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31 Fabry disease in east asia 31 东亚法布里病
Q2 Medicine Pub Date : 2019-04-01 DOI: 10.1136/heartasia-2019-apahff.31
Alex P. W. Lee, Kevin Kh Kam, E. Fung, Josie T Y Chow, Yiting Fan, P. Teekakirikul, K. Wong, J. Y. Chan, David K. H. Chan, B. Sheng
Fabry disease (FD) is an X-linked inherited lysosomal storage disorder that results from mutations in the α-galactosidase A gene (GLA), leading to deficient alpha-galactosidase A (α-GalA) activity and subsequent accumulation of globotriaosylceramide (Gb3) in a variety of tissues.1 The estimated prevalence of classic FD in males ranges from 1:8000 to 1:117,000,2 likely an underestimation given its non-specific manifestations. The mutation IVS4 +919G>A (c.936+919G>A) associated with founder effect in East Asia was first described in Taiwan.3Left ventricular hypertrophy (LVH) is a hallmark of the later-onset cardiac variant of FD. The prevalence of FD among adult patients with LVH is unknown. In an ongoing FD screening programme from August 2017 through 2018, we measured plasma α-GalA activity using dried blood spot testing in 143 consecutive male patients with LVH (defined as maximal LV wall thickness ≥13 mm on echocardiography). Patients with low α-GalA activity underwent GLA gene sequencing analysis and endomyocardial biopsy. Four of 143 patients with LVH (2.8%) had low plasma α-GalA activity (0.4±0.2 μmol/L wb/hr; 3%–15% of the mean in normal controls). All 4 unrelated patients (aged 53–74 years) shared the same IVS4 +919G>A mutation with maximal LV wall thickness ranging from 14–29 mm. None had extracardiac manifestations but presented with hypertrophic cardiomyopathy, hypertension, heart failure, or aortic stenosis. Endomyocardial biopsy performed in one patient showed hypertrophic cardiomyocytes with sarcoplasmic vacuolisation. Our results suggest that FD should be considered as a cause of LVH in adult men even when other more usual causes of LVH are present. References Germain DP. Fabry disease. Orphanet J Rare Dis 2010;5:30. Meikle PJ, Hopwood JJ, Clague AE, Carey WF. Prevalence of lysosomal storage disorders. JAMA 1999;281:249. Lin HY, Chong KW, Hsu JH, Yu HC, Shih CC, Huang CH, Lin SJ, Chen CH, Chiang CC, Ho HJ, Lee PC, Kao CH, Cheng KH, Hsueh C, Niu DM. High incidence of the cardiac variant of Fabry disease revealed by newborn screening in the Taiwan Chinese population. Circ Cardiovasc Genet 2009;2:450.
Fabry病(FD)是一种由α-半乳糖苷酶A基因(GLA)突变引起的X连锁遗传性溶酶体储存障碍,导致α-半乳糖苷酶A(α-GalA)活性不足,随后球三糖基神经酰胺(Gb3)在各种组织中积累。1男性典型FD的估计患病率在1:8000至1:117000之间,2鉴于其非特异性表现,可能被低估了。与东亚创始人效应相关的变异IVS4+919G>A(c.936+919G>A)首次在台湾被描述。3左心室肥大(LVH)是FD晚发心脏变异的标志。FD在LVH成年患者中的患病率尚不清楚。在2017年8月至2018年正在进行的FD筛查计划中,我们使用干血点测试对143名连续患有LVH(定义为最大左心室壁厚≥13 超声心动图上的mm)。α-GaA活性低的患者接受了GLA基因测序分析和心肌内活检。143例LVH患者中有4例(2.8%)血浆α-GalA活性较低(0.4±0.2μmol/L wb/hr;为正常对照组平均值的3%-15%)。所有4名无关患者(年龄53–74岁)共享相同的IVS4+919G>A 左心室最大壁厚为14-29的突变 无心外表现,但表现为肥厚性心肌病、高血压、心力衰竭或主动脉狭窄。一名患者的心肌内活检显示心肌细胞肥大,肌浆空泡化。我们的研究结果表明,即使存在其他更常见的LVH原因,FD也应被视为成年男性LVH的原因。参考Germain DP。法布里病。孤儿J罕见病2010;5:30。Meikle PJ,Hopwood JJ,Clague AE,Carey WF。溶酶体储存障碍的患病率。JAMA 1999;林,Chong KW,Hsu JH,Yu HC,Shih CC,Huang CH,Lin SJ,Chen CH,Chiang CC,Ho HJ,Lee PC,Kao CH,Cheng KH,Hsueh C,Niu DM。台湾华人新生儿筛查显示Fabry病心脏变异的高发病率。Circ Cardiovasc Genet 2009;2:450。
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引用次数: 0
16 ECMO and MCS for patients undergoing PCI: experience from taipei veterans general hospital 16台北荣民总医院PCI手术之ECMO与MCS之经验
Q2 Medicine Pub Date : 2019-04-01 DOI: 10.1136/heartasia-2019-apahff.14
Wei-Ting Wang
