortic dissections was analyzed by logistic discriminant analyses to identify predictors of high operative risk. The patient population had characteristics similar to those in large autopsy series. Sixty-nine percent had type A and 58% had acute dissections. The intimal tear was located in the ascending aorta in 60% of the patients, the descending aorta in 27%, and the transverse arch in 13%. The overall operative mortality rate was 23 +/- 3%. The operative mortality rates were substantially lower between 1977 and 1982: mortality in patients with acute type A dissections, 7 +/- 5%; in those with chronic type A, 11 +/- 7%; in those with acute type B, 13 +/- 12%; and in those with chronic type B, 11 +/- 11%. After preliminary univariate screening, the following factors were determined to be significant independent predictors of operative mortality (in rank order of declining predictive power): type A patients (n = 121), renal dysfunction, tamponade, renal/visceral ischemia, and operative date; type B patients (n = 54), rupture, renal/visceral ischemia, and age; all patients (n = 175), renal dysfunction, renal/visceral ischemia, site of tear (ascending less than descending less than arch), tamponade, operative date, and pulmonary disease. Interestingly, several variables had no important bearing on operative mortality, including type (acute vs chronic) of dissection, age, previous operation, rupture, stroke, paraplegia, Marfan's syndrome, concomitant aortic valve replacement and/or coronary artery bypass grafting, site of tear, and whether or not the tear was resected in type A patients; emergency operation, hypertension, previous cardiac symptoms, paraplegia, site of tear, and resection of tear in type B patients; and, when all patients were considered together, age, sex, cardiac symptoms, prior operation, stroke, paraplegia, acute myocardial infarction, acute aortic regurgitation, Marfan's syndrome, and tear resection. These data allow calculation of any individual patient's operative risk and document that the operative mortality rate today is relatively low for all patients with aortic dissections, irrespective of type or acuity. Earlier surgical referral of patients with acute type A or acute type B dissection before irreversible major end-organ ischemia and/or infarction is probably in part responsible for the substantially improved results since 1977.

PMID 6235061  Circulation. 1984 Sep;70(3 Pt 2):I153-64.
著者: P R Sullivan, A B Wolfson, R D Leckey, J L Burke
雑誌名: Am J Emerg Med. 2000 Jan;18(1):46-50.
Abstract/Text In this article we try to determine how frequently emergency physicians (EPs) suspected the diagnosis in acute aortic dissection (AD). In this retrospective descriptive study, we identified all patients with the final diagnosis of AD initially evaluated in 1 of 3 emergency departments (EDs) over a 5-year period. Patients were included if AD was not suspected before ED evaluation. Patients undergoing thoracic aorta imaging as the initial ED study were defined as suspected AD. Forty-three patients totaling 44 presentations were identified. EPs suspected AD in 19 of 44 presentations. EPs suspected AD in 12 of 14 (86%) cases of chest and back pain and in 5 of 11 (45%) of chest pain. Thirteen of 39 (33%) painful presentations involved abdominal pain; EPs suspected AD in 1 of 13 (8%). EPs suspected the diagnosis in 43% of acute AD; location of pain was most predictive of a suspected diagnosis.

PMID 10674531  Am J Emerg Med. 2000 Jan;18(1):46-50.
著者: Hiroki Watanabe, Nobuyuki Horita, Yuji Shibata, Shintaro Minegishi, Erika Ota, Takeshi Kaneko
雑誌名: Sci Rep. 2016 May 27;6:26893. doi: 10.1038/srep26893. Epub 2016 May 27.
Abstract/Text Diagnostic test accuracy of D-dimer for acute aortic dissection (AAD) has not been evaluated by meta-analysis with the bivariate model methodology. Four databases were electrically searched. We included both case-control and cohort studies that could provide sufficient data concerning both sensitivity and specificity of D-dimer for AAD. Non-English language articles and conference abstract were allowed. Intramural hematoma and penetrating aortic ulcer were regarded as AAD. Based on 22 eligible articles consisting of 1140 AAD subjects and 3860 non-AAD subjects, the diagnostic odds ratio was 28.5 (95% CI 17.6-46.3, I(2) = 17.4%) and the area under curve was 0.946 (95% CI 0.903-0.994). Based on 833 AAD subjects and 1994 non-AAD subjects constituting 12 studies that used the cutoff value of 500 ng/ml, the sensitivity was 0.952 (95% CI 0.901-0.978), the specificity was 0.604 (95% CI 0.485-0.712), positive likelihood ratio was 2.4 (95% CI 1.8-3.3), and negative likelihood ratio was 0.079 (95% CI 0.036-0.172). Sensitivity analysis using data of three high-quality studies almost replicated these results. In conclusion, D-dimer has very good overall accuracy. D-dimer <500 ng/ml largely decreases the possibility of AAD. D-dimer >500 ng/ml moderately increases the possibility of AAD.

PMID 27230962  Sci Rep. 2016 May 27;6:26893. doi: 10.1038/srep26893. E・・・
著者: Y von Kodolitsch, A G Schwartz, C A Nienaber
雑誌名: Arch Intern Med. 2000 Oct 23;160(19):2977-82. doi: 10.1001/archinte.160.19.2977.
Abstract/Text BACKGROUND: Clinical criteria for aortic dissection are poorly defined. Thus, 35% of aortic dissections remain unsuspected in vivo, and 99% of suspected cases can be refuted.
OBJECTIVE: To identify independent predictors of acute aortic dissection and create a prediction model for facilitated estimation of the individual risk of dissection.
METHODS: Two hundred fifty patients with acute chest pain, back pain, or both; absence of an established differential diagnosis of the pain syndrome; and clinical suspicion of acute aortic dissection were evaluated for the presence of 26 clinical variables in a prospective, observational study. Multivariate analysis was performed to create a prediction model of aortic dissection.
RESULTS: Aortic pain with immediate onset, a tearing or ripping character, or both; mediastinal widening, aortic widening, or both on chest radiography; and pulse differentials, blood pressure differentials, or both (P<.001 for all) were identified as independent predictors of acute aortic dissection. Probability of dissection was low with absence of all 3 variables (7%), intermediate with isolated findings of aortic pain or mediastinal widening (31% and 39%, respectively), and high with isolated pulse or blood pressure differentials or any combination of the 3 variables (> or = 83%). Accordingly, 4% of all dissections were assigned to the low-probability group, 19% to the intermediate-probability group, and 77% to the high-probability group of aortic dissection.
CONCLUSIONS: Assessment of 3 clinical variables permitted identification of 96% of the acute aortic dissections and stratification into high-, intermediate-, and low-probability groupings of disease. With better selection for prompt diagnostic imaging, this prediction model can be used as an aid to improve patient care in aortic dissection. Arch Intern Med. 2000;160:2977-2982