Intra-aortic balloon pump (IABP), which simultaneously augments coronary blood flow and decreases myocardial oxygen demand, usually provides haemodynamic support in patients with impaired left ventricular function undergoing percutaneous coronary intervention (PCI)1 2 or for patients with established cardiogenic shock.3 Several observational studies have reported that prophylactic IABP insertion could reduce major adverse cardiovascular events (MACE) compared with a provisional counterpulsation strategy during high-risk PCI.4 However, meta-analysis did not reveal a benefit of routine elective use of IABP or percutaneous ventricular assisted device.5 Due to a lack of national data from Taiwan, this presentation therefore summarises the experience at Taipei Veterans General Hospital, and the patients’ clinical characteristics and long-term outcomes including cardiac mortality and MACE associated with high-risk PCI and/or acute myocardial infarction. References Kern MJ, Aguirre F, Bach R, Donohue T, Siegel R, Segal J. Augmentation of coronary blood flow by intra-aortic balloon pumping in patients after coronary angioplasty. Circulation 1993;87:500–511. Cohen M, Urban P, Christenson JT, Joseph DL, Freedman RJ Jr, Miller MF, Ohman EM, Reddy RC, Stone GW, Ferguson JJ 3rd; Benchmark Registry Collaborators. Intra-aortic balloon counterpulsation in US and non-US centres: results of the Benchmark Registry. Eur Heart J 2003;24:1763–1770. Sjauw KD, Engstrom AE, Vis MM, van der Schaaf RJ, Baan J Jr, Koch KT, de Winter RJ, Piek JJ, Tijssen JG, Henriques JP. A systematic review and meta-analysis of intra-aortic balloon pump therapy in ST-elevation myocardial infarction: should we change the guidelines?Eur Heart J 2009;30:459–468. Mishra S, Chu WW, Torguson R, Wolfram R, Deible R, Suddath WO, Pichard AD, Satler LF, Kent KM, Waksman R. Role of prophylactic intra-aortic balloon pump in high-risk patients undergoing percutaneous coronary intervention. Am J Cardiol 2006;98:608–612. Lee JM, Park J, Kang J, Jeon KH, Jung JH, Lee SE, Han JK, Kim HL, Yang HM, Park KW, Kang HJ, Koo BK, Kim SH, Kim HS. The efficacy and safety of mechanical hemodynamic support in patients undergoing high-risk percutaneous coronary intervention with or without cardiogenic shock: Bayesian approach network meta-analysis of 13 randomized controlled trials. Int J Cardiol2015;184:36–46.
主动脉内球囊泵(IABP)在增加冠状动脉血流量的同时降低心肌需氧量,通常为接受经皮冠状动脉介入治疗(PCI)的左心室功能受损患者或心源性休克患者提供血流动力学支持一些观察性研究报道,与临时反搏策略相比,预防性插入IABP可以减少高危pci期间的主要不良心血管事件(MACE)。然而,荟萃分析并未显示常规选择性使用IABP或经皮心室辅助装置的益处由于缺乏台湾的全国性数据,本报告总结了台北荣民总医院的经验,以及患者的临床特征和长期预后,包括与高危PCI和/或急性心肌梗死相关的心脏死亡率和MACE。引用文献Kern MJ, Aguirre F, Bach R, Donohue T, Siegel R, Segal J.主动脉内球囊泵送对冠状动脉成形术后冠状动脉血流量的影响。发行量1993;87:500 - 511。科恩M,厄本P,克里斯滕森JT,约瑟夫DL,弗里德曼RJ Jr,米勒MF,欧曼EM,雷迪RC,斯通GW,弗格森JJ第三;基准注册表协作者。美国和非美国中心的主动脉内球囊反搏:基准登记的结果。[J] .中华医学杂志,2003;24(1):663 - 670。Sjauw KD, Engstrom AE, Vis MM, van der Schaaf RJ, Baan J Jr, Koch KT, de Winter RJ, Piek JJ, Tijssen JG, Henriques JP。st段抬高型心肌梗死主动脉内球囊泵治疗的系统回顾和荟萃分析:我们应该改变指南吗?[J] .中华医学杂志,2009;30(3):459 - 468。Mishra S, Chu WW, Torguson R, Wolfram R, Deible R, sudth WO, Pichard AD, Satler LF, Kent KM, Waksman R.经皮冠状动脉介入治疗高危患者预防性主动脉内球囊泵的作用。[J]中国生物医学工程学报,2006;22(3):391 - 391。李建民,朴健,姜健,全健,郑建辉,李世世,韩建军,金海龙,杨海明,朴光光,姜海杰,具宝康,金善,金洪生。机械血流动力学支持在高危经皮冠状动脉介入治疗伴或不伴心源性休克患者中的疗效和安全性:13项随机对照试验的贝叶斯方法网络meta分析中华心血管病杂志,2015;19(4):391 - 391。
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引用次数: 1
12 Challenges in shock management in the emergency department 12 急诊科休克管理面临的挑战
Q2 Medicine Pub Date : 2019-04-01 DOI: 10.1136/HEARTASIA-2019-APAHFF.12
C. Graham
The diagnosis and emergency management of patients with shock (poor end organ perfusion with reduced tissue oxygen delivery, usually associated with systolic hypotension) is difficult even in optimum circumstances. The challenge is multiplied when patients have to be managed in overcrowded and poorly resourced emergency departments (ED). In Hong Kong, public hospital EDs manage over two million patient attendances annually, equivalent to 30% of Hong Kong’s population. Around 30% of ED patients require emergency hospital admission, with the majority being more than 80 years old. Hong Kong’s ageing population, with its associated comorbidities and polypharmacy, has inevitably contributed to rising numbers of critically ill ED patients in recent years. Shock is a major cause (and consequence) of critical illness in ED patients. Hypovolaemic shock is frequently secondary to gastrointestinal bleeding and trauma; septic shock is increasingly common due to better recognition in the ED and more patients with chronic immunosuppression. Cardiogenic shock is common, usually due to acute myocardial infarction. Optimum treatment for these patients is undoubtedly emergency revascularisation by primary percutaneous coronary intervention (PCI). Hong Kong currently does not have a regionalised or coordinated PCI service and this may contribute to the poor outcomes seen in elderly patients with cardiogenic shock. Increasingly, patients with acute on chronic heart failure often present with shock and require a coordinated specialist approach at the earliest opportunity to improve outcomes. Comprehensive collaboration between emergency medicine physicians, cardiologists, cardiothoracic surgeons and critical care services and shared clinical management are vital to optimise patient outcomes.
即使在最佳情况下,休克患者(末端器官灌注不良,组织氧输送减少,通常与收缩性低血压有关)的诊断和应急管理也很困难。当病人不得不在人满为患、资源匮乏的急诊室(ED)接受治疗时,挑战就会成倍增加。在香港,公立医院急诊科每年管理超过200万人次的病人,相当于香港人口的30%。大约30%的ED患者需要紧急入院,其中大多数患者年龄超过80岁。香港人口老龄化及其相关的合并症和多药治疗,不可避免地导致了近年来危重ED患者人数的增加。休克是ED患者危重症的主要原因(和后果)。低血糖性休克通常继发于胃肠道出血和创伤;感染性休克越来越常见,这是由于在ED中有更好的识别能力,以及越来越多的慢性免疫抑制患者。心源性休克是常见的,通常由急性心肌梗死引起。对这些患者的最佳治疗无疑是通过直接经皮冠状动脉介入治疗(PCI)进行紧急血运重建。香港目前没有区域化或协调的PCI服务,这可能会导致老年心源性休克患者的不良结局。越来越多的急慢性心力衰竭患者经常出现休克,需要尽早采取协调一致的专家方法来改善结果。急诊医生、心脏病专家、心胸外科医生和重症监护服务之间的全面合作以及共享临床管理对于优化患者结果至关重要。
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引用次数: 0
Author index 作者索引
Q2 Medicine Pub Date : 2019-04-01 DOI: 10.1136/heartasia-2019-apahff.authorindex
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引用次数: 0
19 Update of LVAD therapy in japan 19 日本LVAD治疗进展
Q2 Medicine Pub Date : 2019-04-01 DOI: 10.1136/heartasia-2019-apahff.19
G. Matsumiya
Left ventricular assist device (LVAD) has been increasingly utilised for the treatment of advanced (ACC/AHA stage D) heart failure. Three types of implantable device (HeartMate IITM, Jarvik 2000®, and EVAHEARTTM) are currently available as a bridge-to-transplant in Japan. J-MACS (Japanese registry for mechanically assisted circulatory support) has listed a total of 722 patients between December 2010 and October 2017. Mean age was 43.2 years and mean body surface area was 1.63 m2. Aetiology of heart failure was dilated cardiomyopathy in 77% and ischaemic cardiomyopathy in 11%. Pre-operative INTERMACS profile was level 1 in 10%, level 2 in 39%, level 3 in 48%, and level 4 or 5 in 4%. One hundred forty-nine (21%) patients received extracorporeal life support device first for cardiogenic shock or bridge-to-candidacy, and then underwent switch to implantable devices (bridge-to-bridge). Overall survival rate after LVAD implantation was 90% at 1 year and 86% at 2 years. Competing outcome analyses demonstrated that only 10% of patients reached heart transplantation and 60% were still on device therapy at 3 years. Causes of death were cerebrovascular accident in 44%, infection in 18%, bleeding in 6%, and device failure in 6%. Sixty-six per cent of patients required at least 1 hospital admission during an initial 1 year after device implantation. In conclusion, continuous flow LVAD was effective for bridge-to-transplant. Although the collected data indicate that the improved survival rate is acceptable, there remains potential for major complications which precludes wider application of LVAD.