PMID 11041906  Arch Intern Med. 2000 Oct 23;160(19):2977-82. doi: 10.1・・・
著者: Nozomi Sasamoto, Koichi Akutsu, Takeshi Yamamoto, Toshiaki Otsuka, Hideto Sangen, Hiroshi Hayashi, Hiroshige Murata, Hideki Miyachi, Yusuke Hosokawa, Shuhei Tara, Yukichi Tokita, Satoshi Miyata, Tetsuro Morota, Takashi Nitta, Wataru Shimizu
雑誌名: J Nippon Med Sch. 2021 Mar 9;. doi: 10.1272/jnms.JNMS.2021_88-605. Epub 2021 Mar 9.
Abstract/Text BACKGROUND: An inter-arm difference in blood pressure (IADBP) is characteristic of acute aortic dissection (AAD), but which arm shows lower blood pressure (BP) and the mechanism of IADBP has not been fully elucidatedMethods: We identified consecutive patients with chest and/or back pain and suspected acute cardiovascular disease whose BP had been measured in both arms. We retrospectively compared the characteristics of such patients with AAD (n=93) to those without (non-AAD group, n=122). Additionally, we separately compared patients with type A AAD (TAAD group, n=58) or type B AAD (TBAD group, n=35) to non-AAD group. Characteristics included in these comparisons were patients' backgrounds and IADBP-related factors such as systolic BP (SBP) in the right arm (R) and left arm (L), R-L or L-R as the IADBP. Computed tomography (CT) findings of AD extending to the brachiocephalic artery (BCA) and/or left subclavian artery (LSCA) were examined in patients having IADBP.
RESULTS: In the TAAD group, the prevalence of R<130mmHg (38%-vs.-19%, p=0.009), L-R>15mmHg (19%-vs.-8%, p=0.047), L-R>20mmHg (14%-vs.-4%, p=0.029) was higher than in the non-AAD group. Multivariate analysis showed L-R>15mmHg with R<130mmHg was independently associated with TAAD (OR 25.97, 95% CI 2.45-275.67, p=0.007). However, IADBP-related factors were not associated with TBAD. AAD patients with L-R>20mmHg were all TAAD, and all aortic dissection extended to BCA just before the right common carotid artery on CT.
CONCLUSIONS: IADBP was characterized by R
PMID 33692296  J Nippon Med Sch. 2021 Mar 9;. doi: 10.1272/jnms.JNMS.2・・・
著者: Toshiya Shiga, Zen'ichiro Wajima, Christian C Apfel, Tetsuo Inoue, Yoko Ohe
雑誌名: Arch Intern Med. 2006 Jul 10;166(13):1350-6. doi: 10.1001/archinte.166.13.1350.
Abstract/Text BACKGROUND: Patients with suspected thoracic aortic dissection require early and accurate diagnosis. Aortography has been replaced by less invasive imaging techniques including transesophageal echocardiography (TEE), helical computed tomography (CT), and magnetic resonance imaging (MRI); however, accuracies have varied from trial to trial, and which imaging technique should be applied to which risk population remains unclear. We systematically reviewed the diagnostic accuracy of these imaging techniques in patients with suspected thoracic aortic dissection.
METHODS: Published English-language reports on the diagnosis of thoracic aortic dissection by TEE, helical CT, or MRI were identified from electronic databases. Sensitivity, specificity, and positive and negative likelihood ratios were pooled in a random-effects model.
RESULTS: Sixteen studies involving a total of 1139 patients were selected. Pooled sensitivity (98%-100%) and specificity (95%-98%) were comparable between imaging techniques. The pooled positive likelihood ratio appeared to be higher for MRI (positive likelihood ratio, 25.3; 95% confidence interval, 11.1-57.1) than for TEE (14.1; 6.0-33.2) or helical CT (13.9; 4.2-46.0). If a patient had shown a 50% pretest probability of thoracic aortic dissection (high risk), he or she had a 93% to 96% posttest probability of thoracic aortic dissection following a positive result of each imaging test. If a patient had a 5% pretest probability of thoracic aortic dissection (low risk), he or she had a 0.1% to 0.3% posttest probability of thoracic aortic dissection following a negative result of each imaging test.
CONCLUSION: All 3 imaging techniques, ie, TEE, helical CT, and MRI, yield clinically equally reliable diagnostic values for confirming or ruling out thoracic aortic dissection.

PMID 16831999  Arch Intern Med. 2006 Jul 10;166(13):1350-6. doi: 10.10・・・
著者: Taro Hayashi, Takuro Tsukube, Teruo Yamashita, Tomonori Haraguchi, Ritsu Matsukawa, Shuichi Kozawa, Kyoichi Ogawa, Yutaka Okita
雑誌名: Circulation. 2012 Sep 11;126(11 Suppl 1):S97-S101. doi: 10.1161/CIRCULATIONAHA.111.082685.
Abstract/Text BACKGROUND: Cardiac tamponade is associated with fatal outcomes for patients with acute type A aortic dissection, and the presence of cardiac tamponade should prompt urgent aortic repair. However, treatment of the patient with critical cardiac tamponade who cannot survive until surgery remains unclear. We analyzed our experience of controlled pericardial drainage (CPD) managing critical cardiac tamponade.
METHODS AND RESULTS: Between September 2003 and May 2011, 175 patients with acute type A aortic dissection were treated surgically, including 43 (24.6%) who presented with cardiac tamponade on arrival. Eighteen patients, who did not respond to intravenous volume resuscitation, underwent CPD in the emergency department. An 8F pigtail drainage catheter was inserted percutaneously, and drainage volume was controlled by means of several cycles of intermittent drainage to maintain blood pressure at ≈90 mm Hg. After CPD, all of the patients were transferred to the operating room, and immediate aortic repair was performed. Systolic blood pressure before CPD was 64.3 ± 8.2 mm Hg and elevated significantly in all of the cases after CPD. Systolic blood pressure after CPD was 94.8 ± 10.5 mm Hg, and increase in systolic pressure was 30.5 ± 11.7 mm Hg. Total volume of aspirated pericardial effusion was 40.1 ± 30.6 mL, and 10 patients required only ≤30-mL aspiration volume. All of the patients underwent aortic repair successfully. In-hospital mortality was 16.7%; however, there was no complications or mortality related to CPD.
CONCLUSIONS: Preoperative pericardial drainage with control of volume is a safe and effective procedure for acute type A aortic dissection complicated by critical cardiac tamponade. In our patient population, timely controlled pericardial drainage is warranted.

PMID 22966000  Circulation. 2012 Sep 11;126(11 Suppl 1):S97-S101. doi:・・・
著者: Koichi Akutsu
雑誌名: Gen Thorac Cardiovasc Surg. 2019 Mar;67(3):271-276. doi: 10.1007/s11748-019-01066-x. Epub 2019 Jan 28.
Abstract/Text We discuss the etiology of aortic dissection (AD) from various points of view. The development of AD requires two pathological conditions: medial degeneration and mechanical wall stress. First, histopathological findings of medial degeneration are hypothesized to be due to a loss of elastic fibers and interconnecting elastic fibers. Damage to the vasa vasorum plays a key role in creating an entry site. The clinical causes of medial degeneration include hypertension, aortic aneurysms, obstructive sleep apnea, and connective tissue disorders. Second, mechanical wall stress is supposedly induced by shear stress caused by blood flow, together with hypertension and aortic root movement. Further investigation is necessary in the search for mechanisms responsible for medial degeneration prior to AD development.