左心室辅助装置(LVAD)已越来越多地用于治疗晚期(ACC/AHA D期)心力衰竭。三种类型的植入式设备(HeartMate IITM、Jarvik 2000®和EVAHEARTTM)目前可作为日本移植的桥梁。J-MACS(日本机械辅助循环支持注册中心)列出了2010年12月至2017年10月期间共722名患者。平均年龄43.2岁,平均体表面积1.63平方米。心力衰竭的病因是扩张型心肌病77%,缺血性心肌病11%。术前INTERMACS表现为1级占10%,2级占39%,3级占48%,4或5级占4%。一百四十九(21%)名患者首先接受了体外生命支持装置治疗心源性休克或桥接至候选,然后接受了植入式装置的转换(桥接至桥接)。LVAD植入术后的总生存率在1 2年时为86%。竞争性结果分析表明,只有10%的患者完成了心脏移植,60%的患者在3年时仍在接受设备治疗。死亡原因为脑血管意外占44%,感染占18%,出血占6%,器械故障占6%。66%的患者在最初的1 植入装置后一年。结论:连续流LVAD对桥移植是有效的。尽管收集的数据表明,生存率的提高是可以接受的,但仍有可能出现严重并发症,从而阻碍LVAD的广泛应用。
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引用次数: 0
15 Impella® use in protected PCI and cardiogenic shock Impella®在保护性PCI和心源性休克中的应用
Q2 Medicine Pub Date : 2019-04-01 DOI: 10.1136/heartasia-2019-apahff.15
C. K. Lung
Intra-aortic balloon pump (IABP) has been used for many years in high-risk PCI or cardiogenic shock. However, the support provided by IABP is limited and a major randomised controlled trial failed to demonstrate a beneficial effect.1 Impella® is a family of percutaneous mechanical circulatory support (MCS) devices that are used in protected PCI and cardiogenic shock.2 Depending on the device model, they provide a blood flow ranging from 2.5 to 5 L/min. The resulting augmented support increases mean arterial blood pressure, cardiac output and coronary flow while unloading left ventricular filling pressure to reduce myocardial oxygen demand.2 3 This is an ideal form of temporary support device for the patient. Moreover, the device is easy to set up and can be inserted very quickly to stabilise the patient. This presentation provides a review on the current data regarding the use of Impella MCS devices. References Thiele H, Zeymer U, Neumann FJ, Ferenc M, Olbrich HG, Hausleiter J, Richardt G, Hennersdorf M, Empen K, Fuernau G, Desch S, Eitel I, Hambrecht R, Fuhrmann J, Böhm M, Ebelt H, Schneider S, Schuler G, Werdan K; IABP-SHOCK II Trial Investigators. Intraaortic balloon support for myocardial infarction with cardiogenic shock. N Engl J Med 2012;367:1287–1296. O’Neill WW, Kleiman NS, Moses J, Henriques JP, Dixon S, Massaro J, Palacios I, Maini B, Mulukutla S, Dzavík V, Popma J, Douglas PS, Ohman M. A prospective, randomized clinical trial of hemodynamic support with Impella 2.5 versus intra-aortic balloon pump in patients undergoing high-risk percutaneous coronary intervention: the PROTECT II study. Circulation 2012;126:1717–1727. O’Neill WW, Grines C, Schreiber T, Moses J, Maini B, Dixon SR, Ohman EM. Analysis of outcomes for 15,259 US patients with acute myocardial infarction cardiogenic shock (AMICS) supported with the Impella device. Am Heart J 2018;202:33–38.
主动脉内球囊泵(IABP)已用于高危PCI或心源性休克多年。然而,IABP提供的支持是有限的,一项主要的随机对照试验未能证明其有益效果。1 Impella®是一系列经皮机械循环支持(MCS)设备,用于保护PCI和心源性休克。2根据设备型号,它们可提供2.5至5的血流量 L/分钟。由此产生的增强支撑增加了平均动脉血压、心输出量和冠状动脉流量,同时减轻了左心室充盈压力,以减少心肌氧需求。23这是患者临时支撑装置的理想形式。此外,该设备易于设置,可以很快插入以稳定患者。本演示文稿回顾了有关Impella MCS设备使用的当前数据。参考文献Thiele H、Zeymer U、Neumann FJ、Ferenc M、Olbrich HG、Hausleiter J、Richardt G、Hennersdorf M、Empen K、Fuernau G、Desch S、Eitel I、Hambrecht R、Fuhrmann J、Böhm M、Ebelt H、Schneider S、Schuler G、Werdan K;IABP-SHOCK II试验调查员。主动脉内球囊支持治疗心肌梗死合并心源性休克。《英国医学杂志》2012;367:1287–1296。O'Neill WW、Kleiman NS、Moses J、Henriques JP、Dixon S、Massaro J、Palacios I、Maini B、Mulukutla S、Dzavík V、Popma J、Douglas PS、Ohman M.接受高危经皮冠状动脉介入治疗的患者使用Impella 2.5与主动脉内球囊泵进行血流动力学支持的前瞻性随机临床试验:PROTECT II研究。2012年发行量;126:1717–1727。O'Neill WW,Grines C,Schreiber T,Moses J,Maini B,Dixon SR,Ohman EM。15259名使用Impella设备支持的急性心肌梗死心源性休克(AMICS)美国患者的结果分析。Am Heart J 2018;202:33–38。
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引用次数: 0
29 Management of pulmonary hypertension in systemic lupus erythematosus patients 系统性红斑狼疮患者肺动脉高压的处理
Q2 Medicine Pub Date : 2019-04-01 DOI: 10.1136/heartasia-2019-apahff.29
P. Wong
Pulmonary arterial hypertension (PAH) is a complex and devastating disease. According to a longitudinal United States-based registry, connective tissue disease (CTD) accounted for more than 50% of all patients with PAH, in which systemic sclerosis comprised the largest CTD-related PAH.1 In contrast to Western countries, systemic lupus erythematosus (SLE) is a more common CTD than systemic sclerosis in the Asia-Pacific region.2 A cohort study from China has shown that SLE, instead of systemic sclerosis, comprised the largest proportion of all CTD-related PAH.3 The prevalence of PAH in SLE is estimated at 0.5%–17.5%.4 The pathogenesis of PAH involves multiple mechanisms including vasculitis, in situ thrombosis to interstitial lung disease which may all increase pulmonary vascular resistance and lead to right heart failure. The leading risk factors for the development of PAH in SLE patients include Raynaud’s phenomenon, anti-U1 RNP antibody and anti-cardiolipin antibodies positivity. Since PAH is potentially life-threatening, early detection is crucial to improve the outcomes of this condition. Currently, the diagnostic algorithm for PAH in SLE patients follows that of international guidelines. Diagnosis is confirmed by right heart catheterisation. Treatments are similar to the therapeutic interventions for patients with idiopathic PAH. Since inflammatory and dysregulated immune components may play a major role in the pathogenesis of PAH in SLE, glucocorticoids and immunosuppressive therapies including cyclophosphamide are used, although the immunosuppressive therapy trials were small, uncontrolled studies only. Regular follow-up with prognostic evaluation and risk assessment should be performed and the treatment should be individualised accordingly. References McGoon MD, Miller DP. REVEAL: a contemporary US pulmonary arterial hypertension registry. Eur Respir Rev 2012;21:8–18. Jakes RW, Bae SC, Louthrenoo W, Mok CC, Navarra SV, Kwon N. Systematic review of the epidemiology of systemic lupus erythematosus in the Asia-Pacific region: prevalence, incidence, clinical features, and mortality. Arthritis Care Res (Hoboken) 2012;64:159–168. Zhao J, Wang Q, Liu Y, Tian Z, Guo X, Wang H, Lai J, Huang C, Yang X, Li M, Zeng X. Clinical characteristics and survival of pulmonary arterial hypertension associated with three major connective tissue diseases: A cohort study in China. Int J Cardiol 2017;236:432–437. Condliffe R, Kiely DG, Peacock AJ, Corris PA, Gibbs JS, Vrapi F, Das C, Elliot CA, Johnson M, DeSoyza J, Torpy C, Goldsmith K, Hodgkins D, Hughes RJ, Pepke-Zaba J, Coghlan JG. Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era. Am J Respir Crit Care Med 2009;179:151–157.