PMID 30689200  Gen Thorac Cardiovasc Surg. 2019 Mar;67(3):271-276. doi・・・
著者: Gabriel Sampol, Odile Romero, Armando Salas, José L Tovar, Patricia Lloberes, Teresa Sagalés, Arturo Evangelista
雑誌名: Am J Respir Crit Care Med. 2003 Dec 15;168(12):1528-31. doi: 10.1164/rccm.200304-566OC. Epub 2003 Aug 6.
Abstract/Text Obstructive sleep apnea syndrome (OSAS) is a process that is associated with the development of arterial hypertension, the main risk factor for aortic dissection and during obstructive episodes of the upper airways with marked increases in transmural pressure of the aorta wall. The aim of this work was to study the association between aortic dissection and OSAS. Nineteen consecutive patients with thoracic aorta dissection and 19 hypertensive patients of similar age, sex, and body mass index were studied by clinical questionnaire and polysomnography. Snoring and nonrefreshing sleep were common in both groups. Thirteen patients (68%) from each group showed an apnea-hypopnea index of more than 5 per hour. However, patients with aortic dissection presented a higher apnea-hypopnea index (28 [30.3] versus 11.1 [10.4], p=0.032). Seven patients with dissection presented an apnea-hypopnea index of more than 30 versus 1 patient in the control group (p=0.042). Patients with thoracic aorta dissection presented a high prevalence of previously undiagnosed and frequently severe OSAS. Further studies, including this diagnosis as a prognostic variable in the follow-up of patients with aortic dissection, are required. Our results suggest that in patients with aortic dissection and symptoms consistent with OSAS, a sleep study should be considered in their clinical management.

PMID 12904327  Am J Respir Crit Care Med. 2003 Dec 15;168(12):1528-31.・・・
著者: Bart L Loeys, Ulrike Schwarze, Tammy Holm, Bert L Callewaert, George H Thomas, Hariyadarshi Pannu, Julie F De Backer, Gretchen L Oswald, Sofie Symoens, Sylvie Manouvrier, Amy E Roberts, Francesca Faravelli, M Alba Greco, Reed E Pyeritz, Dianna M Milewicz, Paul J Coucke, Duke E Cameron, Alan C Braverman, Peter H Byers, Anne M De Paepe, Harry C Dietz
雑誌名: N Engl J Med. 2006 Aug 24;355(8):788-98. doi: 10.1056/NEJMoa055695.
Abstract/Text BACKGROUND: The Loeys-Dietz syndrome is a recently described autosomal dominant aortic-aneurysm syndrome with widespread systemic involvement. The disease is characterized by the triad of arterial tortuosity and aneurysms, hypertelorism, and bifid uvula or cleft palate and is caused by heterozygous mutations in the genes encoding transforming growth factor beta receptors 1 and 2 (TGFBR1 and TGFBR2, respectively).
METHODS: We undertook the clinical and molecular characterization of 52 affected families. Forty probands presented with typical manifestations of the Loeys-Dietz syndrome. In view of the phenotypic overlap between this syndrome and vascular Ehlers-Danlos syndrome, we screened an additional cohort of 40 patients who had vascular Ehlers-Danlos syndrome without the characteristic type III collagen abnormalities or the craniofacial features of the Loeys-Dietz syndrome.
RESULTS: We found a mutation in TGFBR1 or TGFBR2 in all probands with typical Loeys-Dietz syndrome (type I) and in 12 probands presenting with vascular Ehlers-Danlos syndrome (Loeys-Dietz syndrome type II). The natural history of both types was characterized by aggressive arterial aneurysms (mean age at death, 26.0 years) and a high incidence of pregnancy-related complications (in 6 of 12 women). Patients with Loeys-Dietz syndrome type I, as compared with those with type II, underwent cardiovascular surgery earlier (mean age, 16.9 years vs. 26.9 years) and died earlier (22.6 years vs. 31.8 years). There were 59 vascular surgeries in the cohort, with one death during the procedure. This low rate of intraoperative mortality distinguishes the Loeys-Dietz syndrome from vascular Ehlers-Danlos syndrome.
CONCLUSIONS: Mutations in either TGFBR1 or TGFBR2 predispose patients to aggressive and widespread vascular disease. The severity of the clinical presentation is predictive of the outcome. Genotyping of patients presenting with symptoms like those of vascular Ehlers-Danlos syndrome may be used to guide therapy, including the use and timing of prophylactic vascular surgery.

Copyright 2006 Massachusetts Medical Society.
PMID 16928994  N Engl J Med. 2006 Aug 24;355(8):788-98. doi: 10.1056/N・・・
著者: M Pepin, U Schwarze, A Superti-Furga, P H Byers
雑誌名: N Engl J Med. 2000 Mar 9;342(10):673-80. doi: 10.1056/NEJM200003093421001.
Abstract/Text BACKGROUND: Ehlers-Danlos syndrome type IV, the vascular type, results from mutations in the gene for type III procollagen (COL3A1). Affected patients are at risk for arterial, bowel, and uterine rupture, but the timing of these events, their frequency, and the course of the disease are not well documented.
METHODS: We reviewed the clinical and family histories of and medical and surgical complications in 220 index patients with biochemically confirmed Ehlers-Danlos syndrome type IV and 199 of their affected relatives. We identified the underlying COL3A1 mutation in 135 index patients.
RESULTS: Complications were rare in childhood; 25 percent of the index patients had a first complication by the age of 20 years, and more than 80 percent had had at least one complication by the age of 40. The calculated median survival of the entire cohort was 48 years. Most deaths resulted from arterial rupture. Bowel rupture, which often involved the sigmoid colon, accounted for about a quarter of complications but rarely led to death. Complications of pregnancy led to death in 12 of the 81 women who became pregnant. The types of complications were not associated with specific mutations in COL3A1.
CONCLUSIONS: Although most affected patients survive the first and second major complications, Ehlers-Danlos syndrome type IV results in premature death. The diagnosis should be considered in young people who come to medical attention because of uterine rupture during pregnancy or arterial or visceral rupture.

PMID 10706896  N Engl J Med. 2000 Mar 9;342(10):673-80. doi: 10.1056/N・・・
著者: Ingrid M B H van de Laar, Denise van der Linde, Edwin H G Oei, Pieter K Bos, Johannes H Bessems, Sita M Bierma-Zeinstra, Belle L van Meer, Gerard Pals, Rogier A Oldenburg, Jos A Bekkers, Adriaan Moelker, Bianca M de Graaf, Gabor Matyas, Ingrid M E Frohn-Mulder, Janneke Timmermans, Yvonne Hilhorst-Hofstee, Jan M Cobben, Hennie T Bruggenwirth, Lut van Laer, Bart Loeys, Julie De Backer, Paul J Coucke, Harry C Dietz, Patrick J Willems, Ben A Oostra, Anne De Paepe, Jolien W Roos-Hesselink, Aida M Bertoli-Avella, Marja W Wessels
雑誌名: J Med Genet. 2012 Jan;49(1):47-57. doi: 10.1136/jmedgenet-2011-100382.
Abstract/Text BACKGROUND: Aneurysms-osteoarthritis syndrome (AOS) is a new autosomal dominant syndromic form of thoracic aortic aneurysms and dissections characterised by the presence of arterial aneurysms and tortuosity, mild craniofacial, skeletal and cutaneous anomalies, and early-onset osteoarthritis. AOS is caused by mutations in the SMAD3 gene.
METHODS: A cohort of 393 patients with aneurysms without mutation in FBN1, TGFBR1 and TGFBR2 was screened for mutations in SMAD3. The patients originated from The Netherlands, Belgium, Switzerland and USA. The clinical phenotype in a total of 45 patients from eight different AOS families with eight different SMAD3 mutations is described. In all patients with a SMAD3 mutation, clinical records were reviewed and extensive genetic, cardiovascular and orthopaedic examinations were performed.
RESULTS: Five novel SMAD3 mutations (one nonsense, two missense and two frame-shift mutations) were identified in five new AOS families. A follow-up description of the three families with a SMAD3 mutation previously described by the authors was included. In the majority of patients, early-onset joint abnormalities, including osteoarthritis and osteochondritis dissecans, were the initial symptom for which medical advice was sought. Cardiovascular abnormalities were present in almost 90% of patients, and involved mainly aortic aneurysms and dissections. Aneurysms and tortuosity were found in the aorta and other arteries throughout the body, including intracranial arteries. Of the patients who first presented with joint abnormalities, 20% died suddenly from aortic dissection. The presence of mild craniofacial abnormalities including hypertelorism and abnormal uvula may aid the recognition of this syndrome.
CONCLUSION: The authors provide further insight into the phenotype of AOS with SMAD3 mutations, and present recommendations for a clinical work-up.