肺动脉高压是一种复杂而严重的疾病。根据美国的一项纵向登记,结缔组织病(CTD)占所有PAH患者的50%以上,其中系统性硬化症是与CTD相关的最大PAH。1与西方国家相比,在亚太地区,系统性红斑狼疮(SLE)是一种比系统性硬化更常见的CTD。2来自中国的一项队列研究表明,PAH在SLE中的患病率估计为0.5%-17.5%。4 PAH的发病机制涉及多种机制,包括血管炎、原位血栓形成和间质性肺病,这些疾病都可能增加肺血管阻力并导致右心衰竭。SLE患者发生PAH的主要危险因素包括雷诺现象、抗U1 RNP抗体和抗心磷脂抗体阳性。由于PAH可能危及生命,早期检测对改善这种情况的结果至关重要。目前,SLE患者PAH的诊断算法遵循国际指南。诊断通过右心导管进行确认。治疗类似于特发性PAH患者的治疗干预。由于炎症和失调的免疫成分可能在SLE PAH的发病机制中发挥主要作用,因此使用了糖皮质激素和包括环磷酰胺在内的免疫抑制疗法,尽管免疫抑制治疗试验规模较小,仅为非对照研究。应定期随访,进行预后评估和风险评估,并对治疗进行相应的个性化处理。参考文献McGoon医学博士、Miller医学博士。揭示:当代美国肺动脉高压登记。Eur Respir Rev 2012;21:8-18。Jakes RW,Bae SC,Louthrenoo W,Mok CC,Navarra SV,Kwon N.亚太地区系统性红斑狼疮流行病学的系统综述:患病率、发病率、临床特征和死亡率。关节炎护理研究(霍博肯)2012;64:159–168.赵J,王Q,刘Y,田Z,郭X,王H,赖J,黄C,杨X,李M,曾X。肺动脉高压合并三种主要结缔组织疾病的临床特征和生存率:中国的一项队列研究。Int J Cardiol 2017;236:432–437。Condlife R、Kiely DG、Peacock AJ、Corris PA、Gibbs JS、Vrapi F、Das C、Elliot CA、Johnson M、DeSoyza J、Torpy C、Goldsmith K、Hodgkins D、Hughes RJ、Pepke Zaba J、Coghlan JG。现代治疗时代与结缔组织疾病相关的肺动脉高压。Am J Respir Crit Care Med 2009;179:151-157。
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引用次数: 0
20 Developing comprehensive mechanical circulatory support in singapore 20 .在新加坡发展综合机械循环支持
Q2 Medicine Pub Date : 2019-04-01 DOI: 10.1136/heartasia-2019-apahff.20
C. Sivathasan
Heart transplantation was initiated in Singapore in 1990. Three to six heart transplants are performed annually. The waiting list mortality was about 30%. Hence, mechanical circulatory support was initiated with HeartMate I in 2001. Extracorporeal membrane oxygenation (ECMO) for acute life support became available by 2003. Durable implantable rotary left ventricular assist device (LVAD) with HeartMate IITM (HMII) was introduced in 2007, followed by HeartWareTM HVADTM in 2012 and HeartMate 3TM (HM3) in 2015. 90 consecutive durable implantable devices (58 HMII, 21 HVAD and 11 HM3), were placed from May 2009 to December 2017. Of these, 65 were placed as bridge-to-transplant. Overall perioperative mortality was 7 (7.7%). 26 were INTERMACS level 1 patients, bridged with ECMO without perioperative mortality. Median ICU stay and hospital stay were 8 and 31 days, respectively. Mean duration of support was 907 days. The longest support was 8.6 years. 45 had been on ongoing support, and 23 were transplanted. Late mortality occurred in 13 patients, 5 due to stroke. Driveline infection developed in 28.7%. 57.1% were associated with driveline trauma. Pocket infection developed in 5 patients. In the initial 78 patients, 17 (21.7%) experienced gastrointestinal bleeding (GIB); 11 with recurrent GIB were successfully treated with thalidomide. 6 developed aortic regurgitation, 5 underwent modified Park stitch. Implantable cardioverter defibrillator placement pre-LVAD did not confer mortality benefit. Issues with hardware occurred in 60%. Cardiac recovery occurred in 2 and their LVADs were decommissioned. Overall survival at 5 years was 84.4%, compared with INTERMACS registry data of 48%.