PMID 22167769  J Med Genet. 2012 Jan;49(1):47-57. doi: 10.1136/jmedgen・・・
著者: Hiroshi Hazui, Masayoshi Nishimoto, Masaaki Hoshiga, Nobuyuki Negoro, Hideyuki Muraoka, Motonobu Murai, Yasuo Ohishi, Hitoshi Fukumoto, Hiroshi Morita
雑誌名: Circ J. 2006 Dec;70(12):1598-601. doi: 10.1253/circj.70.1598.
Abstract/Text BACKGROUND: Previous reports indicate that D-dimer testing (DT) for acute aortic dissection (AAD) has a sensitivity of 100%, but each study comprised less than 30 patients. The aim of this study was to evaluate the positive rate and factors related to the results of DT for AAD in a larger population.
METHODS AND RESULTS: DT (cutoff; upper normal limit) was performed for 113 consecutive AAD patients within 24 h of symptom onset. In total, 104 (92%) patients exhibited positive DT. The positive rate of DT showed a low tendency in patients aged less than 70 years and for a time interval from symptom onset to admission within 120 min, and there were significant differences between those with and without a thrombosed false lumen (TFL) (86.4% (n=59) vs 98.1% (n=54), p=0.033), complete TFL (excluding patients with ulcer-like projection (ULP) from those with a TFL) (81.1% (n=37) vs 97.4% (n=76), p=0.005) and length score (1 (n=28); 78.6%, 2 (n=40); 95.0%, 3 (n=45); 97.8%, p=0.005). Multivariate analysis demonstrated age (odds ratio =1.164, p=0.013), complete TFL (0.048, 0.030) and length score (6.271, 0.033) as independent factors.
CONCLUSIONS: Physicians should be aware that younger patients with short dissection length and a TFL without ULP are liable to have false-negative DT results.

PMID 17127806  Circ J. 2006 Dec;70(12):1598-601. doi: 10.1253/circj.70・・・
著者: A E HIRST, V J JOHNS, S W KIME
雑誌名: Medicine (Baltimore). 1958 Sep;37(3):217-79.
Abstract/Text
PMID 13577293  Medicine (Baltimore). 1958 Sep;37(3):217-79.
著者: K Nishigami, T Tsuchiya, H Shono, Y Horibata, T Honda
雑誌名: Circulation. 2000 Nov 7;102(19 Suppl 3):III243-7.
Abstract/Text BACKGROUND: An aortic intramural hematoma (IMH) is a form of aortic dissection (AD). IMHs regress with time or completely disappear in some patients, whereas they progress to overt AD in other patients. The purpose of the present study was to investigate how IMHs change serially during a follow-up period.
METHODS AND RESULTS: We analyzed 44 consecutive medically treated patients with IMHs, in whom transesophageal echocardiography (TEE) was performed serially at both 1 and 6 months after the onset. After TEE, the patients were followed with interviews (mean follow-up 1552+/-539 days). IMHs disappeared at 6 months in 21 patients (48%) (disappearance group), whereas IMHs were still demonstrated at 6 months in 20 patients (45%) (persistent group); in the disappearance group, IMHs disappeared at 1 month in 8 patients (18%). In 3 patients (7%) in whom an IMH was demonstrated at 1 month, overt AD occurred until 6 months. The disappearance group was younger than the persistent group (64+/-11 versus 72+/-8 years, P:<0.01), and the maximum diameter of the aorta was smaller in the disappearance group than in the persistent group (33+/-5 versus 42+/-7 mm, P:<0.01). During the long-term follow-up, overt AD occurred at 7 and 11 months in 2 patients, and progressive aortic dilatation that required surgical treatment occurred at 12 and 24 months in 2 of the persistent group patients, whereas neither overt AD nor progressive aortic dilatation occurred in the disappearance group. In the patients in whom overt AD occurred, the maximal aortic diameter was >45 mm and an IMH was demonstrated at 1 month. On the other hand, those with a maximal aortic diameter of <45 mm or a disappeared IMH did not have overt AD.
CONCLUSIONS: IMHs disappeared until 1 month in 18% and until 6 months in 48% of patients with IMHs. The disappearance of IMHs was related to the maximum diameter of the aorta and age. Both a disappeared IMH and a maximal aortic diameter of <45 mm suggest a good prognosis.

PMID 11082395  Circulation. 2000 Nov 7;102(19 Suppl 3):III243-7.
著者: Keiji Uchida, Norihisa Karube, Keiichiro Kasama, Tomokazu Minami, Shota Yasuda, Motohiko Goda, Shinichi Suzuki, Kiyotaka Imoto, Munetaka Masuda
雑誌名: J Thorac Cardiovasc Surg. 2018 Aug;156(2):483-489. doi: 10.1016/j.jtcvs.2018.02.007. Epub 2018 Feb 13.
Abstract/Text OBJECTIVE: The control of malperfusion is the key to improving the outcomes of surgery for type A acute aortic dissection. We revised our treatment strategy to reperfuse each ischemic organ before central repair.
METHODS: Our current early reperfusion strategy consists of percutaneous coronary artery intervention for coronary malperfusion, direct surgical fenestration for carotid artery occlusion, active perfusion of the superior mesenteric artery for visceral malperfusion, and external shunting from the brachial artery to the femoral artery for lower limb ischemia. Central repair is performed without delay after reperfusion therapy, but if irreversible organ damage is recognized, further aggressive treatment is discontinued.
RESULTS: Among 438 patients who underwent initial treatment for type A acute aortic dissection, malperfusion in one or more organs was diagnosed in 108 patients (24%). We applied an early reperfusion strategy in 33 patients, (coronary, 14 patients; carotid, 4; visceral, 7; lower extremity, 8). Central repair was then performed in 28 patients. One patient (3.6%) died of pneumonia; 27 patients overcame the ischemic organ damage and survived. Among the 108 patients with malperfusion, 10 patients (9.3%) were treated medically without early reperfusion and central repair. During the same period, mortality from central repair procedures in patients with malperfusion who had not received early reperfusion therapy was 12 of 65 (18%), and the mortality of patients without malperfusion was 9 of 262 (3.4%). Malperfusion was a serious risk factor for hospital death, but the mortality rate of the patients with an early reperfusion strategy was significantly (P < .01) lower than the patients without early reperfusion.
CONCLUSIONS: Our strategy might improve the outcomes of surgery for type A acute aortic dissection with malperfusion. This strategy enables us to avoid unproductive central repair procedures in irreversibly damaged patients.