心脏移植于1990年在新加坡开始。每年进行三到六次心脏移植。等待名单上的死亡率约为30%。因此,HeartMate I于2001年启动了机械循环支持。用于急性生命支持的体外膜肺氧合(ECMO)于2003年开始使用。2007年推出了带有HeartMate IITM(HMII)的耐用植入式旋转左心室辅助装置(LVAD),随后于2012年推出了HeartWareTM HVADTM,并于2015年推出了HeartMate 3TM(HM3)。2009年5月至2017年12月,放置了90个连续耐用植入式装置(58个HMII、21个HVAD和11个HM3)。其中65个被放置作为移植的桥梁。围手术期总死亡率为7(7.7%)。26例为INTERMACS 1级患者,与ECMO桥接,无围手术期死亡率。中位ICU住院时间和住院时间分别为8天和31天。平均支持时间为907天。最长的支持期为8.6年。45人一直在接受支持,23人被移植。晚期死亡13例,其中5例死于中风。传动系感染发生率为28.7%,57.1%与传动系创伤有关。5名患者出现口袋感染。在最初的78例患者中,17例(21.7%)出现胃肠道出血(GIB);11例复发性GIB患者用沙利度胺成功治疗。6例出现主动脉瓣反流,5例接受改良Park缝合术。LVAD前植入的心律转复除颤器不会带来死亡率益处。60%发生硬件问题。2例发生心脏恢复,LVAD停用。5年的总生存率为84.4%,而INTERMACS注册数据为48%。
{"title":"20 Developing comprehensive mechanical circulatory support in singapore","authors":"C. Sivathasan","doi":"10.1136/heartasia-2019-apahff.20","DOIUrl":"https://doi.org/10.1136/heartasia-2019-apahff.20","url":null,"abstract":"Heart transplantation was initiated in Singapore in 1990. Three to six heart transplants are performed annually. The waiting list mortality was about 30%. Hence, mechanical circulatory support was initiated with HeartMate I in 2001. Extracorporeal membrane oxygenation (ECMO) for acute life support became available by 2003. Durable implantable rotary left ventricular assist device (LVAD) with HeartMate IITM (HMII) was introduced in 2007, followed by HeartWareTM HVADTM in 2012 and HeartMate 3TM (HM3) in 2015. 90 consecutive durable implantable devices (58 HMII, 21 HVAD and 11 HM3), were placed from May 2009 to December 2017. Of these, 65 were placed as bridge-to-transplant. Overall perioperative mortality was 7 (7.7%). 26 were INTERMACS level 1 patients, bridged with ECMO without perioperative mortality. Median ICU stay and hospital stay were 8 and 31 days, respectively. Mean duration of support was 907 days. The longest support was 8.6 years. 45 had been on ongoing support, and 23 were transplanted. Late mortality occurred in 13 patients, 5 due to stroke. Driveline infection developed in 28.7%. 57.1% were associated with driveline trauma. Pocket infection developed in 5 patients. In the initial 78 patients, 17 (21.7%) experienced gastrointestinal bleeding (GIB); 11 with recurrent GIB were successfully treated with thalidomide. 6 developed aortic regurgitation, 5 underwent modified Park stitch. Implantable cardioverter defibrillator placement pre-LVAD did not confer mortality benefit. Issues with hardware occurred in 60%. Cardiac recovery occurred in 2 and their LVADs were decommissioned. Overall survival at 5 years was 84.4%, compared with INTERMACS registry data of 48%.","PeriodicalId":12858,"journal":{"name":"Heart Asia","volume":" ","pages":"A9 - A9"},"PeriodicalIF":0.0,"publicationDate":"2019-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1136/heartasia-2019-apahff.20","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48464818","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Antithrombotic therapy after femoropopliteal artery stenting: 12-month results from Japan Postmarketing Surveillance. 股腘动脉支架植入术后的抗血栓治疗:日本上市后监测的12个月结果
Q2 Medicine Pub Date : 2019-03-30 eCollection Date: 2019-01-01 DOI: 10.1136/heartasia-2018-011114
Osami Kawarada, Michikazu Nakai, Kunihiro Nishimura, Hideki Miwa, Yusuke Iwasaki, Daitaro Kanno, Tatsuya Nakama, Yoshito Yamamoto, Nobuhiko Ogata, Masato Nakamura, Satoshi Yasuda

Objective: To investigate the effects of antithrombotic therapy on target lesion revascularisation (TLR) and major adverse cardiovascular and cerebrovascular events (MACCEs) at 12 months after femoropopliteal intervention with second-generation bare metal nitinol stents.

Methods: A total of 277 lesions in 258 limbs of 248 patients with de novo atherosclerosis in the above-the-knee femoropopliteal segment were analysed from the Japan multicentre postmarketing surveillance.

Results: At discharge, dual antiplatelet therapy (DAPT) was prescribed in 68.5% and cilostazol in 30.2% of patients. At 12 months of follow-up, prescriptions of DAPT significantly (p=0.0001) decreased to 51.2% and prescription of cilostazol remained unchanged (p=0.592) at 28.0%. Prescription of warfarin also remained unchanged (14.5% at discharge, 13.3% at 12 months, p=0.70). At 12 months, freedoms from TLR and MACCE were 89.4% and 89.7%, respectively. In a multivariate Cox proportional hazards model, neither DAPT nor cilostazol at discharge was associated with both TLR and MACCE at 12 months. However, warfarin at discharge was only independently associated with TLR at 12 months. Kaplan-Meier estimates demonstrated that warfarin at discharge yielded a significantly (p=0.013) lower freedom from TLR at 12 months than no warfarin at discharge. Freedom from TLR at 12 months by the Kaplan-Meier estimates was 77.8% (95% CI 59.0% to 88.8%) in patients with warfarin at discharge and 91.2% (95% CI 86.3% to 94.3%) in those without warfarin at discharge.

Conclusions: Clinical benefits of DAPT or cilostazol might be small in terms of TLR and MACCE at 12 months. Anticoagulation with warfarin at discharge might increase TLR at 12 months.