Copyright © 2018 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.
PMID 29548594  J Thorac Cardiovasc Surg. 2018 Aug;156(2):483-489. doi:・・・
著者: Takuro Tsukube, Taro Hayashi, Toshihiro Kawahira, Tomonori Haraguchi, Ritsu Matsukawa, Shuichi Kozawa, Kyoichi Ogawa, Yutaka Okita
雑誌名: Circulation. 2011 Sep 13;124(11 Suppl):S163-7. doi: 10.1161/CIRCULATIONAHA.110.011551.
Abstract/Text BACKGROUND: Management of acute type A aortic dissection (AADA) complicated by coma remains controversial. We analyzed our experience in managing AADA complicated by coma to determine the relationship of duration of preoperative coma to postoperative neurological recovery.
METHODS AND RESULTS: Between September 2003 and October 2010, 181 patients with AADA were treated, including 27 presenting with coma (Glasgow Coma Scale <11) on arrival. Twenty-one patients were repaired immediately (immediate group); time from onset of symptoms to operating room was <5 hours. For brain protection, deep hypothermia with antegrade cerebral perfusion was used, and postoperative therapeutic hypothermia with magnesium treatment was performed. Six patients initially were managed medically, and 3 of them were followed by eventual repair because time from onset was >5 hours (delayed group). The preoperative National Institutes of Health Stroke Scale score was 31.4 ± 6.6 in the immediate group and 28.3 ± 9.5 in the delayed group. Hospital mortality was 14% in the immediate group and 67% in the delayed group. Full recovery of consciousness was achieved in 86% of patients in the immediate group and in 17% in the delayed group. In immediate group, the postoperative National Institutes of Health Stroke Scale score significantly improved to 6.4 ± 8.4, cumulative survival rate was 71.8% in 3 years, and independence in daily activities was achieved in 52% (11/21).
CONCLUSIONS: Aortic repair, if performed immediately from the onset of symptoms, showed satisfactory recovery of consciousness and neurological function in patients with AADA complicated by coma. In this patient population, immediate aortic repair is warranted.

PMID 21911807  Circulation. 2011 Sep 13;124(11 Suppl):S163-7. doi: 10.・・・
著者: Kazuhisa Kodama, Kazuhiro Nishigami, Tomohiro Sakamoto, Tadashi Sawamura, Touitsu Hirayama, Hiroyasu Misumi, Koichi Nakao
雑誌名: Circulation. 2008 Sep 30;118(14 Suppl):S167-70. doi: 10.1161/CIRCULATIONAHA.107.755801.
Abstract/Text BACKGROUND: Although type B aortic dissection has been treated with beta blockers to lower the arterial blood pressure (BP), there has been little evidences about reduction in heart rate (HR). We assessed whether tight HR control improved the outcome of medical treatment in patients with aortic dissection.
METHODS AND RESULTS: From 1997 to 2005, 171 patients with acute aortic dissection medically treated and controlled to lower BP under 120 mm Hg were enrolled. Based on the average HR at 3, 5, and 7 days after the onset, patients were divided into tight HR (<60 beat per minute) control group (32 patients; mean HR of 56.6+/-3.1 beat per minute) and conventional HR (>/=60 beat per minute) control group (139 patients; mean HR of 71.7+/-8.2 beat per minute). We compared the frequency of aortic events including late organ or limb ischemia, aortic rupture, recurrent dissection, and aortic expansion of >5 mm, and surgical requirement between two groups. During a median follow-up of 27.0 months, late organ or limb ischemia, aortic rupture, recurrent dissection, pathological aortic expansion, and aortic surgery occurred in 0, 8, 14, 39, and 26 patients, respectively. Reduction in aortic events was observed in tight HR control group (12.5%) compared to conventional HR control group (36.0%), (Odds ratio: 0.25, C.I.: 0.08 to 0.77, P<0.01).
CONCLUSIONS: The present study demonstrated that tight heart rate control improved the outcome of medical treatment in patients with aortic dissection.

PMID 18824750  Circulation. 2008 Sep 30;118(14 Suppl):S167-70. doi: 10・・・
著者: M Genoni, M Paul, R Jenni, K Graves, B Seifert, M Turina
雑誌名: Eur J Cardiothorac Surg. 2001 May;19(5):606-10.
Abstract/Text OBJECTIVES: To compare the medical treatment of chronic type B aortic dissection with beta-blockers versus other antihypertensive treatments in terms of their requirement for surgical intervention and treatment costs.
METHODS: Case records of the 130 patients treated for aortic dissection type B in this unit between 1988 and 1997 were reviewed. Seventy-eight of 130 patients with chronic dissection have received isolated medical treatment. Seventy-one of 78 patients were discharged alive. Fifty-one of 71 received beta-blocker treatment, 20/71 were treated with other antihypertensive drugs.
RESULTS: Surgery for aortic dissection became necessary in 20/71 patients (28%) during follow-up (mean, 4.2 years): 10/51 in the beta-blocker group and 9/20 in the other antihypertensive drug group. The freedom from subsequent aortic operation was 80 and 47%, respectively (P=0.001). Indications for emergency surgery were increased aortic diameter (79%), symptomatic aortic aneurysm (11%), and renal artery hypoperfusion (5%). The median hospitalization time during follow-up (dissection-related) was 2 days for patients who received beta-blockers and 16 days for patients who received other antihypertensive drug treatments (P=0.001). The cost of treatment/patient per year amounted to 644 and 12748 euros, respectively.
CONCLUSIONS: A substantial proportion of patients with chronic type B dissection who receive initial medical management will later need surgery. Long-term treatment with beta-blockers reduces the progression of aortic dilatation, the incidence of subsequent hospital admissions, as well as the incidence of late dissection-related aortic procedures and the cost of treatment. Patients with chronic type B dissection need, in addition to frequent follow-up of aortic diameter, continuous treatment with beta-blocking agents.

PMID 11343940  Eur J Cardiothorac Surg. 2001 May;19(5):606-10.
著者: Yusuke Jo, Toshihisa Anzai, Yasuo Sugano, Kotaro Naito, Koji Ueno, Takashi Kohno, Tsutomu Yoshikawa, Satoshi Ogawa
雑誌名: Heart Vessels. 2008 Sep;23(5):334-40. doi: 10.1007/s00380-008-1048-7. Epub 2008 Sep 20.
Abstract/Text We have reported that serum C-reactive protein (CRP) elevation is an independent predictor of lung oxygenation impairment (LOI) after distal type acute aortic dissection (AAD). Systemic activation of the inflammatory system after aortic injury may play a role in the development of LOI. The aim of this study is to clarify the effect of beta-blockers on systemic inflammation and the development of LOI after distal type AAD. A total of 49 patients, who were admitted with distal type AAD and treated conservatively, were examined. White blood cell (WBC) count, serum CRP level, and arterial blood gases were measured serially. Forty patients received beta-blocker treatment within 24 h of the onset, while 9 patients received no beta-blocker treatment. Maximum WBC count, maximum CRP level, lowest PaO(2)/FiO(2) (P/F) ratio, and patient background were compared between the two groups. There was no difference between the groups according to age, sex, coronary risk factors, blood pressure, serum level of CRP, WBC count, and oxygenation index on admission. Beta-blocker treatment was associated with lower maximum WBC count (P = 0.0028) and lower maximum serum CRP level (P = 0.0004). The minimum P/F ratio was higher in patients with beta-blocker treatment than in those without (P = 0.0076). Multivariate analysis revealed that administration of a beta-blocker was an independent negative determinant of LOI (P/F ratio < or = 200 mmHg). In conclusion, early use of beta-blockers prevented excessive inflammation and LOI after distal type AAD, suggesting a pleiotropic effect of beta-blockers on the inflammatory response after AAD.

PMID 18810582  Heart Vessels. 2008 Sep;23(5):334-40. doi: 10.1007/s003・・・
著者: Satoshi Takeshita, Shingo Sakamoto, Shuichi Kitada, Koichi Akutsu, Hideki Hashimoto
雑誌名: Circ J. 2008 Nov;72(11):1758-61. Epub 2008 Sep 29.
Abstract/Text BACKGROUND: Medical therapy gives excellent short-term outcomes for patients with Stanford type B acute aortic dissection. However, the affected aorta is often compromised by aneurysmal dilatation and rupture, resulting in poor long-term outcome. The present study investigated which antihypertensive treatment may prevent long-term aortic events in these patients.
METHODS AND RESULTS: The study group comprised 78 consecutive patients with Stanford type B acute aortic dissection who were treated medically and followed-up for an average of 873+/-548 days. The optimal hypertensive drug regimen to reduce aortic events was determined by multivariate analyses. Of the 78 patients, 73 (94%) were discharged from hospital with medical therapy, and aortic events occurred in 13 (18%) of them (aortic rupture/recurrent dissection in 2 (3%); aortic expansion >or=60 mm in 7 (10%), rapid aortic expansion >or=10 mm/year in 3 (4%), and development of visceral/limb ischemia in 1 (1%)) during follow-up. By multivariate analysis, patients given angiotensin-converting enzyme inhibitor (ACEI) were less likely to have long-term aortic events than those without (odds ratio: 0.18, 95% confidence interval: 0.04-0.85).
CONCLUSIONS: Use of ACEI is associated with a reduced risk of long-term aortic events in patients with medically treated type B aortic dissection.

PMID 18827369  Circ J. 2008 Nov;72(11):1758-61. Epub 2008 Sep 29.
著者: Daniel G Hackam, Deva Thiruchelvam, Donald A Redelmeier
雑誌名: Lancet. 2006 Aug 19;368(9536):659-65. doi: 10.1016/S0140-6736(06)69250-7.
Abstract/Text BACKGROUND: Angiotensin-converting enzyme (ACE) inhibitors prevent the expansion and rupture of aortic aneurysms in animals. We investigated the association between ACE inhibitors and rupture in patients with abdominal aortic aneurysms.
METHODS: We did a population-based case-control study of linked administrative databases in Ontario, Canada. The sample included consecutive patients older than 65 (n=15,326) admitted to hospital with a primary diagnosis of ruptured or intact abdominal aortic aneurysm between April 1, 1992, and April 1, 2002.
FINDINGS: Patients who received ACE inhibitors before admission were significantly less likely to present with ruptured aneurysm (odds ratio [OR] 0.82, 95% CI 0.74-0.90) than those who did not receive ACE inhibitors. Adjustment for demographic characteristics, risk factors for rupture, comorbidities, contraindications to ACE inhibitors, measures of health-care use, and aneurysm screening yielded similar results (0.83, 0.73-0.95). Consistent findings were noted in subgroups at high risk of rupture, including patients older than 75 years and those with a history of hypertension. Conversely, such protective associations were not observed for beta blockers (1.02, 0.89-1.17), calcium channel blockers (1.01, 0.89-1.14), alpha blockers (1.15, 0.86-1.54), angiotensin receptor blockers (1.24, 0.71-2.18), or thiazide diuretics (0.91, 0.78-1.07).
INTERPRETATION: ACE inhibitors are associated with a reduced risk of ruptured abdominal aortic aneurysm, unlike other antihypertensive agents. Randomised trials of ACE inhibitors for prevention of aortic rupture might be warranted.

PMID 16920471  Lancet. 2006 Aug 19;368(9536):659-65. doi: 10.1016/S014・・・
著者: Bart L Loeys, Harry C Dietz, Alan C Braverman, Bert L Callewaert, Julie De Backer, Richard B Devereux, Yvonne Hilhorst-Hofstee, Guillaume Jondeau, Laurence Faivre, Dianna M Milewicz, Reed E Pyeritz, Paul D Sponseller, Paul Wordsworth, Anne M De Paepe
雑誌名: J Med Genet. 2010 Jul;47(7):476-85. doi: 10.1136/jmg.2009.072785.
Abstract/Text The diagnosis of Marfan syndrome (MFS) relies on defined clinical criteria (Ghent nosology), outlined by international expert opinion to facilitate accurate recognition of this genetic aneurysm syndrome and to improve patient management and counselling. These Ghent criteria, comprising a set of major and minor manifestations in different body systems, have proven to work well since with improving molecular techniques, confirmation of the diagnosis is possible in over 95% of patients. However, concerns with the current nosology are that some of the diagnostic criteria have not been sufficiently validated, are not applicable in children or necessitate expensive and specialised investigations. The recognition of variable clinical expression and the recently extended differential diagnosis further confound accurate diagnostic decision making. Moreover, the diagnosis of MFS--whether or not established correctly--can be stigmatising, hamper career aspirations, restrict life insurance opportunities, and cause psychosocial burden. An international expert panel has established a revised Ghent nosology, which puts more weight on the cardiovascular manifestations and in which aortic root aneurysm and ectopia lentis are the cardinal clinical features. In the absence of any family history, the presence of these two manifestations is sufficient for the unequivocal diagnosis of MFS. In absence of either of these two, the presence of a bonafide FBN1 mutation or a combination of systemic manifestations is required. For the latter a new scoring system has been designed. In this revised nosology, FBN1 testing, although not mandatory, has greater weight in the diagnostic assessment. Special considerations are given to the diagnosis of MFS in children and alternative diagnoses in adults. We anticipate that these new guidelines may delay a definitive diagnosis of MFS but will decrease the risk of premature or misdiagnosis and facilitate worldwide discussion of risk and follow-up/management guidelines.

PMID 20591885  J Med Genet. 2010 Jul;47(7):476-85. doi: 10.1136/jmg.20・・・
著者: Ronald V Lacro, Harry C Dietz, Lynn A Sleeper, Anji T Yetman, Timothy J Bradley, Steven D Colan, Gail D Pearson, E Seda Selamet Tierney, Jami C Levine, Andrew M Atz, D Woodrow Benson, Alan C Braverman, Shan Chen, Julie De Backer, Bruce D Gelb, Paul D Grossfeld, Gloria L Klein, Wyman W Lai, Aimee Liou, Bart L Loeys, Larry W Markham, Aaron K Olson, Stephen M Paridon, Victoria L Pemberton, Mary Ella Pierpont, Reed E Pyeritz, Elizabeth Radojewski, Mary J Roman, Angela M Sharkey, Mario P Stylianou, Stephanie Burns Wechsler, Luciana T Young, Lynn Mahony, Pediatric Heart Network Investigators
雑誌名: N Engl J Med. 2014 Nov 27;371(22):2061-71. doi: 10.1056/NEJMoa1404731. Epub 2014 Nov 18.
Abstract/Text BACKGROUND: Aortic-root dissection is the leading cause of death in Marfan's syndrome. Studies suggest that with regard to slowing aortic-root enlargement, losartan may be more effective than beta-blockers, the current standard therapy in most centers.
METHODS: We conducted a randomized trial comparing losartan with atenolol in children and young adults with Marfan's syndrome. The primary outcome was the rate of aortic-root enlargement, expressed as the change in the maximum aortic-root-diameter z score indexed to body-surface area (hereafter, aortic-root z score) over a 3-year period. Secondary outcomes included the rate of change in the absolute diameter of the aortic root; the rate of change in aortic regurgitation; the time to aortic dissection, aortic-root surgery, or death; somatic growth; and the incidence of adverse events.
RESULTS: From January 2007 through February 2011, a total of 21 clinical centers enrolled 608 participants, 6 months to 25 years of age (mean [±SD] age, 11.5±6.5 years in the atenolol group and 11.0±6.2 years in the losartan group), who had an aortic-root z score greater than 3.0. The baseline-adjusted rate of change in the mean (±SE) aortic-root z score did not differ significantly between the atenolol group and the losartan group (-0.139±0.013 and -0.107±0.013 standard-deviation units per year, respectively; P=0.08). Both slopes were significantly less than zero, indicating a decrease in the aortic-root diameter relative to body-surface area with either treatment. The 3-year rates of aortic-root surgery, aortic dissection, death, and a composite of these events did not differ significantly between the two treatment groups.
CONCLUSIONS: Among children and young adults with Marfan's syndrome who were randomly assigned to losartan or atenolol, we found no significant difference in the rate of aortic-root dilatation between the two treatment groups over a 3-year period. (Funded by the National Heart, Lung, and Blood Institute and others; ClinicalTrials.gov number, NCT00429364.).