目的:探讨抗血栓治疗对第二代裸金属镍钛诺股腘动脉支架术后12个月靶区血管重建(TLR)和主要不良心脑血管事件(MACCEs)的影响。方法:分析日本多中心上市后监测的248例膝上股腘段新发动脉粥样硬化患者258个肢体的277个病变。结果:出院时双重抗血小板治疗(DAPT)占68.5%,西洛他唑占30.2%。随访12个月时,DAPT处方显著减少(p=0.0001)至51.2%,西洛他唑处方保持不变(p=0.592)至28.0%。华法林的处方也保持不变(出院时14.5%,12个月时13.3%,p=0.70)。在12个月时,TLR和MACCE的自由度分别为89.4%和89.7%。在多变量Cox比例风险模型中,出院时DAPT和西洛他唑均与12个月时的TLR和MACCE无关。然而,出院时华法林仅与12个月时的TLR独立相关。Kaplan-Meier估计表明,与未使用华法林相比,出院时使用华法林在12个月时产生的TLR自由度显著(p=0.013)降低。Kaplan-Meier估计,出院时使用华法林的患者12个月无TLR的发生率为77.8% (95% CI为59.0%至88.8%),出院时不使用华法林的患者为91.2% (95% CI为86.3%至94.3%)。结论:在12个月的TLR和MACCE方面,DAPT或西洛他唑的临床获益可能较小。出院时使用华法林抗凝可能增加12个月时的TLR。
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引用次数: 1
Prevalence of non-acute ischaemic cardiomyopathy in a Pakistani cohort. 巴基斯坦队列中的非急性缺血性心肌病患病率。
Q2 Medicine Pub Date : 2019-03-25 eCollection Date: 2019-01-01 DOI: 10.1136/heartasia-2018-011043
Muhammad Salman Ghazni, Saba Aijaz, Rehan Malik, Asad Z Pathan

Heart failure with reduced left ventricular ejection fraction (HFrEF) is a frequently encountered clinical scenario. Coronary angiography (CAG) is usually performed to assess obstructive epicardial coronary artery disease (CAD) and the resultant ischaemia as causes of HFrEF.

Objectives: To determine the frequency of obstructive CAD (OCAD) in patients with HFrEF and its independent predictors and outcomes.

Methods: Retrospective observational study in Tabba Heart Institute on patients who underwent CAG during the past 4 years. Patients with prior known CAD or revascularisation were excluded. OCAD was defined as per the criteria from Felker et al. Regression modelling was performed to evaluate the predictors of OCAD. Survival was compared between the groups using the log rank test.

Results: Out of 2235 patients who underwent CAG, 260 had HFrEF as a primary indication for CAG and, of these, 119 (45.8%) had OCAD. Major predictors of OCAD were age >50 years at presentation (OR 2.0, 95% CI 1.1 to 3.7), presence of chest pain (OR 4.3, 95% CI 2.3 to 8.1), family history of premature CAD (OR 2.8, 95% CI 1.3 to 5.9) and utilisation of non-invasive (NIV) stress testing before CAG (OR 3.6, 95% CI 1.8 to 7.1). Survival was significantly lower (log rank p<0.001) in patients with OCAD with no revascularisation compared with OCAD with revascularisation or those who had non-obstructive CAD, and the latter two groups had comparable survival.

Conclusions: OCAD is detected in nearly half of the patients with reduced left ventricular systolic function undergoing CAG. Clinical judgement based on thorough history and use of NIV stress testing can help in appropriate patient selection for this test.

左心室射血分数降低的心力衰竭(HFrEF)是临床上经常遇到的一种情况。冠状动脉造影术(CAG)通常用于评估阻塞性心外膜冠状动脉疾病(CAD)及其导致的缺血,这是导致 HFrEF 的原因:确定阻塞性冠状动脉疾病(OCAD)在 HFrEF 患者中的发生率及其独立预测因素和结果:方法:在塔巴心脏研究所对过去4年中接受CAG手术的患者进行回顾性观察研究。排除了既往有已知 CAD 或血管再通的患者。根据 Felker 等人的标准定义 OCAD。使用对数秩检验比较各组间的存活率:在 2235 名接受 CAG 的患者中,有 260 名以 HFrEF 作为 CAG 的主要适应症,其中 119 人(45.8%)有 OCAD。OCAD 的主要预测因素包括:发病时年龄大于 50 岁(OR 2.0,95% CI 1.1 至 3.7)、出现胸痛(OR 4.3,95% CI 2.3 至 8.1)、早发 CAD 家族史(OR 2.8,95% CI 1.3 至 5.9)以及在接受 CAG 前使用无创 (NIV) 压力测试(OR 3.6,95% CI 1.8 至 7.1)。存活率明显降低(对数秩p结论:在接受 CAG 检查的左心室收缩功能减退的患者中,近一半的患者可检测到 OCAD。根据详尽的病史和使用 NIV 压力测试进行临床判断有助于选择合适的患者进行该测试。
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引用次数: 0
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Heart Asia
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