PMID 25405392  N Engl J Med. 2014 Nov 27;371(22):2061-71. doi: 10.1056・・・
著者: Maarten Groenink, Alexander W den Hartog, Romy Franken, Teodora Radonic, Vivian de Waard, Janneke Timmermans, Arthur J Scholte, Maarten P van den Berg, Anje M Spijkerboer, Henk A Marquering, Aeilko H Zwinderman, Barbara J M Mulder
雑誌名: Eur Heart J. 2013 Dec;34(45):3491-500. doi: 10.1093/eurheartj/eht334. Epub 2013 Sep 2.
Abstract/Text AIM: Patients with Marfan syndrome have an increased risk of life-threatening aortic complications, mostly preceded by aortic dilatation. Treatment with losartan, an angiotensin-II receptor-1 blocker, may reduce aortic dilatation rate in Marfan patients.
METHODS AND RESULTS: In this multicentre, open-label, randomized controlled trial with blinded assessments, we compared losartan treatment with no additional treatment in operated and unoperated adults with Marfan syndrome. The primary endpoint was aortic dilatation rate at any predefined aortic level after 3 years of follow-up, as determined by magnetic resonance imaging. A total of 233 participants (47% female) underwent randomization to either losartan (n = 116) or no additional treatment (n = 117). Aortic root dilatation rate after 3.1 ± 0.4 years of follow-up was significantly lower in the losartan group than in controls (0.77 ± 1.36 vs. 1.35 ± 1.55 mm, P = 0.014). Aortic dilatation rate in the trajectory beyond the aortic root was not significantly reduced by losartan. In patients with prior aortic root replacement, aortic arch dilatation rate was significantly lower in the losartan group when compared with the control group (0.50 ± 1.26 vs. 1.01 ± 1.31 mm, P = 0.033). No significant differences in separate clinical endpoints or the composite endpoint (aortic dissection, elective aortic surgery, cardiovascular death) between the groups could be demonstrated.
CONCLUSION: In adult Marfan patients, losartan treatment reduces aortic root dilatation rate. After aortic root replacement, losartan treatment reduces dilatation rate of the aortic arch.

PMID 23999449  Eur Heart J. 2013 Dec;34(45):3491-500. doi: 10.1093/eur・・・
著者: Thomas T Tsai, Arturo Evangelista, Christoph A Nienaber, Truls Myrmel, Gabriel Meinhardt, Jeanna V Cooper, Dean E Smith, Toru Suzuki, Rossella Fattori, Alfredo Llovet, James Froehlich, Stuart Hutchison, Alessandro Distante, Thoralf Sundt, Joshua Beckman, James L Januzzi, Eric M Isselbacher, Kim A Eagle, International Registry of Acute Aortic Dissection
雑誌名: N Engl J Med. 2007 Jul 26;357(4):349-59. doi: 10.1056/NEJMoa063232.
Abstract/Text BACKGROUND: Patency or thrombosis of the false lumen in type B acute aortic dissection has been found to predict outcomes. The prognostic implications of partial thrombosis of the false lumen have not yet been elucidated.
METHODS: We examined 201 patients with type B acute aortic dissection who were enrolled in the International Registry of Acute Aortic Dissection between 1996 and 2003 and who survived to hospital discharge. Kaplan-Meier mortality curves were stratified according to the status of the false lumen (patent, partial thrombosis, or complete thrombosis) as determined during the index hospitalization. Cox proportional-hazards analysis was performed to identify independent predictors of death.
RESULTS: During the index hospitalization, 114 patients (56.7%) had a patent false lumen, 68 patients (33.8%) had partial thrombosis of the false lumen, and 19 (9.5%) had complete thrombosis of the false lumen. The mean (+/-SD) 3-year mortality rate for patients with a patent false lumen was 13.7+/-7.1%, for those with partial thrombosis was 31.6+/-12.4%, and for those with complete thrombosis was 22.6+/-22.6% (median follow-up, 2.8 years; P=0.003 by the log-rank test). Independent predictors of postdischarge mortality were partial thrombosis of the false lumen (relative risk, 2.69; 95% confidence interval [CI], 1.45 to 4.98; P=0.002), a history of aortic aneurysm (relative risk, 2.05; 95% CI, 1.07 to 3.93; P=0.03), and a history of atherosclerosis (relative risk, 1.87; 95% CI, 1.01 to 3.47; P=0.05).
CONCLUSIONS: Mortality is high after discharge from the hospital among patients with type B acute aortic dissection. Partial thrombosis of the false lumen, as compared with complete patency, is a significant independent predictor of postdischarge mortality in these patients.

Copyright 2007 Massachusetts Medical Society.
PMID 17652650  N Engl J Med. 2007 Jul 26;357(4):349-59. doi: 10.1056/N・・・
著者: Christoph A Nienaber, Stephan Kische, Hervé Rousseau, Holger Eggebrecht, Tim C Rehders, Guenther Kundt, Aenne Glass, Dierk Scheinert, Martin Czerny, Tilo Kleinfeldt, Burkhart Zipfel, Louis Labrousse, Rossella Fattori, Hüseyin Ince, INSTEAD-XL trial
雑誌名: Circ Cardiovasc Interv. 2013 Aug;6(4):407-16. doi: 10.1161/CIRCINTERVENTIONS.113.000463. Epub 2013 Aug 6.
Abstract/Text BACKGROUND: Thoracic endovascular aortic repair (TEVAR) represents a therapeutic concept for type B aortic dissection. Long-term outcomes and morphology after TEVAR for uncomplicated dissection are unknown.
METHODS AND RESULTS: A total of 140 patients with stable type B aortic dissection previously randomized to optimal medical treatment and TEVAR (n=72) versus optimal medical treatment alone (n=68) were analyzed retrospectively for aorta-specific, all-cause outcomes, and disease progression using landmark statistical analysis of years 2 to 5 after index procedure. Cox regression was used to compare outcomes between groups; all analyses are based on intention to treat. The risk of all-cause mortality (11.1% versus 19.3%; P=0.13), aorta-specific mortality (6.9% versus 19.3%; P=0.04), and progression (27.0% versus 46.1%; P=0.04) after 5 years was lower with TEVAR than with optimal medical treatment alone. Landmark analysis suggested a benefit of TEVAR for all end points between 2 and 5 years; for example, for all-cause mortality (0% versus 16.9%; P=0.0003), aorta-specific mortality (0% versus 16.9%; P=0.0005), and for progression (4.1% versus 28.1%; P=0.004); Landmarking at 1 year and 1 month revealed consistent findings. Both improved survival and less progression of disease at 5 years after elective TEVAR were associated with stent graft induced false lumen thrombosis in 90.6% of cases (P<0.0001).
CONCLUSIONS: In this study of survivors of type B aortic dissection, TEVAR in addition to optimal medical treatment is associated with improved 5-year aorta-specific survival and delayed disease progression. In stable type B dissection with suitable anatomy, preemptive TEVAR should be considered to improve late outcome.
CLINICAL TRIAL REGISTRATION URL: http://www.clinicaltrials.gov. Unique identifier: NCT01415804.

PMID 23922146  Circ Cardiovasc Interv. 2013 Aug;6(4):407-16. doi: 10.1・・・
著者: Hitoshi Matsuda
雑誌名: Gen Thorac Cardiovasc Surg. 2017 Feb;65(2):74-79. doi: 10.1007/s11748-016-0734-0. Epub 2016 Dec 5.
Abstract/Text Hospital mortality after the replacement of chronic type B aortic dissection is around 8-10% and adverse outcomes include paraplegia and stroke. However, the level of evidence for indication of thoracic endovascular aortic repair (TEVAR) for type B chronic aortic dissection is Class IIa. Results of the INSTEAD-XL trial have verified that preemptive TEVAR for uncomplicated type B aortic dissection improves prognosis. The indication for this procedure is reportedly a maximum aortic diameter >40 mm during the acute phase and a patent primary entry site in the thoracic aorta, while the optimal timing for TEVAR would be the subacute phase, from 2 weeks to 3-6 months after onset. Prevention of chronic type B aortic dissection with aneurysmal degeneration and attainment of aortic remodeling with preemptive TEVAR are needed to free patients from the need for long-term strict control of blood pressure and periodic follow-ups involving radiological exposure and to avoid the eventual need for extensive open surgery.

PMID 27921203  Gen Thorac Cardiovasc Surg. 2017 Feb;65(2):74-79. doi: ・・・
著者: Loren F Hiratzka, George L Bakris, Joshua A Beckman, Robert M Bersin, Vincent F Carr, Donald E Casey, Kim A Eagle, Luke K Hermann, Eric M Isselbacher, Ella A Kazerooni, Nicholas T Kouchoukos, Bruce W Lytle, Dianna M Milewicz, David L Reich, Souvik Sen, Julie A Shinn, Lars G Svensson, David M Williams, American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, Society for Vascular Medicine
雑誌名: J Am Coll Cardiol. 2010 Apr 6;55(14):e27-e129. doi: 10.1016/j.jacc.2010.02.015.
Abstract/Text
PMID 20359588  J Am Coll Cardiol. 2010 Apr 6;55(14):e27-e129. doi: 10.・・・
著者: P G Hagan, C A Nienaber, E M Isselbacher, D Bruckman, D J Karavite, P L Russman, A Evangelista, R Fattori, T Suzuki, J K Oh, A G Moore, J F Malouf, L A Pape, C Gaca, U Sechtem, S Lenferink, H J Deutsch, H Diedrichs, J Marcos y Robles, A Llovet, D Gilon, S K Das, W F Armstrong, G M Deeb, K A Eagle
雑誌名: JAMA. 2000 Feb 16;283(7):897-903.
Abstract/Text CONTEXT: Acute aortic dissection is a life-threatening medical emergency associated with high rates of morbidity and mortality. Data are limited regarding the effect of recent imaging and therapeutic advances on patient care and outcomes in this setting.
OBJECTIVE: To assess the presentation, management, and outcomes of acute aortic dissection.
DESIGN: Case series with patients enrolled between January 1996 and December 1998. Data were collected at presentation and by physician review of hospital records.
SETTING: The International Registry of Acute Aortic Dissection, consisting of 12 international referral centers.
PARTICIPANTS: A total of 464 patients (mean age, 63 years; 65.3% male), 62.3% of whom had type A dissection.
MAIN OUTCOME MEASURES: Presenting history, physical findings, management, and mortality, as assessed by history and physician review of hospital records.
RESULTS: While sudden onset of severe sharp pain was the single most common presenting complaint, the clinical presentation was diverse. Classic physical findings such as aortic regurgitation and pulse deficit were noted in only 31.6% and 15.1% of patients, respectively, and initial chest radiograph and electrocardiogram were frequently not helpful (no abnormalities were noted in 12.4% and 31.3% of patients, respectively). Computed tomography was the initial imaging modality used in 61.1%. Overall in-hospital mortality was 27.4%. Mortality of patients with type A dissection managed surgically was 26%; among those not receiving surgery (typically because of advanced age and comorbidity), mortality was 58%. Mortality of patients with type B dissection treated medically was 10.7%. Surgery was performed in 20% of patients with type B dissection; mortality in this group was 31.4%.
CONCLUSIONS: Acute aortic dissection presents with a wide range of manifestations, and classic findings are often absent. A high clinical index of suspicion is necessary. Despite recent advances, in-hospital mortality rates remain high. Our data support the need for continued improvement in prevention, diagnosis, and management of acute aortic dissection.

PMID 10685714  JAMA. 2000 Feb 16;283(7):897-903.
著者: Y Kozai, S Watanabe, M Yonezawa, Y Itani, T Inoue, J Takasu, Y Masuda
雑誌名: Jpn Circ J. 2001 May;65(5):359-63.
Abstract/Text Between 1973 and 1998, 263 patients with acute aortic dissection were medically treated only. They were divided into 4 groups: Stanford type A and B with open false lumen (open) or with early thrombosed false lumen (thrombosed). An event was defined as death by dissection or re-dissection. Gender, age, maximum diameter of dissected aorta and presence of shock at onset were examined as risk factors. In the open false lumen group, the presence of shock was associated with the event. During the chronic period, the diameter of the aorta was associated with prognosis in open type B dissection. The rate of event was higher in the open type A and B groups than in the thrombosed type A and B groups; however, there was no difference in the event-free rate between types A and B in patients surviving the acute period. The prognosis of medically treated dissecting aorta was not poor in patients with type B or with early thrombosed false lumen. The presence of shock at onset with open false lumen and the diameter of the aorta (> or =40 mm) in type B were significantly correlated with a poor prognosis.

PMID 11348035  Jpn Circ J. 2001 May;65(5):359-63.
著者: Raimund Erbel, Victor Aboyans, Catherine Boileau, Eduardo Bossone, Roberto Di Bartolomeo, Holger Eggebrecht, Arturo Evangelista, Volkmar Falk, Herbert Frank, Oliver Gaemperli, Martin Grabenwöger, Axel Haverich, Bernard Iung, Athanasios John Manolis, Folkert Meijboom, Christoph A Nienaber, Marco Roffi, Hervé Rousseau, Udo Sechtem, Per Anton Sirnes, Regula S von Allmen, Christiaan J M Vrints, ESC Committee for Practice Guidelines
雑誌名: Eur Heart J. 2014 Nov 1;35(41):2873-926. doi: 10.1093/eurheartj/ehu281. Epub 2014 Aug 29.
Abstract/Text
PMID 25173340  Eur Heart J. 2014 Nov 1;35(41):2873-926. doi: 10.1093/e・・・
著者: Ryuzo Sakata, Yoshitaka Fujii, Hiroyuki Kuwano
雑誌名: Gen Thorac Cardiovasc Surg. 2010 Jul;58(7):356-83. doi: 10.1007/s11748-010-0604-0.
Abstract/Text
PMID 20628854  Gen Thorac Cardiovasc Surg. 2010 Jul;58(7):356-83. doi:・・・

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